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Questions and Answers
What is the most common extranodal site for diffuse large B cell lymphoma?
Which gene rearrangement is highly associated with diffuse large B cell lymphoma?
What is a key characteristic of the neoplastic B cells in diffuse large B cell lymphoma?
In which age group is mediastinal large B cell lymphoma most likely to occur?
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What is the expected prognosis of diffuse large B cell lymphoma without treatment?
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What characterizes malignant tumors of lymphoid tissue?
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Which of the following statements is true regarding Chronic Lymphocytic Leukemia (CLL)?
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What is the relevance of the t(14;18) translocation in follicular lymphomas?
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Which cells are primarily involved in the morphologic structure of follicular lymphomas?
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How does Small Lymphocytic Lymphoma (SLL) present in relation to Peripheral Blood?
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What is a common presentation of CLL?
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What does the presence of neoplastic proliferation indicate in lymphoid neoplasms?
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Which of the following accurately describes the prognosis of follicular lymphomas?
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What are the two types of cells found in follicular lymphoma?
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What marker is associated with germinal center B cells?
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Which condition is NOT commonly associated with MALT-type lymphoma?
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What age group is primarily affected by mantle cell lymphoma?
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What cytological feature can be observed in mantle cell lymphoma?
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What is the median survival rate for patients with aggressive mantle cell lymphoma?
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Which of the following is a characteristic presentation of diffuse large B-cell lymphoma (DLBL)?
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Which genetic alteration is specifically associated with mantle cell lymphoma?
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Study Notes
Malignant Tumors of Lymphoid Tissue
- Characterized by abnormal proliferation of B and T cells in lymphoid tissues.
- Two main types: Leukemia (bone marrow and peripheral blood involvement) and Lymphoma (lymph nodes/other organs).
- Plasma cell myeloma is confined to bones, appears as discrete masses, does not involve lymph nodes or peripheral blood.
- Neoplastic proliferations are monoclonal; reactive conditions are polyclonal.
- Lymphoid neoplasms can disrupt normal immune function, causing immunodeficiency and autoimmunity.
Non-Hodgkin’s Lymphoma and B-Cell Neoplasms
- Precursor B-cell neoplasms include Acute Lymphoblastic Leukemia (ALL).
- Mature B-cell neoplasms include Small Lymphocytic Lymphoma (SLL) and Chronic Lymphocytic Leukemia (CLL).
- CLL: most common leukemia in adults with a median age of 70 years; presents as painless generalized lymphadenopathy.
- 80% of CLL cases involve bone marrow at diagnosis; 30-40% may progress to Diffuse Large B-cell Lymphoma (DBLCL).
Follicular Lymphoma
- Morphology: Vaguely nodular appearance with disrupted architecture and regions of neoplastic cells (centrocytes and centroblasts).
- Centrocytes are slightly larger, with angular cleaved nuclei; centroblasts are larger with fine chromatin and prominent nucleoli.
- Immunophenotyping commonly shows B-cell markers and germinal center markers (CD10, BCL6).
- Characterized by a specific translocation (t(14;18)) resulting in BCL2 gene overexpression.
MALT-Type Lymphoma (Extranodal Marginal Zone Lymphoma)
- Associated with chronic inflammation or autoimmune disorders, such as Helicobacter gastritis or Hashimoto thyroiditis.
- Persistent at the site of origin; withdrawal of inciting cause can lead to tumor regression.
- Morphological features: small round to irregular cells infiltrating involved tissues, often forming lymphoepithelial lesions.
Mantle Cell Lymphoma
- Composed of naive B cells resembling those in mantle zones of normal lymphoid follicles.
- Primarily affects men over 50 years, commonly presenting with fatigue and lymphadenopathy.
- Associated with a specific translocation (t(11;14)) leading to cyclin D1 overexpression, resulting in an aggressive disease with a median survival of 4 to 6 years.
Diffuse Large B-cell Lymphoma (DLBCL)
- Most common lymphoma type in adults; presents typically in the 60s age range.
- Rapidly enlarging symptomatic mass may occur; common extranodal sites include the gastrointestinal tract.
- Associated with mutations/rearrangements of the BCL6 gene, often arising from follicular lymphomas with t(14;18) translocation.
- Morphology: large neoplastic B cells (3-4 times size of resting lymphocytes) with rare mitoses, resembling centroblasts or immunoblasts. Immunophenotyping includes CD10 and CD20.
Subtypes of DLBCL
- EBV-associated lymphomas common in AIDS, iatrogenic immunosuppression, and elderly patients.
- Kaposi sarcoma herpes virus (HHV-8) linked to primary effusion lymphomas.
- Mediastinal large B-cell lymphoma is prevalent in young women, with a propensity for abdominal and CNS spread.
Prognosis
- DLBCL without treatment is aggressive and rapidly fatal; intensive therapy can lead to complete remission or potential cure.
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