Hematopoietic and Lymphoid Systems Chapter 9
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Questions and Answers

What is the purpose of reserving hematopoietic stem cell transplantation for younger patients?

  • To reduce the risk of neoplastic cell growth
  • To ensure a higher success rate of transplantation (correct)
  • To prioritize treatment for patients with a worse prognosis
  • To reserve conventional therapies for older patients

    • What is the characteristic of lymph nodes in patients with mutations leading to a worse prognosis?

  • Effacement of the nodes (correct)
  • Necrosis of the nodes
  • Inflammation of the nodes
  • Enlargement of the nodes

    • What is the primary goal of BCL2 antagonists in treating hematopoietic and lymphoid systems disorders?

  • To induce cellular apoptosis (correct)
  • To promote cellular differentiation
  • To inhibit cellular migration
  • To enhance cellular proliferation

    • What type of cells are typically present in lymph nodes and are larger than usual in certain disease states?

    <p>Centrocytes</p> Signup and view all the answers

    In which scenario is cure typically only achievable?

    <p>With hematopoietic stem cell transplantation</p> Signup and view all the answers

    What is the significance of TP53 presence in the context of hematopoietic and lymphoid systems disorders?

    <p>It induces cell apoptosis</p> Signup and view all the answers

    What is the typical immunoglobulin class of surface immunoglobulin on neoplastic cells?

    <p>IgM</p> Signup and view all the answers

    What is the characteristic nuclear feature of neoplastic cells?

    <p>Dispersed chromatin</p> Signup and view all the answers

    What is the typical cytoplasmic feature of neoplastic cells?

    <p>Basophilic cytoplasm with prominent ribosomes</p> Signup and view all the answers

    What is the characteristic of the extranodal marginal zone lymphoma?

    <p>Extranodal location with centrocyte-like cells</p> Signup and view all the answers

    What is the occasional feature of neoplastic cells?

    <p>Anaplastic features</p> Signup and view all the answers

    What is a characteristic of Burkitt lymphoma?

    <p>It is associated with translocations involving the MYC gene</p> Signup and view all the answers

    What is the association of neoplastic cells with Helicobacter pylori?

    <p>H. pylori is associated with gastric MALT lymphoma</p> Signup and view all the answers

    What is the characteristic of the nuclear contour of neoplastic cells?

    <p>Round or irregular shape</p> Signup and view all the answers

    Where is Burkitt lymphoma most commonly found?

    <p>In parts of Africa</p> Signup and view all the answers

    What is the presentation of neoplastic cells in the salivary gland?

    <p>Extranodal marginal zone lymphoma of the salivary gland</p> Signup and view all the answers

    What is the result of the translocation involving the MYC gene in Burkitt lymphoma?

    <p>Overexpression of the MYC transcription factor</p> Signup and view all the answers

    What type of neoplasm is Burkitt lymphoma?

    <p>B-cell neoplasm</p> Signup and view all the answers

    What is the morphology of Burkitt lymphoma characterized by?

    <p>The presence of starry sky appearance</p> Signup and view all the answers

    What is Hairy cell leukemia?

    <p>A type of lymphoid neoplasm with a distinctive morphology</p> Signup and view all the answers

    What is the association between Burkitt lymphoma and geographic areas?

    <p>It is endemic in parts of Africa and occurs sporadically in other geographic areas</p> Signup and view all the answers

    What is the result of the translocation involving the MYC gene in terms of the cell cycle?

    <p>It leads to uncontrolled cell proliferation</p> Signup and view all the answers

    What is the typical genetic alteration found in myeloma cells?

    <p>Chromosomal translocations involving the IGH locus on chromosome 14</p> Signup and view all the answers

    What is the significance of monoclonal gammopathy of undetermined significance (MGUS)?

    <p>It is a precursor to myeloma</p> Signup and view all the answers

    What is the typical presentation of patients with monoclonal gammopathy of undetermined significance (MGUS)?

    <p>They are typically asymptomatic and diagnosed incidentally</p> Signup and view all the answers

    What is the role of the IGH locus on chromosome 14?

    <p>It is a common site for chromosomal translocations in myeloma cells</p> Signup and view all the answers

    What is the significance of the cyclyn D1 gene in the context of myeloma?

    <p>It is an oncogene that promotes cell proliferation</p> Signup and view all the answers

    What is the characteristic of myeloma cells in terms of immunoglobulin production?

    <p>They produce monoclonal immunoglobulins</p> Signup and view all the answers

    What is the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in older adults?

    <p>It is common, affecting up to 5% of older adults</p> Signup and view all the answers

    What is the significance of the presence of monoclonal proteins in the blood or urine?

    <p>It is often seen in patients with MGUS</p> Signup and view all the answers

    What type of cells are primarily affected in Myeloma, leading to defects in humoral immunity?

    <p>B cells</p> Signup and view all the answers

    Which of the following is a characteristic appearance of Reed-Sternberg cells?

    <p>Puffy nucleus resembling popcorn</p> Signup and view all the answers

    In Myeloma, what leads to renal failure?

    <p>Obstructive protein casts</p> Signup and view all the answers

    What type of infections are patients with L&H variants more susceptible to?

    <p>Bacterial infections</p> Signup and view all the answers

    Which characteristic best describes the lymph nodes in individuals with Myeloma?

    <p>Large nodules with excessive macrophages</p> Signup and view all the answers

    What is the consequence of increased numbers of macrophages in tumor nodules associated with Myeloma?

    <p>Reduced immune response to pathogens</p> Signup and view all the answers

    Which statement about Myeloma's impact on immunity is correct?

    <p>It causes a deficiency in both humoral and cell-mediated immunity.</p> Signup and view all the answers

    What are the small B cells in Myeloma nodules typically associated with?

    <p>Uncontrolled clonal expansion</p> Signup and view all the answers

    The median survival time of patients with Adu T-cell leukemia/lymphoma is more than 10 years.

    <p>False</p> Signup and view all the answers

    Peripheral T-cell lymphoma encompasses a homogeneous group of tumors.

    <p>False</p> Signup and view all the answers

    Reed-Sternberg cells are characteristic of non-Hodgkin lymphomas.

    <p>False</p> Signup and view all the answers

    Patients with peripheral T-cell lymphoma often suffer from symptoms related to tumor-derived inflammatory products.

