Lymphoid Neoplasms IV

Question 1

Explain Mycosis Fungoides.

Answer 1

• Mycosis fungoides aka CTCL presents with skin patches/plaques, characterized by atypical CD4+ cells with “cerebriform” nuclei.
• May progress to Sézary syndrome which is lekemia phase of CTCL(T-cell leukemia).
• Associated with epidermotropism and Pautrier microabscesses.
• Sezary syndrome causes erythroderma.

Question 2

Explain Adult T Cell Lymphoma.

Answer 2

• Caused by HTLV (associated with IV drug abuse)
• Adults present with cutaneous lesions; especially affects populations in Japan, West Africa, and the Caribbean.
• Lytic bone lesions, hypercalcemia.
• Generalized lymphadenopathy with skin lesions
• Hepatosplenomegaly

Question 3

Explain Anaplastic Large Cell Lymphoma.

Answer 3

Consists of large anaplstic cells positive for CD30+

• t(2;5) - rearrangement of ALK gene, this happens in children and younger adults, associated with good prognosis
• No ALK rearrangement Anaplastic Large B cell lymhoma happens in older adults, associated with poor prognosis

Question 4

Explain Peripheral T Cell Lymphoma, Unspecified.

Answer 4

• Proliferation of mature T-cells
• Generalized lymphadenopathy
• Fever, pruritus, weight loss
• Eosinophilia
• Generally poor prognosis. Lymph nodes
• Architectural effacement
• Vascular proliferation
• Immunophenotype CD2+, CD5+, CD3+, TCR-αβ or γδ
• Genetics T-cell receptor gene rearrangement

Question 5

Compare Hodgkinvs Non Hodgkinlymphoma as compared in First Aid.

Question 6

Compare Hodgkin vs Non-Hodgkin lymphoma as compared in this lecture.

Question 7

What are Reed Sternberg cells?

Question 8

Explain the features of Hodgkin lymphoma.

Answer 8

• Lymphadenopathy, splenomegaly
• Constitutional (“B”) symptoms –fever, night sweats, weight loss
• Immune dysfunction
• Prognosis –excellent at early stage (90% cure rate) Risk of second malignancy in long-term survivors
• Myelodysplastic syndromes and acute myeloid leukemia
• Lung cancer, breast cancer, gastric cancer, sarcoma, melanoma

Question 9

What are the 2 types of Hodgkin lymphoma?

Answer 9

Classical Hodgekin lymphoma and Nodular lymphocyte predominance Hodgekin lymphoma

Question 10

What are the pathological findings of classical Hodgkin’s lymphoma?Explain the genetics of this neoplasm.

Answer 10

Reed Sternberg cells + polymorphous cellular background.Important to know that Reed Sternberg cells are CD15+ and CD30+and they are negative for most B and T cell markers.Genetics:

• Reed-Sternberg cells arise from germinal center B- cells
• NF-κB activation
• Transformed cells escape from apoptotic pathways to proliferate
• Ig gene rearrangements in most cases No detectable Ig due to
• “Crippled” rearrangements
• Upstream mutations
• Transcriptional inactivation

Question 11

What are the further divisions of classical Hodgkin lymphoma

Answer 11

Nodular sclerosis

• Broad bands of fibrous tissue
• Numerous lacunar variants of Reed-Sternberg cells in a mixed cellular background
• Mixed cellularity - Numerous Reed-Sternberg cells in a mixed cellular background Lymphocyte depletion
• Rare classic Reed-Sternberg cells
• Scant lymphocytes
• Lymphocyte rich –rare