Lymphoma Types Quiz
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Questions and Answers

What is the characteristic of M4-ACUTE MYELOMONOCYTIC LEUKEMIA in terms of monoblast percentage?

  • 10 to 20% monoblast
  • 50 to 80% monoblast
  • 30 to 50% monoblast
  • 20 to 80% monoblast (correct)

    • What is the duration of Acute leukemia?

  • Days to 6 months (correct)
  • Months to 1 year
  • Years to 2 years
  • Days to 1 month

    • What is the characteristic of M5b- ACUTE MONOBLASTIC LEUKEMIA WITH MATURATION?

  • >50% erythrocytic precursors
  • >80% monocytic cells
  • 30 % blast
  • All of the above (correct)

    • What is the term for malignancy that starts in the lymph system?

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

    What is the characteristic of M7-ACUTE MEGAKARYOCYTIC LEUKEMIA?

    <p>All of the above</p> Signup and view all the answers

    What is the characteristic of M3-ACUTE PROMYELOCYTIC LEUKEMIA?

    <p>All of the above</p> Signup and view all the answers

    What is the term for a form of cancer of the plasma cells?

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

    What is the characteristic of FAB M1?

    <p>All of the above</p> Signup and view all the answers

    What is the characteristic appearance of the tissue in CHLOROMA due to the presence of the enzyme MPO?

    <p>Green appearance</p> Signup and view all the answers

    What is the typical shape of the nuclei of myeloblasts in peripheral blood smears?

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

    Which of the following is a common initial symptom of M2?

    <p>Easy bruising</p> Signup and view all the answers

    What is the characteristic of promyelocytes in M3?

    <p>Coarsely granular</p> Signup and view all the answers

    What is the result of the balanced reciprocal translocation between chromosomes 15 and 17 in M3?

    <p>Fusion between PML gene and RARA</p> Signup and view all the answers

    What is the association of M3 with?

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

    What is the name of the type of leukemia that M3 is also referred to?

    <p>Naegeli type monocytic leukemia</p> Signup and view all the answers

    What is the characteristic of the cytoplasm of myeloblasts?

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

    What is the characteristic nuclear outline of lymphoid cells in Mantle Cell Lymphoma?

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

    Which type of lymphoma is characterized by a 'starry sky' pattern?

    <p>Burkitt Lymphoma</p> Signup and view all the answers

    What is the origin of Follicular Lymphoma?

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

    What is the characteristic of Sezary cells?

    <p>Small to medium-sized lymphoid cells with irregular nuclear outlines (cerebriform nuclei)</p> Signup and view all the answers

    What is the characteristic of the lymphoid proliferation in Mantle Cell Lymphoma?

    <p>Diffuse proliferation of monotonous, medium-sized lymphoid cells</p> Signup and view all the answers

    Which type of lymphoma affects T-lymphocytes?

    <p>Mycosis Fungoides</p> Signup and view all the answers

    What is the characteristic of the cytoplasm of lymphoid cells in Burkitt Lymphoma?

    <p>Basophilic vacuolated</p> Signup and view all the answers

    How many variants of Burkitt Lymphoma are listed in the WHO classification?

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

    What is the range of scores used to rate the intensity of staining in the LAP score calculation?

    <p>0 to 41</p> Signup and view all the answers

    What is the purpose of using two examiners to calculate the LAP score?

    <p>To reduce the subjectivity of the scoring</p> Signup and view all the answers

    What is the characteristic cytoplasmic feature of hairy cell leukemia?

    <p>Fine, 'hairy' projections</p> Signup and view all the answers

    What is the clinical finding commonly associated with hairy cell leukemia?

    <p>Fatigue, anemia, and leukocytopenia</p> Signup and view all the answers

    What is the name of the stain used to identify hairy cell leukemia?

    <p>Tartrate-resistant acid phosphatase (TRAP) stain</p> Signup and view all the answers

    What is the postulated cell of origin in hairy cell leukemia?

    <p>Peripheral B cell of post-germinal center stage</p> Signup and view all the answers

    What is the common complication of hairy cell leukemia?

    <p>Bleeding and infection</p> Signup and view all the answers

    What is the term used to describe the group of disorders that includes hairy cell leukemia?