    <p>True</p> Signup and view all the answers

    Hodgkin lymphomas are characterized by the presence of small B cells.

    <p>False</p> Signup and view all the answers

    Peripheral T-cell lymphoma makes up more than 50% of non-Hodgkin lymphomas.

    <p>False</p> Signup and view all the answers

    Adu T-cell leukemia/lymphoma is a type of non-aggressive tumor.

    <p>False</p> Signup and view all the answers

    Peripheral T-cell lymphoma is a type of cancer that can be cured with chemotherapy.

    <p>False</p> Signup and view all the answers

    Extranodal marginal zone lymphoma is a common type of lymphoma found in the stomach.

    <p>False</p> Signup and view all the answers

    DLBCL is a indolent and slowly progressing disease.

    <p>False</p> Signup and view all the answers

    Burkitt lymphoma is a type of lymphoma that typically affects older adults.

    <p>False</p> Signup and view all the answers

    The bone marrow is a common site of involvement in DLBCL.

    <p>True</p> Signup and view all the answers

    The presence of monoclonal gammopathy of undetermined significance (MGUS) is a definitive diagnosis of myeloma.

    <p>False</p> Signup and view all the answers

    DLBCL is typically cured in approximately 20% of patients.

    <p>False</p> Signup and view all the answers

    Burkitt lymphoma is a type of lymphoma that typically involves the lymph nodes.

    <p>False</p> Signup and view all the answers

    The treatment of DLBCL typically involves only chemotherapy.

    <p>False</p> Signup and view all the answers

    Monoclonal gammopathy of undetermined significance (MGUS) is a rare condition in older adults.

    <p>False</p> Signup and view all the answers

    The IGH locus on chromosome 14 is involved in the translocation of pro-oncogenes in Myeloma.

    <p>True</p> Signup and view all the answers

    Myeloma cells typically produce immunoglobulins with a mixed kappa and lambda light chain.

    <p>False</p> Signup and view all the answers

    Monoclonal proteins in the blood or urine are always indicative of Myeloma.

    <p>False</p> Signup and view all the answers

    The cyclyn D1 gene is a tumor suppressor gene in Myeloma.

    <p>False</p> Signup and view all the answers

    Chromosomal translocations are a rare genetic alteration found in Myeloma cells.

    <p>False</p> Signup and view all the answers

    Patients with Monoclonal gammopathy of undetermined significance (MGUS) always present with symptoms.

    <p>False</p> Signup and view all the answers

    The presence of monoclonal proteins in the blood or urine is diagnostic of Myeloma.

    <p>False</p> Signup and view all the answers

    Langerhans cells are typically found in the lung, skin, or gut.

    <p>True</p> Signup and view all the answers

    Reed-Sternberg cells are known for their resemblance to cell types found in the lung.

    <p>False</p> Signup and view all the answers

    Unifocal unisystem Langerhans cell disease is a multifocal unisystem disease.

    <p>False</p> Signup and view all the answers

    Langerhans cells can be identified by CD1a.

    <p>True</p> Signup and view all the answers

    The proliferating plasma cells show deleterious effects on the skeletal system.

    <p>True</p> Signup and view all the answers

    Langerhans cell histiocytosis can be cured with antibiotics.

    <p>False</p> Signup and view all the answers

    The kidney contributes to morbidity alongside the skeletal system in certain hematological conditions.

    <p>True</p> Signup and view all the answers

    Variances found in Reed-Sternberg cells have a strong resemblance to those observed in the gastrointestinal tract.

    <p>False</p> Signup and view all the answers

    Unifocal unisystem Langerhans cell disease is often asymptomatic.

    <p>True</p> Signup and view all the answers

    Langerhans cells are a type of T cell.

    <p>False</p> Signup and view all the answers

    Mixed cellularity subtype is commonly associated with the reproductive system.

    <p>False</p> Signup and view all the answers

    Multifocal unisystem Langerhans cell disease is a single-site disease.

    <p>False</p> Signup and view all the answers

    Diversity in cell appearance can be significant indicators of various underlying conditions in the lymphatic system.

    <p>True</p> Signup and view all the answers

    Langerhans cell histiocytosis is a type of cancer.

    <p>False</p> Signup and view all the answers

    What is the characteristic of follicular lymphoma that correlates with a more aggressive clinical behavior?

    <p>Cleaved nucleus</p> Signup and view all the answers

    What is the typical median survival time for patients with follicular lymphoma?

    <p>Less than 1 year</p> Signup and view all the answers

    What is the characteristic of centrocytes in follicular lymphoma?

    <p>Mixed with variable sized cells</p> Signup and view all the answers

    What is the significance of centrobasts in follicular lymphoma?

    <p>Predominance correlates with more aggressive behavior</p> Signup and view all the answers

    What is the characteristic of the nuclear contour of neoplastic cells in follicular lymphoma?

    <p>Cleaved</p> Signup and view all the answers

    What is the significance of vesicular chromatin in follicular lymphoma?

    <p>Coarse and condensed</p> Signup and view all the answers

    What is the typical cytoplasmic feature of neoplastic cells in follicular lymphoma?

    <p>Scant</p> Signup and view all the answers

    What is the significance of the presence of multiple nucleoli in follicular lymphoma?

    <p>Uncommon</p> Signup and view all the answers

    What is the approximate percentage of cases where a wide variety of neoplasm transforms into a symptomatic neoplasm?

    <p>About 1%</p> Signup and view all the answers

    What is the role of cytokine interleukin 6 (IL-6) in the context of myeloma cells?

    <p>It supports multiple myeloma</p> Signup and view all the answers

    What is the characteristic of a group of tumors that comprises Langerhans cells?

    <p>They have a broad description</p> Signup and view all the answers

    What is the rarity of very rare cases where a wide variety of neoplasm transforms into a symptomatic sarcoma?

    <p>Very rare</p> Signup and view all the answers

    What is the association between some neoplasms and Helicobacter pylori?

    <p>Occasional</p> Signup and view all the answers

    What is the typical genetic alteration found in myeloma cells in terms of immunoglobulin production?

    <p>They are primarily affected in terms of humoral immunity</p> Signup and view all the answers

    What is the significance of the presence of monoclonal proteins in the blood or urine?

