Lymphoid Neoplasms III Flashcards
1
Q
What does plasma cell neoplasms consist of?
A
Aberrant proliferation of plasma cells, diseases like multiple myeloma (MM) and monoclonal gammopathy of undetermined significance (MGUS) are examples plasma cell neoplasms.Important to know MGUS can develop into MM.
2
Q
Explain MM.
A
Important to know that death by MM is primarily due to infection or renal involvement.
3
Q
Explain the different types of Burkitt lymphoma/leukemia.
A
- Endemic form of Burkitt lymphoma is in Africa, >95% is associated with EBV in, presents as jaw lesions
- Sporadic BL is in the US that involves children and adults, only 20% associated with EBV, this does not present as jaw lesion but instead can have extranodal sites in the abdomen/pelvis.
- HIV associated BL, 25% associated with EBV.
4
Q
Explain Burkitt lymphoma.
A
- Rapidly progressive tumor mass but higher cure rates with aggressive chemotherapy, 90% cure rate in children.
- Tumor lysis syndrome is common when treatment is initiated
- “Starry sky” appearance, sheets of lymphocytes with interspersed “tingible body” macrophages
- t(8;14)—translocation of c-myc (8) and heavy-chain Ig (14)
- In lymph nodes we see diffuse infilterate of medium-sized, non cleaved lymphocytes CD19+, CD20+, CD10+, CD5-, sIg+ and TdT-
5
Q
Explain Mantle Cell lymphoma.
A
- Very aggressive, patients typically present with late-stage disease.
- Adult males
- t(11;14)—translocation of cyclin D1 (11) and heavy-chain Ig (14) CD19+, CD20+, CD10-, CD5+. sIg+ and Cyclin D1+
6
Q
Explain Extranodal Marginal Zone B cell lymphoma of Mucosa Associated lympoid tissue.
A
- Can be along anywhere in the GI tract, lung, head & neck, eye, skin.
- Association with chronic infection (e.g. H. pylori ) and autoimmune disorders (e.g. Hashimoto thyroiditis, Sjögren syndrome)
- Usually early stage, rarely involves the bone marrow
- Expanded marginal zone is observed, lymphoepithelial lesions are seen. t(11:18)