    <p>Lymphoproliferative disorders</p> Signup and view all the answers

    What is the primary characteristic of Sézary syndrome?

    <p>Disseminated disease with leukemic presentation and skin and lymph node involvement</p> Signup and view all the answers

    What distinguishes Hodgkin's lymphoma from other lymphomas?

    <p>The presence of REED-STENBERG CELLS</p> Signup and view all the answers

    What is the primary characteristic of Nodular Lymphocyte Predominant Hodgkin?

    <p>A B-cell neoplasm composed of relatively rare neoplastic cells</p> Signup and view all the answers

    What is the purpose of the Ann Arbor classification of Hodgkin?

    <p>To determine the extent of tissue involvement</p> Signup and view all the answers

    What is the common feature of Myeloproliferative Disorders/Neoplasms?

    <p>Clonal expansion of one or more myeloid cell lines</p> Signup and view all the answers

    What is the primary characteristic of Myeloproliferative Disorders/Neoplasms?

    <p>The clonal expansion of one or more myeloid cell lines</p> Signup and view all the answers

    Which of the following is NOT a characteristic of Myeloproliferative Disorders/Neoplasms?

    <p>The presence of REED-STENBERG CELLS</p> Signup and view all the answers

    What is the relationship between Myeloproliferative Disorders/Neoplasms and acute leukemias?

    <p>Myeloproliferative Disorders/Neoplasms have the propensity to transform into acute leukemias</p> Signup and view all the answers

    What is the primary feature of leukemia that distinguishes it from other hematopoietic disorders?

    <p>Abnormal, uncontrolled proliferation and accumulation of one or more of the hematopoietic cells</p> Signup and view all the answers

    What is the common symptom of leukemia, lymphoma, and myeloma?

    <p>Fatigue and weakness</p> Signup and view all the answers

    What is the term for a disease that starts in the lymph system, mainly the lymph nodes?

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

    What is the characteristic of chronic leukemia?

    <p>1 or 2 years or more</p> Signup and view all the answers

    What is the result of the abnormal proliferation of hematopoietic cells in leukemia?

    <p>Crowding out of healthy cells in bone marrow</p> Signup and view all the answers

    Which of the following is a common feature of leukemia, lymphoma, and myeloma?

    <p>Fatigue and weakness</p> Signup and view all the answers

    What is the primary purpose of cytochemical staining in diagnosing leukemia?

    <p>To detect cellular enzymes and other chemicals in peripheral blood films</p> Signup and view all the answers

    Which of the following is NOT a method used to subdivide leukemia?

    <p>Clinical history</p> Signup and view all the answers

    What is the minimum percentage of peripheral blood and bone marrow blasts required to define acute leukemia according to the World Health Organization?

    <p>20%</p> Signup and view all the answers

    What is the primary focus of cytogenetic analysis in leukemia diagnosis?

    <p>Detecting visible chromosome abnormalities</p> Signup and view all the answers

    What is the purpose of immunophenotyping in leukemia diagnosis?

    <p>To identify cells on the basis of the types of markers or antigens present</p> Signup and view all the answers

    What is the primary difference between leukemias and other hematologic diseases?

    <p>The presence of blast cells in peripheral blood</p> Signup and view all the answers

    What is the purpose of using antibodies in the technique described?

    <p>To identify the lineage of cells</p> Signup and view all the answers

    What is the characteristic of the lymphoblasts in ALL L1?

    <p>They are small and homogeneous</p> Signup and view all the answers

    What is the characteristic of the nucleus in ALL L1?

    <p>It is round and irregular in shape</p> Signup and view all the answers

    What is the prognosis of ALL L3?

    <p>Poor prognosis</p> Signup and view all the answers

    What is the characteristic of the cytoplasm in ALL L2?

    <p>It is abundant and basophilic</p> Signup and view all the answers

    What is the range of monoblast percentage in M4-ACUTE MYELOMONOCYTIC LEUKEMIA?

    <p>20-80%</p> Signup and view all the answers

    What is the characteristic of M5B-ACUTE MONOBLASTIC LEUKEMIA WITH MATURATION?