    <p>It is a characteristic of myeloma cells</p> Signup and view all the answers

    What is the consequence of increased numbers of macrophages in tumor nodules associated with myeloma?

    <p>It leads to renal failure</p> Signup and view all the answers

    What is the primary function of the MYC gene in relation to cancer metabolism?

    <p>The MYC gene acts as a master regulator of Warburg metabolism, promoting rapid cell growth.</p> Signup and view all the answers

    How is Burkitt lymphoma associated with the MYC gene translocation?

    <p>Burkitt lymphoma involves a translocation that places the MYC gene on chromosome 8 under the control of active promoters, enhancing its expression.</p> Signup and view all the answers

    What laboratory finding is commonly associated with Burkitt lymphoma in blood smears?

    <p>Scattered 'starry sky' or 'glee cells' can be identified in peripheral blood smears.</p> Signup and view all the answers

    What condition is characterized by a decline in all three blood cell types?

    <p>Pancytopenia is characterized by a decrease in red blood cells, white blood cells, and platelets.</p> Signup and view all the answers

    In the context of therapeutic responses, what makes Burkitt lymphoma particularly noteworthy?

    <p>Burkitt lymphoma is notable for its sensitivity to specific chemotherapeutic agents and usually has an excellent prognosis.</p> Signup and view all the answers

    What role do germinal center B cells play in the pathology of certain lymphomas?

    <p>Germinal center B cells are the origin of specific lymphomas, including Burkitt lymphoma and follicular lymphoma.</p> Signup and view all the answers

    What is a common observable feature in the spleen of patients with lymphomas?

    <p>The spleen is usually enlarged, a condition known as splenomegaly.</p> Signup and view all the answers

    How does the presence of MYC in Burkitt lymphoma indicate its clinical implications?

    <p>The presence of MYC indicates a high proliferation rate, suggesting a more aggressive disease with specific treatment needs.</p> Signup and view all the answers

    What are the effects of proliferating plasma cells on the skeleton?

    <p>Proliferating plasma cells have deleterious effects on the skeleton, leading to bone resorption and structural compromise.</p> Signup and view all the answers

    How do Reed-Sternberg cells appear in relation to the immune system?

    <p>Reed-Sternberg cells resemble those of the immune system and indicate the presence of Hodgkin lymphoma.</p> Signup and view all the answers

    What type of cellular composition is seen in the mixed cellularity subtype of lymphoma?

    <p>The mixed cellularity subtype features a diverse population of inflammatory cells alongside Reed-Sternberg cells.</p> Signup and view all the answers

    In what locations can Reed-Sternberg cells contribute to morbidity?

    <p>Reed-Sternberg cells can contribute to morbidity in lymph nodes, spleen, and other lymphatic tissues.</p> Signup and view all the answers

    What is a characteristic feature of the neoplastic cells in terms of morphology?

    <p>Neoplastic cells often show atypical nuclear features, such as enlarged nuclei and irregular shapes.</p> Signup and view all the answers

    What role does the immune system play in the context of Reed-Sternberg cells?

    <p>Reed-Sternberg cells interact with the immune system, often evoking a dysfunctional immune response.</p> Signup and view all the answers

    Explain why, despite the understanding that Hodgkin lymphoma is a tumor of B cells, it is still classified separately from non-Hodgkin lymphomas.

    <p>Hodgkin lymphomas are classified separately due to the presence of unique Reed-Sternberg cells, which have a distinct morphology and comprise only a small fraction of the tumor cells. Additionally, Hodgkin lymphomas exhibit a unique biology and response to therapy, further distinguishing them from non-Hodgkin lymphomas.</p> Signup and view all the answers

    Describe the significance of the nodular sclerosis type of Hodgkin lymphoma in terms of the structure of lymph nodes.

    <p>The nodular sclerosis type of Hodgkin lymphoma is characterized by well-defined bands of acellular collagen, which divide the tumor cells into nodules within the lymph node. These collagen bands are a hallmark of this specific subtype and contribute to its distinct histological appearance.</p> Signup and view all the answers

    Explain why, even with the advancement in understanding the origin of Hodgkin lymphoma, the need for distinct classification persists.

    <p>Despite the understanding of Hodgkin lymphoma as a B-cell tumor, it is still differentiated from other B-cell lymphomas because of its unique biology, response to therapy, and the presence of characteristic Reed-Sternberg cells. These distinct features warrant its separate classification.</p> Signup and view all the answers

    Describe the role of acellular collagen in the histological appearance of the nodular sclerosis type of Hodgkin lymphoma.

    <p>Acellular collagen forms well-defined bands within the lymph node in the nodular sclerosis type, creating a distinct appearance with tumor cells compartmentalized into nodules. This collagenous structure is crucial in differentiating this subtype from other Hodgkin lymphomas.</p> Signup and view all the answers

    Explain the clinical significance of recognizing the five subtypes of Hodgkin lymphoma.

    <p>Recognizing the five subtypes of Hodgkin lymphoma is crucial as each subtype has distinct biological characteristics, therapeutic responses, and prognostic implications. This knowledge allows for tailored treatment strategies and accurate prediction of disease outcomes.</p> Signup and view all the answers

    Explain why despite the current understanding of Hodgkin lymphoma as a B-cell tumor, it is still classified separately from non-Hodgkin lymphomas.

    <p>Hodgkin lymphoma, despite its B-cell origin, is classified separately due to the presence of unique Reed-Sternberg cells, which have a distinct morphology and comprise only a small fraction of the tumor cells. Additionally, Hodgkin lymphoma exhibits unique biology and response to therapy, further differentiating it from non-Hodgkin lymphomas.</p> Signup and view all the answers

    Describe the significance of the five subtypes of Hodgkin lymphoma in relation to treatment and prognosis.

    <p>Recognizing the five subtypes of Hodgkin lymphoma is essential for personalized treatment strategies and accurate prediction of disease outcomes. Each subtype exhibits distinct biological characteristics, therapeutic responses, and prognostic implications, influencing the choice of treatment and patient prognosis.</p> Signup and view all the answers

    Explain how the nodular sclerosis type of Hodgkin lymphoma differs from other subtypes in terms of its histological appearance and clinical significance.

