Hematology - Lymphoma
Terms
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- What are lymphomas and how are they different from leukemias? What are 2 major types of lymphomas?
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Lymphomas: solid tumors of lymphoid system (LN, tonsils, GI tract, spleen, liver). Not found in bone marrow like leukemias.
2 types: Hodgkin's and Non-Hodgkin's - Compare follicular vs. diffuse type NHL in terms of prognosis and cell type.
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Follicular: better px -- difficult to cure but indolent course, B-cell type
Diffuse: agressive but 50% curable, either B or T-cell type - What is the genetic abnormality in follicular lymphoma (small cleaved cell)?
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t (14;18)
bcl-2 expression (involved in apoptosis) - What is the most common lymphoma in children? in adults?
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Children: lymphoblastic lymphoma
Adults: follicular lymphoma - How do lymphoblastic lymphoma patients present?
- Presents with ALL and mediastinal mass (very aggressive)
- What markers are seen in lymphoblastic lymphoma?
- TdT and T-cell marker (T-cell type)
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Burkitt's Lymphoma:
1) cell type?
2) linked to what virus?
3) difference b/w African vs. US form -
1) B-cell
2) EBV
3) African: involves jaw
US: abdomen more common - Describe histology of Burkitt's
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"starry sky pattern"
-- dark background of densely packed lymphocytes with light colored spots of macrophages - Genetic abnormality of Burkitt's
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c-myc gene fused to Ig heavy chain - What is cutaneous T-cell lymphoma (CTCL, mycosis fungoides)?
- slowly progressive CD4+ T-cell lymphoma of skin, usually in elderly
- Describe histology of CTCL
- cerebriform nuclei (nuclei look like cerebral gyri)
- Clinical presentation of CTCL
- systemic erythroderma, erythematous and pruritic rash
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Hodgkin's Lymphoma:
1) age distribution
2) gender
3) most commonly associated virus -
1) bimodal
2) young men
3) EBV (up to 50%) - Presentation of HL
- Constitutional si/sx: low grade fever, night sweats, weight loss, **in some pts, sx worsen with EtOH intake**
- What is the distinctive tumor giant cell seen in HL?
- Reed-Sternberg cells (binucleate giant cell -- owl eyes -- with eosinophilic inclusions
- Name and describe a variant of R-S cell
- Lacunar cell: mononucleated giant cell in nodular sclerosis
- HL severity proportional to ____
- number of R-S cells
- Name 4 variants of HL
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Lymphocyte predominant
Nodular sclerosing
Mixed cellularity
Lymphocyte depletion - Which HL variant is most common?
- Nodular sclerosing
- Which HL variant(s) have excellent prognosis?
- Nodular sclerosing and lymphocyte predominant
- Which HL variant(s) have poor prognosis?
- Lymphocyte depletion
- Which HL variant more common in women, primarily young adults?
- Nodular sclerosing
- Which HL variant in <35yo males?
- Lymphocyte predominant
- Which HL variant has most number of RS cells?
- Mixed cellularity
- Which HL variant(s) has lacunar cell RS variant?
- Nodular sclerosing
- Describe 4 clinical stages.
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I: LN
II: >1 LN on same side of diaphragm
III: both sides of diaphragm
IV: disseminated, organ or extranodal tissue involved