    <p>It has &gt;80% monocytic cells</p> Signup and view all the answers

    What is the purpose of flow cytometry in the technique described?

    <p>To identify the CD markers or antigens</p> Signup and view all the answers

    What is the primary characteristic of hairy cell leukemia?

    <p>Fine (“hairy”) cytoplasmic projections</p> Signup and view all the answers

    What is the origin of Follicular Lymphoma?

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

    What is the characteristic of the lymphoid proliferation in Burkitt Lymphoma?

    <p>Diffuse pattern</p> Signup and view all the answers

    Which type of lymphoma is characterized by a 'starry sky' pattern?

    <p>Burkitt Lymphoma</p> Signup and view all the answers

    What is the characteristic of the cytoplasm of lymphoid cells in Mantle Cell Lymphoma?

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

    What is the primary characteristic of Mycosis Fungoides?

    <p>Affects T-lymphocytes</p> Signup and view all the answers

    Which type of lymphoma affects the B-cells within the lymph nodes?

    <p>Mantle Cell Lymphoma</p> Signup and view all the answers

    What is the characteristic of the cytoplasm of lymphoid cells in Burkitt Lymphoma?

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

    How many variants of Burkitt Lymphoma are listed in the WHO classification?

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

    What is the characteristic of Sezary cells?

    <p>Small to medium-sized lymphoid cells with irregular nuclear outlines</p> Signup and view all the answers

    What is the reason for the ruddy skin coloration in patients?

    <p>Increase in RBC concentration and viscosity of the blood</p> Signup and view all the answers

    What type of polycythemia is characterized by normal RBC mass, increased hematocrit, and normal EPO levels?

    <p>Relative polycythemia</p> Signup and view all the answers

    What is the term for polycythemia caused by high altitude?

    <p>Secondary absolute polycythemia</p> Signup and view all the answers

    What is the characteristic of polycythemia vera?

    <p>Increased RBC mass and hematocrit</p> Signup and view all the answers

    What is the term for a genetic disorder that causes polycythemia?

    <p>Primary familial congenital polycythemia</p> Signup and view all the answers

    What is the characteristic of secondary polycythemia with inappropriately increased EPO?

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

    What is the term for polycythemia caused by dehydration?

    <p>Spurious polycythemia</p> Signup and view all the answers

    What is the characteristic of genetic polycythemia?

    <p>Increased RBC mass and hematocrit</p> Signup and view all the answers

    What is the term for polycythemia caused by Cyanotic heart disease?

    <p>Secondary absolute polycythemia</p> Signup and view all the answers

    What is the characteristic of absolute polycythemia?

    <p>Increased RBC mass and hematocrit</p> Signup and view all the answers

    What is the primary function of the Janus kinase 2 protein in the bone marrow?

    <p>To regulate the growth and division of blood cells</p> Signup and view all the answers

    Which of the following myeloproliferative neoplasms is characterized by an absolute increase in RBC, WBC, and platelets?

    <p>Polycythemia Vera</p> Signup and view all the answers

    What is the name of the mutation that leads to the production of an abnormal JAK2 protein, promoting uncontrolled growth and division of blood cells?

    <p>JAK2V617F</p> Signup and view all the answers

    Which of the following is a characteristic feature of Myelofibrosis with Myeloid Metaplasia?

    <p>All of the above</p> Signup and view all the answers

    What is the term for the translocation of one arm of chromosome 22 to chromosome 9, characteristic of Chronic Myelogenous Leukemia?

    <p>Philadelphia chromosome</p> Signup and view all the answers

    Which of the following myeloproliferative neoplasms is characterized by thrombocytosis in excess of 1000x109/L, with spontaneous aggregation of functionally abnormal platelets?

    <p>Essential Thrombocythemia</p> Signup and view all the answers

    What is the percentage of Primary Myelofibrosis patients who have the JAK2V617F mutation?

    <p>65%</p> Signup and view all the answers

    Which of the following is a common clinical manifestation of Essential Thrombocythemia?

    <p>All of the above</p> Signup and view all the answers

    What is the name of the protein involved in the cellular signaling pathway that regulates the growth and division of blood cells in the bone marrow?