    <p>The nodular sclerosis type is characterized by well-defined bands of acellular collagen, creating a distinct histological appearance with tumor cells compartmentalized into nodules. This subtype often has a more favorable prognosis compared to other subtypes, influencing treatment decisions and patient outcomes.</p> Signup and view all the answers

    Mycosfungoides and Sézary syndrome are tumors of neoplastic ______ T cells.

    <p>CD4+</p> Signup and view all the answers

    Mycosfungoides usually manifests as a rash that progresses over ______ to plaques and cutaneous tumors.

    <p>time</p> Signup and view all the answers

    Sézary syndrome is characterized by a generalized ______ erythroderma and the presence of tumor cells (Sézary cells) in the peripheral blood.

    <p>exfoliative</p> Signup and view all the answers

    Adult T-cell leukemia/lymphoma (ATL) is a neoplasm of ______ T cells caused by a retrovirus.

    <p>CD4+</p> Signup and view all the answers

    Adult T-cell leukemia/lymphoma (ATL) is caused by a retrovirus called ______ type 1 (HTLV-1).

    <p>Human T-cell</p> Signup and view all the answers

    Patients diagnosed with early-phase mycosfungoides often ______ for many years.

    <p>survive</p> Signup and view all the answers

    Patients with tumor-phase disease, disseminated disease, or Sézary syndrome survive on average ______ years.

    <p>1 to 3</p> Signup and view all the answers

    Adult T-cell leukemia/lymphoma (ATL) is a type of ______ caused by a retrovirus.

    <p>neoplasm</p> Signup and view all the answers

    The pathogenesis of lymphomas often arises in tissues that are ______ due to autoimmune disorders.

    <p>inflamed</p> Signup and view all the answers

    Approximately 30% of tumors have a (14;18) ______.

    <p>translocation</p> Signup and view all the answers

    The BCL2 gene is often involved in lymphomas, resulting in ______ of the anti-apoptotic protein.

    <p>overexpression</p> Signup and view all the answers

    Some lymphomas may represent "transformed" ______ cells.

    <p>B</p> Signup and view all the answers

    Eradication of H. pylori with antibiotic therapy often leads to ______ lymphomas.

    <p>nodular</p> Signup and view all the answers

    Nodular lymphomas virtually always have the (14;18) ______.

    <p>translocation</p> Signup and view all the answers

    Helicobacter pylori is associated with ______ marginal zone lymphoma of the stomach.

    <p>extranodal</p> Signup and view all the answers

    The ______ gene is commonly involved in Burkitt lymphoma, leading to uncontrolled cell growth.

    <p>MYC</p> Signup and view all the answers

    The most common subtypes of classical Hodgkin lymphoma are nodular sclerosis Hodgkin lymphoma, mixed cellularity Hodgkin lymphoma, lymphocyte-rich Hodgkin lymphoma, and ______ predominant Hodgkin lymphoma.

    <p>lymphocyte</p> Signup and view all the answers

    The ______ cells in Hodgkin lymphoma share certain morphologic and immunophenotypic features.

    <p>Reed-Sternberg</p> Signup and view all the answers

    Nodular sclerosis Hodgkin lymphoma often involves the ______ .

    <p>mediastinum</p> Signup and view all the answers

    Hodgkin lymphoma is most commonly diagnosed in ______ or young adults.

    <p>adolescents</p> Signup and view all the answers

    The ______ of Hodgkin lymphoma subtypes is not completely understood.

    <p>pathogenesis</p> Signup and view all the answers

    The ______ response to Reed-Sternberg cells defines the different subtypes of classical Hodgkin lymphoma.

    <p>tissue</p> Signup and view all the answers

    The Reed-Sternberg variants are grouped together under the rubric of classical ______ .

    <p>Hodgkin</p> Signup and view all the answers

    Hodgkin lymphoma is a type of ______ .

    <p>lymphoma</p> Signup and view all the answers

    Lymph ___ are affected by tumor cells growing.

    <p>nodes</p> Signup and view all the answers

    The tumor cells usually are significantly larger than normal ____cytes.

    <p>lymph</p> Signup and view all the answers

    The tumor cells express surface IgM and IgD, the B-cell ____ CD20.

    <p>antigen</p> Signup and view all the answers

    The bone marrow is involved in most cases and the peripheral ____ in about 20% of cases.

    <p>blood</p> Signup and view all the answers

    Tumor cells have irregular nuclei and inconspicuous ____.

    <p>nucleoli</p> Signup and view all the answers

    The findings are diagnostically ____ for clinical features.

    <p>relevant</p> Signup and view all the answers

    The tumor cells have high levels of cyclin D1 protein, a finding that is diagnostically _____.

    <p>significant</p> Signup and view all the answers

    In the context of hematopoietic and lymphoid disorders, the presence of ____ is significant.

    <p>TP53</p> Signup and view all the answers

    Langerhans cell histiocytosis is associated with ______ mutations that activate the serine/threonine kinase BRAF.

    <p>driver</p> Signup and view all the answers

    BRAF is a component of the ______ signaling pathway that supports cellular proliferation and survival.

    <p>RAS</p> Signup and view all the answers

    Tumors without BRAF mutations often have mutations in a different serine/threonine kinase called ______ that acts downstream of BRAF.

    <p>MAP2K1</p> Signup and view all the answers

    Proliferating Langerhans cells have abundant ______ and vesicular nuclei.

    <p>cytoplasm</p> Signup and view all the answers

    Langerhans cells resemble ______, also known as histiocytes.

    <p>tissue</p> Signup and view all the answers

    Numerous reactive ______ are often admixed with Langerhans cells.

    <p>eosinophils</p> Signup and view all the answers

    The term "histiocytosis" comes from the resemblance of Langerhans cells to ______.

    <p>histiocytes</p> Signup and view all the answers

    The BRAF mutations in Langerhans cell histiocytosis further implicate this pathway in the ______ of the disease.

