Medicine Marrow Pg 141-150 (Hematology)
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Medicine Marrow Pg 141-150 (Hematology)

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Which treatment regimen is considered the treatment of choice for fit patients with Hairy Cell Leukemia?

  • FCR regimen (correct)
  • Chemotherapy alone
  • Radiation therapy
  • BR regimen

    • Patients with Hairy Cell Leukemia are primarily affected in the age group of 30-50 years.

    False

    What is the primary cell type from which Hairy Cell Leukemia originates?

    Memory B cells

    In Hairy Cell Leukemia, patients may experience __________ due to bone marrow infiltration.

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

    Match the following prognostic factors with their associated outcomes:

    <p>Hypermutated Ig heavy chain = Good prognosis Chromosome 13q deletion = Good prognosis All other chromosome deletions = Bad prognosis Increased beta microglobulin = Bad prognosis</p> Signup and view all the answers

    Which organism is associated with causing MALTOMA?

    <p>H.pylori</p> Signup and view all the answers

    Stage II lymphoma involves lymph node regions on both sides of the diaphragm.

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

    What are the four stages of lymphoma according to the Ann-Arbor staging system?

    <p>Stage I, Stage II, Stage III, Stage IV</p> Signup and view all the answers

    ____ is the causative organism associated with Adult T-cell leukemia/lymphoma.

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

    Match the following lymphomas with their causative organisms:

    <p>Burkitt's lymphoma = EBV DLBCL = HIV Primary effusion lymphoma = HHV-8 Waldenstrom's macroglobulinemia = HCV</p> Signup and view all the answers

    What is the most common type of marginal zone lymphoma (MZL)?

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

    Richter transformation occurs in 5% of patients with low-grade lymphoma per year.

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

    What is the treatment approach for symptomatic cases of Non-Hodgkin's lymphoma with a low tumor burden?

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

    The primary association of Maltoma is with ______ in 95% of cases.

    <p>H.pylori</p> Signup and view all the answers

    Match the following clinical features to their respective lymphomas:

    <p>Follicular lymphoma = Small cleaved cells, large non-cleaved cells Burkitt's lymphoma = Aggressive centroblast DLBCL = High-grade lymphoma Maltoma = Upper GI symptoms</p> Signup and view all the answers

    What is the most common presenting feature of Mantle Cell Lymphoma?

    <p>Asymptomatic painless lymphadenopathy</p> Signup and view all the answers

    Mantle Cell Lymphoma primarily affects younger individuals.

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

    Which genetic marker is commonly associated with Mantle Cell Lymphoma?

    <p>t(11;14)</p> Signup and view all the answers

    Mantle Cell Lymphoma is classified as a ___ malignancy originating from pre-germinal centers.

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

    Match the following features with their associated type of lymphoma:

    <p>CD10(+) = Follicular Lymphoma SOX-11 positive = Classical Mantle Cell Lymphoma BCL-2 expression = Follicular Lymphoma SOX-11 negative = Leukemic Non-nodal Mantle Cell Lymphoma</p> Signup and view all the answers

    Which immunophenotyping markers are positive for Hairy Cell Leukemia?

    <p>CD19, CD20, CD25</p> Signup and view all the answers

    Fried egg appearance is a characteristic associated with Hairy Cell Leukemia bone marrow biopsy findings.

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

    What is the primary treatment option for Hairy Cell Leukemia?

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

    In Hairy Cell Leukemia, patients may exhibit __________ on a peripheral smear.

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

    Match the following treatments with their descriptions:

    <p>Cladribine = Purine nucleoside analogue Vemurafenib = BRAF inhibitor Rituximab = Maintenance therapy Pentostatin = Alternative purine analogue</p> Signup and view all the answers

    What is a common blood count finding in Chronic Lymphocytic Leukemia?

    <p>Lymphocytosis of 60,000-70,000</p> Signup and view all the answers

    Richter transformation is characterized by low-grade lymphoma with increased lymphadenopathy.