    <p>Janus kinase 2</p> Signup and view all the answers

    Which of the following myeloproliferative neoplasms is characterized by a stem cell disorder affecting the granulocytic, monocytic, erythrocytic, and megakaryocytic cell lines?

    <p>Chronic Myelogenous Leukemia</p> Signup and view all the answers

    What is the primary characteristic of myelodysplastic syndromes (MDS)?

    <p>Acquired clonal hematologic disorders</p> Signup and view all the answers

    What is the median age at diagnosis for myelodysplastic syndromes (MDS)?

    <p>70</p> Signup and view all the answers

    What is the term previously used to describe myelodysplastic syndromes (MDS)?

    <p>All of the above</p> Signup and view all the answers

    What is the characteristic of the bone marrow in myelodysplastic syndromes (MDS)?

    <p>Hypercellular bone marrow</p> Signup and view all the answers

    What are the defects present in myelodysplastic syndromes (MDS)?

    <p>Defects in erythroid, myeloid, and/or megakaryocytic maturation</p> Signup and view all the answers

    What is the traditional classification of myelodysplastic syndromes (MDS)?

    <p>FAB Cooperative Group</p> Signup and view all the answers

    Study Notes

    Lymphoproliferative Disorders

    • Mantle Cell Lymphoma: characterized by medium-sized lymphoid cells with irregular nuclear outlines, replacement of normal nodal architecture with a diffuse proliferation of monotonous, medium-sized lymphoid cells.
    • Follicular Lymphoma: originates from germinal center B cells, recapitulates follicular architecture, and is characterized by numerous closely spaced follicles replacing normal nodal architecture.
    • Burkitt Lymphoma: characterized by medium-sized, highly proliferating lymphoid cells with basophilic vacuolated cytoplasm, a diffuse lymphoid proliferation with a "starry sky" pattern, and three variants (endemic, sporadic, and immunodeficiency-associated).

    Cutaneous Lymphoma

    • Mycosis Fungoides & Sézary Syndrome: affects T lymphocytes, characterized by small to medium-sized lymphoid cells with irregular nuclear outlines (cerebriform nuclei), and peripheral blood smear and bone marrow showing predominance of myeloblasts.

    Acute Leukemia

    • M2: characterized by fatigue, symptoms of bleeding, hepatomegaly, splenomegaly, and lymphadenopathy, with myeloblasts predominant in peripheral blood smears.
    • M3 (Acute Promyelocytic Leukemia): characterized by a balanced reciprocal translocation between chromosomes 15 and 17, associated with DIC, and a severe bleeding tendency with a fatal course.
    • Duration: classified into acute (days to 6 months), subacute (2 to 6 months), and chronic (1 or 2 years or more) leukemia.

    Lymphoma

    • General: a malignancy that starts in the lymph system, mainly the lymph nodes, with two main types: Hodgkin lymphoma and non-Hodgkin lymphoma.

    Myeloma

    • A form of cancer: affecting plasma cells, characterized by overgrowth, forming a mass or tumor in the bone marrow.

    Other Lymphoproliferative Disorders

    • Hairy Cell Leukemia: characterized by small B lymphocytes with abundant cytoplasm and fine ("hairy") cytoplasmic projections, more common in males than females, with clinical findings of fatigue, anemia, leukocytopenia, thrombocytopenia, splenomegaly, and marrow fibrosis.
    • Hodgkin's Lymphoma: a malignant lymphoma distinguished by the presence of Reed-Sternberg cells, divided into classical Hodgkin and nodular lymphocyte-predominant Hodgkin.
    • Myeloproliferative Disorders/Neoplasms: characterized by excessive proliferation of one or more mature myeloid cell lines, with the propensity to transform into other MPNs or progress into acute leukemias.