    <p>pathogenesis</p> Signup and view all the answers

    Match the following lymphoma characteristics with their corresponding types of lymphoma:

    <p>Centrally placed nucleus, abundant pale cytoplasm = Burkitt lymphoma Extranodal marginal zone = MALT lymphoma Reed-Sternberg cells = Hodgkin lymphoma Round or multilobated nucleus, dispersed chromatin = Myeloma</p> Signup and view all the answers

    Match the following morphology with their corresponding types of lymphoma:

    <p>Monoclonal gammopathy of undetermined significance (MGUS) = Round or multilobated nucleus, dispersed chromatin Hairy cell leukemia = Nucleus with irregular shape, abundant cytoplasm DLBCL = Large, multilobated nucleus, scant cytoplasm Burkitt lymphoma = Round nucleus, abundant pale cytoplasm</p> Signup and view all the answers

    Match the following molecular features with their corresponding types of lymphoma:

    <p>t(8;14) translocation = Burkitt lymphoma Cyclin D1 overexpression = Mantle cell lymphoma MYC gene rearrangement = Myeloma BCL-2 gene rearrangement = Follicular lymphoma</p> Signup and view all the answers

    Match the following clinical features with their corresponding types of lymphoma:

    <p>Gastrointestinal symptoms = MALT lymphoma Generalized lymphadenopathy = DLBCL Skin lesions = Peripheral T-cell lymphoma Anemia, bone pain = Myeloma</p> Signup and view all the answers

    Match the following histological features with their corresponding types of lymphoma:

    <p>Follicular growth pattern = Follicular lymphoma Diffuse growth pattern = DLBCL Nodular growth pattern = Mantle cell lymphoma Monoclonal plasma cells = Myeloma</p> Signup and view all the answers

    Match the following associated diseases with their corresponding types of lymphoma:

    <p>Helicobacter pylori = MALT lymphoma Epstein-Barr virus = Burkitt lymphoma HIV = DLBCL Autoimmune disorders = Myeloma</p> Signup and view all the answers

    Match the following cell features with their corresponding types of lymphoma:

    <p>Small, mature-appearing lymphocytes = Chronic lymphocytic leukemia Large, blasts with prominent nucleoli = Burkitt lymphoma Plasma cells with eccentric nuclei = Myeloma Hairy projections on the surface = Hairy cell leukemia</p> Signup and view all the answers

    Match the following prognosis with their corresponding types of lymphoma:

    <p>Good prognosis = Follicular lymphoma Aggressive behavior = Burkitt lymphoma Indolent behavior = Mantle cell lymphoma Poor prognosis = Peripheral T-cell lymphoma</p> Signup and view all the answers

    Match the following clinical features with their corresponding association in the context of multiple myeloma:

    <p>Bence-Jones proteins = Associated with more extensive disease and a worse prognosis Low-stage disease = Often presents with fewer symptoms and a slower progression Bone marrow plasma cell infiltration = Characteristic feature of myeloma and contributes to bone lesions Hypercalcemia = Can be a consequence of bone destruction and is often seen in myeloma patients</p> Signup and view all the answers

    Match the following terms related to multiple myeloma with their correct definitions:

    <p>Monoclonal gammopathy of undetermined significance (MGUS) = An asymptomatic condition characterized by the presence of a monoclonal protein in the blood or urine Myeloma = A malignant plasma cell disorder characterized by bone marrow infiltration, monoclonal protein production, and other complications Plasma cell = A type of white blood cell responsible for producing antibodies Bence-Jones protein = A type of abnormal protein found in the urine of some myeloma patients</p> Signup and view all the answers

    Match the following clinical manifestations of myeloma with their associated consequences:

    <p>Bone lesions = Can lead to pain, fractures, and hypercalcemia Renal failure = Often due to the accumulation of light chains in the kidneys Anemia = Can result from suppression of normal hematopoiesis in the bone marrow Increased risk of infections = Caused by the impairment of the immune system due to the overproduction of abnormal antibodies</p> Signup and view all the answers

    Match the following factors associated with myeloma with their impact on prognosis:

    <p>Younger age at diagnosis = Generally associated with a more favorable prognosis More extensive disease = Often indicates a poorer prognosis Presence of high-risk genetic abnormalities = Can contribute to a higher risk of relapse and poorer outcome Response to treatment = A major factor influencing the duration of remission and overall survival</p> Signup and view all the answers

    Match the following clinical features of lymphoma with their corresponding type of lymphoma:

    <p>Burkitt lymphoma = Characterized by a rapidly growing tumor, often involving the jaw or abdomen Hodgkin lymphoma = Defined by the presence of Reed-Sternberg cells in lymph node biopsies Peripheral T-cell lymphoma = A heterogeneous group of lymphomas with varying clinical presentations Extranodal marginal zone lymphoma = Often associated with chronic inflammation or infection, particularly in the stomach</p> Signup and view all the answers

    Match the following characteristics with their corresponding type of lymphoma:

    <p>Reed-Sternberg cells = Characteristic of Hodgkin lymphoma Small B cells = Commonly seen in chronic lymphocytic leukemia (CLL) Large, atypical cells with prominent nucleoli = Characteristic of Burkitt lymphoma Involvement of peripheral T cells = Typically seen in peripheral T-cell lymphoma</p> Signup and view all the answers

    Match the following genetic alterations with their associated lymphoma types:

    <p>MYC gene translocation = Associated with Burkitt lymphoma, leading to uncontrolled cell growth BCL2 gene translocation = Commonly found in follicular lymphoma, contributing to resistance to apoptosis TP53 gene mutation = Often associated with aggressive lymphomas and poorer prognosis IGH gene translocation = Frequently seen in multiple myeloma, involving the immunoglobulin heavy chain locus</p> Signup and view all the answers

    Match the following clinical presentations with their corresponding lymphoma subtypes:

    <p>Painless, enlarged lymph nodes = Often seen in indolent lymphomas, such as follicular lymphoma Rapidly growing tumor mass = Typical of aggressive lymphomas, such as Burkitt lymphoma Fever, night sweats, and weight loss = Known as B symptoms, commonly associated with Hodgkin lymphoma Involvement of extranodal sites = Can occur in various lymphoma types, depending on the specific subtype</p> Signup and view all the answers

    Match the following lymphoma types with their associated characteristics:

    <p>Follicular lymphoma = Strongly associated with (14;18) translocation Burkitt lymphoma = Characterized by MYC translocation Chronic lymphocytic leukemia = Typically involves small B cells Diffuse large B-cell lymphoma = Common site of bone marrow involvement</p> Signup and view all the answers