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

    What is the first stage of the Rai and Binet staging system for Chronic Lymphocytic Leukemia?

    <p>Stage 0</p> Signup and view all the answers

    The peripheral smear in Chronic Lymphocytic Leukemia often shows small, blue, round __________.

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

    Match the following stages of Chronic Lymphocytic Leukemia with their features:

    <p>Stage 0 = Lymphocytosis Stage 2 = Hepatosplenomegaly Stage 3 = Anemia Stage 4 = Pancytopenia</p> Signup and view all the answers

    Which marker is NOT associated with the Pre-germinal center?

    <p>CD10</p> Signup and view all the answers

    Centrocytes are the product of somatic hypermutation in the Germinal Center.

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

    Name one type of lymphoma associated with the Post-germinal cell zone.

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

    The marker _______ indicates a Plasma Cell variant associated with Multiple Myeloma.

    <p>CD138</p> Signup and view all the answers

    Match the following lymphomas with their associated markers:

    <p>Chronic Lymphocytic Leukemia (CLL) = CD5, CD10, CD23+ Mantle Cell Lymphoma (MCL) = CD5, CD10, CD23- Follicular Lymphoma = CD10, CD19, CD20, CD23 Marginal Zone Lymphoma = CD19, CD20, CD21</p> Signup and view all the answers

    Which of the following is a characteristic feature of Diffuse Large B-cell Lymphoma (DLBCL)?

    <p>Rapidly progressive, painless lymphadenopathy</p> Signup and view all the answers

    Burkitt's lymphoma is characterized by elevated LDH and hypoalbuminemia.

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

    What is a hallmark cell associated with DLBCL?

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

    Anaplastic large B-cell lymphoma is associated with the genetic alteration known as t(;).

    <p>2,5</p> Signup and view all the answers

    Match the following lymphoma types with their characteristics:

    <p>Diffuse Large B-cell Lymphoma = Painless lymphadenopathy with B-symptoms Burkitt's Lymphoma = Not specified Anaplastic Large B-cell Lymphoma = CD30+ and t(2;5)</p> Signup and view all the answers

    What is the median age of diagnosis for Chronic Lymphocytic Leukemia (CLL)?

    <p>72 years</p> Signup and view all the answers

    Chronic Lymphocytic Leukemia primarily affects females.

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

    What percentage of patients with Chronic Lymphocytic Leukemia are asymptomatic?

    <p>80-85%</p> Signup and view all the answers

    In Chronic Lymphocytic Leukemia, defective apoptosis occurs in __________ cells.

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

    Match the following features with their associated characteristics of Chronic Lymphocytic Leukemia:

    <p>CD5 = Acquired marker in CLL Median survival = 10 years Spontaneous regression = 1-2% of cases Clinical presentation = Painless lymphadenopathy</p> Signup and view all the answers

    Which of the following lymphomas is categorized as high grade?

    <p>Burkitt's lymphoma</p> Signup and view all the answers

    Anaplastic large cell lymphoma is associated with the genetic marker ALK (CD30)+.

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

    What is the most common type of Non-Hodgkin's lymphoma?

    <p>Diffuse large B-cell lymphoma (DLBCL)</p> Signup and view all the answers

    The lymphomas primarily originating from B-cells include __________ and __________.