    Leukemia

    • Recognized as a distinct clinical disorder by Virchow between 1839 and 1845
    • Named "leukemia" due to the white appearance of blood from patients with fever, weakness, and lymphadenopathy
    • Abnormal, uncontrolled proliferation and accumulation of one or more hematopoietic cells

    Symptoms of Leukemia

    • Fatigue and weakness
    • Increased infections
    • Bleeding and bruising
    • Anemia
    • Swollen lymph nodes
    • Enlarged spleen or liver
    • Bone pain or tenderness

    Leukemia vs Lymphoma vs Myeloma

    • Leukemia: a disease of the blood and bone marrow, characterized by overproduction of immature or mature leukocytes
    • Lymphoma: a malignancy that starts in the lymph system, mainly the lymph nodes
    • Myeloma: a form of cancer of the plasma cells, characterized by the overgrowth of cells, forming a mass or tumor in the bone marrow

    Classification of Leukemia

    • Duration: acute (days to 6 months), subacute (2 to 6 months), and chronic (1 or 2 years or more)
    • Number of white blood cells present in peripheral blood (PBS)
    • World Health Organization (WHO) classification: acute leukemia (>20% peripheral blood and bone marrow blasts)

    Subclassification of Leukemia

    • Cellular morphology
    • Cytochemical stains (cytochemistry)
    • Immunophenotyping (flow cytometry)
    • Cytogenetic abnormalities
    • Clinical syndrome

    Cytogenetic Analysis

    • Laboratory study of visible chromosome abnormalities (e.g., deletions, translocations, aneuploidy)
    • Identifies abnormalities in the number or structure of chromosomes

    Cytochemistry

    • Uses specialized stains to detect cellular enzymes and other chemicals in peripheral blood films and bone marrow aspirate smears
    • Used to differentiate hematologic diseases, especially leukemias

    Immunophenotyping (Flow Cytometry)

    • Identifies cells based on the types of markers or antigens present on the cell's surface, nucleus, or cytoplasm
    • Uses antibodies to detect markers or antigens on the cells

    Acute Lymphoblastic Leukemia (FAB Classification)

    • ALL L1: small and homogenous lymphoblasts, scanty cytoplasm, and inconspicuous nucleoli
    • ALL L2: large and heterogenous lymphoblasts, abundant cytoplasm, and nucleoli present
    • ALL L3: Burkitt-type, large and homogenous lymphoblasts, vacuolated

    Acute Myeloid Leukemia (FAB Classification)

    • MO: acute myeloid leukemia, minimally differentiated
    • M1: acute myeloid leukemia without maturation
    • M2: acute myeloid leukemia with maturation
    • M3: acute promyelocytic leukemia
    • M4: acute myelomonocytic leukemia
    • M5B: acute monoblastic leukemia with maturation

    Lymphoproliferative Disorders

    • Hairy cell leukemia: characterized by small B lymphocytes with abundant cytoplasm and fine (“hairy”) cytoplasmic projections.
    • More common in males than in females.
    • Bone marrow produces too many abnormal B-lymphocytes.
    • Cytochemical feature: strong acid phosphatase reaction that is not inhibited by tartaric acid or tartrate-resistant acid phosphatase (TRAP) stain.

    Mantle Cell Lymphoma

    • Affects B-cells within the lymph nodes, particularly the "mantle zone".
    • Subtype of non-Hodgkin lymphoma (NHL).
    • Considered fast-growing compared to other lymphomas.
    • Originates from B-cells in the lymph nodes.
    • Characterized by medium-sized lymphoid cells with irregular nuclear outlines.
    • Derived from the follicular mantle zone.

    Follicular Lymphoma

    • Originates from germinal center B cells and recapitulates follicular architecture.
    • Numerous closely spaced follicles replace the normal nodal architecture.

    Burkitt Lymphoma

    • Characterized by medium-sized, highly proliferating lymphoid cells with basophilic vacuolated cytoplasm.
    • Lymphoid proliferation is diffuse and shows a prominent “starry sky” pattern at low magnification.
    • Three variants: endemic (occurring predominantly in Africa), sporadic, and immunodeficiency-associated.

    Mycosis Fungoides & Sezary Syndrome

    • Mycosis fungoides is the most common cutaneous lymphoma.
    • Affects T lymphocytes.
    • Sezary cells: small to medium-sized lymphoid cells with irregular nuclear outlines (cerebriform nuclei).