    Match the following cellular markers with their relevance in lymphomas:

    <p>CD20 = Surface marker on B cells BCL2 = Anti-apoptotic protein overexpressed in certain lymphomas Germinal center B cells = Normal counterpart in follicular lymphoma Immunoglobulin = Surface expression in neoplastic B cells</p> Signup and view all the answers

    Match the following subtypes of Hodgkin lymphoma with their characteristics:

    <p>Nodular sclerosis = Well-defined bands of pink, acellular collagen Mixed cellularity = Variable presence of Reed-Sternberg cells Lymphocyte-rich = Few Reed-Sternberg cells and abundant lymphocytes Lymphocyte-depleted = Infrequent Reed-Sternberg cells in a background of collagen</p> Signup and view all the answers

    Match the following terms related to Reed-Sternberg cells with their descriptions:

    <p>Diagnostic criteria = Presence of unique bilobed nuclei Morphological feature = Abundant cytoplasm and prominent nucleoli Functional role = B-cell derived atypical giant cells Histological distinction = Sparse surrounding lymphocytes</p> Signup and view all the answers

    Match the following clinical features with their respective lymphomas:

    <p>Follicular lymphoma = Painless generalized lymphadenopathy Myeloma = Defects in humoral immunity Hodgkin lymphoma = Presence of Reed-Sternberg cells Peripheral T-cell lymphoma = Symptoms from tumor-derived inflammatory products</p> Signup and view all the answers

    Match the following genetic alterations with their corresponding conditions:

    <p>(14;18) translocation = Follicular lymphoma MYC gene translocation = Burkitt lymphoma Cyclin D1 gene alteration = Associated with myeloma TP53 mutations = Worse prognosis in various lymphomas</p> Signup and view all the answers

    Match the following factors with their impact on Hodgkin lymphoma prognosis:

    <p>Age = Younger patients typically have a better prognosis Stage at diagnosis = Advanced stages correlate with worse outcomes Histological subtype = Nodular sclerosis often has a favorable prognosis B symptoms = Presence may indicate poorer prognosis</p> Signup and view all the answers

    Match the following age-related characteristics with the corresponding lymphomas:

    <p>Follicular lymphoma = Affects adults usually older than 50 Burkitt lymphoma = Typically affects younger individuals Chronic lymphocytic leukemia = Most common in older adults Diffuse large B-cell lymphoma = Common in all age groups</p> Signup and view all the answers

    Match the following treatment modalities for Hodgkin lymphoma with their indications:

    <p>Radiation therapy = Often used in early-stage favorable cases Chemotherapy = Standard treatment for advanced stages Stem cell transplantation = Considered for relapsed or refractory cases Immunotherapy = Emerging option for recurrent Hodgkin lymphoma</p> Signup and view all the answers

    Match the following populations with their incidence of certain lymphomas:

    <p>Asians = Less frequent occurrence of follicular lymphoma Older adults = Higher prevalence of monoclonal gammopathy Children = More likely to develop Burkitt lymphoma General population = Typical cases of diffuse large B-cell lymphoma</p> Signup and view all the answers

    Match the following findings with their corresponding diagnostic techniques for Hodgkin lymphoma:

    <p>Lymph node biopsy = Gold standard for diagnosis Imaging studies = Used to assess staging and spread Flow cytometry = Can help characterize Reed-Sternberg cells PET scan = Used to evaluate treatment response</p> Signup and view all the answers

    Match the following symptoms with their corresponding lymphoma:

    <p>Painless lymphadenopathy = Follicular lymphoma Increased risk of infections = Myeloma Constitutional B symptoms = Hodgkin lymphoma Cytopenias = Peripheral T-cell lymphoma</p> Signup and view all the answers

    Match the following symptoms with their association in Hodgkin lymphoma:

    <p>Fever = A sign of systemic involvement Night sweats = Often reported by patients Weight loss = May indicate disease progression Pruritis = Commonly experienced by patients</p> Signup and view all the answers

    Match the following lymphoma subtypes with their respective features:

    <p>Chronic lymphocytic leukemia = Often involves peripheral blood Hodgkin lymphoma = Characterized by Reed-Sternberg cells Non-Hodgkin lymphoma = Includes follicular and diffuse types Extranodal marginal zone lymphoma = Often associated with Helicobacter pylori</p> Signup and view all the answers

    Match the following risk factors with their relation to Hodgkin lymphoma:

    <p>Family history = Increased risk for first-degree relatives Previous Epstein-Barr virus infection = Associated with a higher incidence Age at diagnosis = Commonly affects young adults Gender = Males have a slightly higher incidence</p> Signup and view all the answers

    Match the following clinical features with their implications for Hodgkin lymphoma management:

    <p>Enlarged lymph nodes = Often a sign of treatment response Spleen involvement = May alter treatment strategy Persistent symptoms = Indicate potential treatment failure Extra-nodal spread = Generally indicates advanced disease</p> Signup and view all the answers

    Match the following characteristics of Langerhans cell histiocytosis with their descriptions:

    <p>Unifocal unifocal disease = Proliferation confined to a single site in one organ system Common sites = Bone, soft tissues, lung, skin, or gut involvement Symptoms = Asymptomatic or may cause pain, tenderness, and pathological fractures Cure methods = Local excision or irradiation deemed effective</p> Signup and view all the answers

    Match the following clinical features of Langerhans cell histiocytosis with their implications:

    <p>CD1a positive = Indicates the presence of Langerhans cells Asymptomatic condition = No noticeable symptoms present Pain and tenderness = Possible manifestation of disease Pathological fractures = Result of bone involvement in disease</p> Signup and view all the answers

    Match the following terms with their definitions related to Langerhans cell histiocytosis:

    <p>Langerhans cells = Unique dendritic cells capable of being identified through specific staining methods Histiocytosis = Pathological condition characterized by an abnormal proliferation of histiocytes Proliferation = Increase in the number of cells due to uncontrolled growth Skeletal involvement = A common occurrence where bones are affected by the disease</p> Signup and view all the answers

    Match the following types of Langerhans cell histiocytosis with their characteristics:

    <p>Localized disease = Proliferation usually confined to a single site Systemic disease = Involvement of multiple organs in the proliferative process Bone lesions = Represent the most commonly affected area in Langerhans cell histiocytosis Soft tissue lesions = Can also be found in areas like skin and lung</p> Signup and view all the answers

    Match the following treatment approaches for Langerhans cell histiocytosis with their descriptions:

    <p>Local excision = Surgical removal of the affected area Irradiation = Use of radiation therapy to target affected tissues Observation = Monitoring the condition without immediate intervention Chemotherapy = Utilizes drugs to manage systemic cases</p> Signup and view all the answers

    Match the following potential complications of Langerhans cell histiocytosis with their manifestations:

    <p>Pathological fractures = Fractures resulting from the weakening of bone structure Pain = Localized discomfort due to tissue involvement Tenderness = Increased sensitivity in the affected area Asymptomatic progression = Development of disease without observable symptoms</p> Signup and view all the answers

    Match the following forms of treatment for unifocal unisystem disease with their expected outcomes:

    <p>Local excision = Surgical intervention aiming for complete removal Irradiation = Reduces tumor burden through targeted radiation Observation = May lead to spontaneous regression in some cases Combination therapy = Utilizes multiple modalities for comprehensive care</p> Signup and view all the answers

    Match the following types of systemic involvement in Langerhans cell histiocytosis with typical characteristics:

    <p>Bone involvement = Commonly presents with aggressive symptoms in localized areas Soft tissue involvement = Can lead to aesthetic or functional issues depending on location Lung involvement = May result in respiratory complications Gastrointestinal involvement = Can provoke symptoms related to digestive dysfunction</p> Signup and view all the answers

    Study Notes

    Hematopoietic and Lymphoid Systems

    • B-cell receptor signaling and mutations in TP53 correlate with a worse prognosis in hematological malignancies.
    • Lymph nodes typically become effaced due to nodular proliferation, which is a key characteristic in diagnoses.
    • Hematopoietic stem cell transplantation is preferred for younger patients over conventional therapies.
    • Centrocytes are larger neoplastic cells, displaying unique nuclear features, surface immunoglobulin IgM, and a variable amount of cytoplasm.

    Clinical Features

    • Marginal zone lymphomas may present with central nuclei and abundant pale or basophilic cytoplasm.
    • Tumors can also cause swelling in glands such as salivary, thyroid, or orbital regions.
    • Anaplastic tumor cells may be present and associated with H. pylori-induced gastritis.

    Burkitt Lymphoma

    • Characterized by translocations involving the MYC gene leading to overexpression of MYC protein.
    • Burkitt lymphoma is endemic in certain African regions but can be sporadic, with cases in other geographic areas including the United States.
    • Associated with unique morphologies, such as a 'starry sky' appearance due to macrophages in the tumor.
    • Myeloma involves defects in humoral immunity, increasing infection risk.
    • Cells may resemble popped corn (popcorn cells) with distinctive morphology.
    • The presence of Reed-Sternberg cells, characterized by large multinucleated nuclei, is indicative of specific lymphomas.

    Monoclonal Gammopathy of Undetermined Significance (MGUS)

    • Commonly observed in older adults without evident symptoms; characterized by monoclonal immunoglobulin presence.
    • Associated with chromosomal translocations, particularly involving the IGH locus on chromosome 14 and proto-oncogenes.
    • MGUS may progress to more severe conditions like multiple myeloma, thus warranting monitoring.

    Extranodal Lymphomas

    • Extranodal lymphomas are prevalent, with gastrointestinal tract tumors being the most common type.
    • Both endemic and non-endemic forms of lymphomas can affect almost any organ or tissue.
    • DLBCL (Diffuse Large B-cell Lymphoma) is aggressive and can rapidly progress if untreated, often manifesting as maxillary or mandibular masses.
    • In North America, other common sites include the bowel, retroperitoneum, and ovaries.
    • Complete remission is achievable in 60% to 80% of patients with intensive combination chemotherapy and anti-CD20 immunotherapy.
    • Approximately 30% of childhood non-Hodgkin lymphomas present with liver and spleen involvement.
    • Burkitt lymphoma is notably aggressive, with 50% of patients remaining disease-free after treatment.

    Peripheral T-cell Lymphoma

    • Peripheral T-cell lymphoma encompasses a heterogeneous group, making up about 10% of non-Hodgkin lymphomas.
    • These tumors are generally aggressive and respond poorly to therapy, often causing tumor-derived inflammatory symptoms.
    • Median survival time is typically only about 8 months due to the aggressive nature of these lymphomas.

    Hodgkin Lymphoma

    • Characterized by the presence of Reed-Sternberg cells and variants, which are distinct giant tumor cells.
    • These lymphomas exhibit significant variation in appearance and behavior compared to other lymphoma types.
    • The tumors can negatively impact the immune system and bone health.

    Monoclonal Gammopathy of Undetermined Significance (MGUS)

    • MGUS is common among older adults, often characterized by the presence of monoclonal immunoglobulins without significant symptoms.
    • It can be associated with chromosomal translocations, notably involving the IGH locus on chromosome 14.

    Langerhans Cell Histiocytosis

    • Langerhans cells are identified by the unique protein Langerin and express CD1a.
    • The most prevalent subtype is unifocal unisystem disease, typically localized to one organ system, often affecting bone or soft tissues.
    • Symptoms may include pain or tenderness, and it can cause pathological fractures. If localized, it can be cured through excision or radiotherapy.

    CLL/SLL Overview

    • CLL (Chronic Lymphocytic Leukemia) and SLL (Small Lymphocytic Lymphoma) exhibit resting lymphocytes with distinctive "cleaved" nuclei.
    • Transformation to aggressive tumors can occur, leading to appearances similar to diffuse large B-cell lymphoma.

    Histological Features

    • Key histologic findings include:
      • Coarse condensed chromatin and prominent nucleoli.
      • Centrocytes mixed with centroblasts and varying amounts of cytoplasm.
    • Centroblast predominance correlates with more aggressive clinical behavior in Follicular Lymphoma.

    Pathogenetic Insights

    • Mutations in serine/threonine kinase BRAF impact cell signaling and can lead to oncogenic processes.
    • Disease is progressive if untreated, associated with aggressive growth patterns in Burkitt Lymphoma and CLL/SLL.