    <p>mantle cell lymphoma (MCL), follicular lymphoma</p> Signup and view all the answers

    Match the following types of lymphomas with their corresponding cell of origin:

    <p>Burkitt's lymphoma = Centrocytes Extranodal NK T-lymphoma = Mature T-cell Follicular lymphoma = Post-germinal centre Diffuse large B-cell lymphoma = Centroblasts</p> Signup and view all the answers

    Study Notes

    Hairy Cell Leukemia

    • Rare, low-grade B cell neoplasm
    • Originates from memory B cells
    • Predominantly affects individuals over 60 years old, with males being more susceptible than females
    • Characterized by a BRAF mutation
    • Typically presents with massive splenomegaly, pancytopenia (neutropenia, monocytopenia), and fibrosis
    • Patients are prone to infections like aspergillosis, MAC, and Mycobacterium Kansasi

    Clinical Manifestations

    • *Spleen: Massive splenomegaly
    • *Bone Marrow: Pancytopenia (neutropenia, monocytopenia)
    • *Other: Fibrosis

    Treatment

    • First line treatment for fit patients is the FCR regimen (Fludarabine, Cyclophosphamide, Rituximab)
    • The BR regimen (Bendamustine, Rituximab) is more commonly used due to less side effects and easier administration

    Prognostic Factors

    • Good prognostic factors: Ig heavy chain hypermutation and Chromosome 13q deletion
    • Poor prognostic factors: Unmutated Ig heavy chain, all other chromosome deletions (11q del, 17p del, trisomy 12), increased beta microglobulin, increased LDH, ZAP-70

    Chronic Lymphocytic Leukemia (CLL)

    • Most common lymphoid leukemia, affecting mostly males
    • Typically presents as low-grade leukemia with asymptomatic cases in 80-85%
    • Median age of diagnosis is 72 years
    • Median survival is 10 years, which can be improved with medication
    • CLL can undergo spontaneous regression in 1-2% of cases.

    Pathophysiology of CLL

    • Normal Physiology: Naive B cells in the blood express CD19, CD20, CD21, CD22, CD79a, CD79b, and surface IgM, and they normally undergo apoptosis.
    • CLL Pathology: Defective apoptosis of naive B cells leads to their clonal expansion. These expanded cells acquire CD5 and CD23 expression.

    Small Lymphocytic Leukemia (SLL)

    • Arises from memory B cells.
    • Immunophenotyping is the same as CLL
    • Features are identical to CLL.
    • Monoclonal B cell lymphocytosis in a lymph node presents as painless, asymptomatic lymphadenopathy and can involve splenomegaly. If lymphocyte count exceeds 5000/µL, the diagnosis changes to CLL.

    CLL Etiology

    • Strong familial inheritance is observed
    • No association with radiation risk

    CLL Investigations

    • Blood Count: Unexplained lymphocytosis (around 60,000-70,000), occasionally normochromic anemia, and thrombocytopenia.
    • Peripheral Smear: Small, blue, round lymphocytes with pale cytoplasm, no granules, and moderate chromatin condensation
    • Immunophenotyping: Used for confirmation
    • Lymph node biopsy: Diffuse effacement of lymph node architecture (for small lymphocytic lymphoma - SLL).

    Indicators of High Risk in CLL

    • Richter Transformation: Low-grade lymphoma transforms into a high-grade lymphoma

      • Features: Increased lymphadenopathy, B symptoms (weight loss, fever, elevated LDH), increased lymph node uptake on PET scan.

    • Warm antibody AIHA association: Identified by DAT/Coombs's test.

    Rai and Binet Staging of CLL

    • Stage 0: Lymphocytosis - no treatment required
    • Stage 1: Lymphocytosis + lymphadenopathy - no treatment required
    • Stage 2: Hepatosplenomegaly - no treatment required
    • Stage 3: Anemia - treatment required
    • Stage 4: Pancytopenia - treatment required

    Indications for CLL Treatment

    • Features of Richter transformation
    • AIHA unresponsive to steroids
    • Stage 2, 3, and 4 with massive splenomegaly or marrow failure
    • Note:* The presence of a high-grade lymphoma (classically DLBCL) suggests a transformation from CLL.

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    Description

    Explore the essential aspects of Hairy Cell Leukemia, a rare B cell neoplasm primarily affecting older adults. This quiz covers its clinical manifestations, treatment options, and prognostic factors. Test your knowledge on the characteristics and management of this unique leukemia type.

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