    Myeloproliferative Disorders / Neoplasms

    • Characterized by excessive proliferation of one or more mature myeloid cell lines (granulocytes, erythrocytes, megakaryocytes, or mast cells)
    • Clonal hematopoietic stem cell disorders
    • Involve abnormal Janus kinase 2 (JAK2) protein, leading to uncontrolled blood cell growth and division

    JAK2 Gene and Mutation

    • Provides instructions for making Janus kinase 2 protein, involved in cellular signaling pathway regulating blood cell growth and division in the bone marrow
    • JAK2V617F mutation leads to abnormal JAK2 protein, promoting uncontrolled growth and division of blood cells

    Types of Myeloproliferative Neoplasms

    • Essential Thrombocythemia (ET)
    • Polycythemia Vera (PV)
    • Idiopathic Myelofibrosis (IMF)
    • Chronic Myelogenous Leukemia (CML)
    • Chronic Neutrophilic Leukemia (CNL)
    • Varies Nucleated Erythrocytes/Megakaryocytes in Myeloproliferation (VNEL)

    Chronic Myelogenous Leukemia (CML)

    • Affecting granulocytic, monocytic, erythrocytic, and megakaryocytic cell lines
    • 90% of cases involve Philadelphia chromosome (chromosome 22 translocated to chromosome 9)
    • Associated with BCR/ABL1 abnormality
    • Poorer prognosis for patients without Philadelphia chromosome

    Myelofibrosis with Myeloid Metaplasia

    • Primary Myelofibrosis (PMF)
    • Agnogenic Myelofibrosis
    • Characterized by fibrosis and granulocytic hyperplasia of the bone marrow
    • Granulocytic and megakaryocytic proliferation in the liver and spleen
    • Associated with splenomegaly, ineffective hematopoiesis, and JAK2 V617F mutation (65% of PMF patients)

    Essential Thrombocythemia (ET)

    • Chronic MPD characterized by thrombocytosis (> 1000 x 10^9/L)
    • Functionally abnormal platelets, leading to hemorrhage, platelet dysfunction, and thrombosis
    • JAK2 V617F mutation found in 50-60% of ET patients
    • Supports diagnosis of ET

    Polycythemia Vera (PV)

    • Characterized by absolute increase in RBC, WBC, and platelets
    • JAK2 V617F mutation found in 90-97% of PV patients
    • Primary absolute polycythemia, also known as polycythemia Rubra vera
    • Patients exhibit ruddy skin coloration due to increased RBC concentration and blood viscosity

    Pathophysiological Classification of Polycythemia

    • Relative Polycythemia: normal RBC mass, increased hematocrit, normal EPO, decreased plasma volume (dehydration, diarrhea, burns, shock)
    • Absolute Polycythemia:
      • Primary Absolute Polycythemia: Polycythemia Vera
      • Secondary Polycythemia with appropriately increased EPO: hypoxia, high altitudes, pulmonary disease, cyanotic heart disease, carboxyhemoglobinemia, high oxygen hemoglobinopathy, 2-3DPG deficiency
      • Secondary Polycythemia with inappropriately increased EPO: neoplasms, acute hepatitis, hepatoma, renal carcinoma, post-renal transplant, Wilms' tumor
      • Genetic Polycythemia/Congenital Secondary Polycythemia: primary familial congenital polycythemia, Chuvash polycythemia

    Myelodysplastic Syndromes (MDS)

    • Group of disorders resulting from clonal abnormalities of hematopoietic pluripotential stem cells
    • Characterized by a hypercellular bone marrow and abnormalities in the cellular maturation of erythroid cells, granulocytes, and megakaryocytes
    • Acquired clonal hematologic disorders characterized by progressive cytopenias in the peripheral blood, reflecting defects in erythroid, myeloid, and/or megakaryocytic maturation

    Historical Context

    • Historically referred to as refractory anemia, smoldering leukemia, oligoblastic leukemia, or preleukemia

    Demographics

    • Median age at diagnosis: 70

    Traditional FAB Cooperative Group Classification of MDS

    • Refractory anemia (RA): no specific characteristics mentioned
    • Note: The classification table provided is incomplete, and further information is needed to fully understand the classification of MDS.

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    Description

    Test your knowledge on Mantle Cell Lymphoma and Follicular Lymphoma, two types of lymphoproliferative disorders. Learn about their characteristics and differences.

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