    Prognosis and Survival

    • Median survival time post-transformation is less than one year, indicating a serious prognosis.
    • CLL/SLL typically shows a relatively good prognosis compared to other lymphomas, especially when treated early.

    Hodgkin Lymphoma Distinction

    • Hodgkin Lymphoma characterized by Reed-Sternberg cells, accounting for a small faction of aggregate tumor cells.
    • Different subtypes exist, including:
      • Nodular Sclerosis, Mixed Cellularity.
    • Historical perspectives viewed Hodgkin Lymphoma differently due to unique cell features and reaction to therapy.

    Clinical Presentation and Diagnosis

    • Includes splenomegaly and possible pancytopenia in more than half of cases.
    • Diagnosis frequently requires differentiation from non-Hodgkin Lymphomas, given shared symptoms and characteristics.

    Rare Transformations and Cases

    • Transformation to symptomatic neoplasms occurs in approximately 1% of cases, typically linked with more aggressive disease phenotypes.
    • Rare presentations include highly malignant neoplasms like rhabdomyosarcomas, contrasting with benign reactive hyperplastic processes.

    Cytokine Influence

    • Interleukin-6 (IL-6) plays a significant role in the proliferation of myeloma cells, impacting disease progression.

    Note on Morphology

    • Tumors in Hodgkin Lymphoma show defined collagen bands and comprised structures, important for histopathological classification.

    Lymph Nodes and Tumor Infiltration

    • Lymph nodes can be effaced by tumor cells, which exhibit diffuse or vaguely nodular growth patterns.
    • Tumor cells tend to be significantly larger than normal lymphocytes, displaying irregular nuclei and inconspicuous nucleoli, along with scant cytoplasm.
    • Bone marrow involvement is common in most cases, with peripheral blood impacted in around 20% of cases.

    Tumor Cell Markers

    • Tumor cells often express surface immunoglobulins IgM and IgD, as well as B-cell antigen markers CD20 and CD5.
    • High levels of cyclin D1 protein are indicative of certain tumors and can serve as a diagnostic marker.

    Clinical Features and Pathogenesis

    • Tumors can arise in tissues affected by autoimmune disorders (e.g., Sjögren syndrome) or chronic infections (e.g., Helicobacter pylori).
    • About 30% of tumors may have a (14;18) translocation involving the BCL2 gene, leading to overexpression of the anti-apoptotic BCL2 protein.

    Mycosis Fungoides and Sézary Syndrome

    • Mycosis fungoides and Sézary syndrome involve neoplastic CD4+ T cells, primarily located in the skin.
    • Mycosis fungoides manifests as a rash that progresses to plaques and cutaneous tumors, potentially leading to systemic dissemination.
    • Sézary syndrome is characterized by generalized exfoliative erythroderma and the presence of Sézary cells in the peripheral blood.
    • Patients with early-phase mycosis fungoides can have prolonged survival, while those with advanced disease or Sézary syndrome have a median survival of 1 to 3 years.

    Adult T-Cell Leukemia/Lymphoma (ATL)

    • ATL is a neoplasm of CD4+ T cells caused by Human T-Cell Leukemia Virus type 1 (HTLV-1).
    • The disease has multiple subtypes, including sclerotic, mixed cellularity, lymphocyte-rich, lymphocyte-depleted, and lymphocyte-predominant forms, each with distinct morphologic and immunophenotypic features.

    Hodgkin Lymphoma

    • Classical Hodgkin lymphoma is marked by Reed-Sternberg cells and is categorized into several subtypes.
    • The most common subtype, nodular sclerosis Hodgkin lymphoma, frequently involves the mediastinum.
    • Pathogenesis can involve various genetic factors and mutations, particularly those affecting BRAF, a part of the RAS signaling pathway critical for cell proliferation and survival.

    Langerhans Cell Histiocytosis

    • Involves proliferating Langerhans cells which can resemble macrophages, leading to the term histiocytosis.
    • Exhibits reactive eosinophilia and characterized by an abundance of cytoplasm and vesicular nuclei in neoplastic Langerhans cells.

    Follicular Lymphoma

    • Characterized by a (14;18) chromosomal translocation that increases BCL2 gene expression, linked to reduced apoptosis.
    • Approximately 25% of adult non-Hodgkin lymphoma cases in the United States are follicular lymphoma.
    • Associated with germinal center B cells and expresses CD20 and surface immunoglobulin (typically IgM).

    Clinical Features

    • Primarily affects adults over the age of 50, usually presenting as painless generalized lymphadenopathy.
    • Common morphological features include dispersed B cells with oval nuclei and varying amounts of pale cytoplasm.
    • Cells may exhibit vesicular nuclei, with one or two prominent nucleoli.

    Pathogenesis

    • More than 85% of follicular lymphomas exhibit nodular patterns.
    • Predominantly occurs in populations less frequently in Asian demographics.
    • Lymphoid tissues involved may include the spleen, marrow, and liver, with anaplasia observed in some cases.

    Distinction from Hodgkin Lymphoma

    • Hodgkin lymphoma differs from non-Hodgkin lymphoma primarily by the presence of Reed-Sternberg cells.
    • These cells visually appear distinct and constitute a small fraction of tumor cells, leading to historical differentiation of these two lymphoma groups.

    General Prognosis

    • Prognosis often varies based on subtype; younger patients typically experience better outcomes.
    • Staging and response to therapy impact patient survival and disease progression.
    • Clinicians assess lymphoid masses by considering associated symptoms such as fever, weight loss, and night sweats.

    Langerhans Cell Histiocytosis

    • Characterized by Langerhans cells expressing specific markers such as langerin (CD207) and CD1a.
    • Can be classified into systemic and unifocal forms, with the unifocal presentation usually involving a specific organ, most often bone.

    Treatment Considerations

    • Treatment options may include localized excision or irradiation, particularly for unifocal disease.
    • Responds to therapies tailored to specific lymphoma types, emphasizing the importance of accurate diagnosis and staging for optimal outcomes.

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    Robbins Essential Pathology PDF, Hematopoietic and Lymphoid Systems

    Description

    This quiz covers the hematopoietic and lymphoid systems, including B-cell receptor signaling, BCL2 antagonists, and TP53 presence. It also discusses lymph node morphology.

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