Lymphoid Malignancies Flashcards

1
Q

What is the basic work up for lymphadenopathy?

A

CBC with diff, peripheral smear, ESR, HIV, EBV. Possibly include viral/bacterial markers, connective tissue and AI disease. CXR to assess for hilar LAD. Other studies depending on location, size, and duration.

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2
Q

What common viruses are linked to various lymphoma types?

A

HIV: anaplastic large cell NHL and Burkitt lymphoma. HTLV-1 with T-cell NHL. Hep C with low-grade B-cell NHL, and EBV with Burkitt lymphoma, PTLD, and Hodgkin lymphoma.

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3
Q

Which bacteria predisposes to lymphoma?

A

H. Pylori can cause MALT lymphoma (mucosa-associated lymphoid tissue).

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4
Q

What malignancy is Hodgkin’s patient that are treated at risk for?

A

NHL

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5
Q

Which pathologic testing is performed when lymphoma is suspected?

A

Molecular (cytogenetics, FISH, gene expression), histopathology, and immunophenotyping.

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6
Q

What is the prognosis for germinal center B-cell phenotype lymphoma vs. activated B-cell phenotype?

A

Germinal center has a more favorable prognosis than activated B-cell phenotype.

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7
Q

What are the progression and cure status of indolent vs. aggressive lymphomas?

A

Indolent are a prolonged progression-free interval (7-10) years but are incurable where as aggressive lymphomas can be quickly fatal without treatment but have 40-80% cure rates if CD20 positive.

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8
Q

What determines prognosis is aggressive lymphomas?

A

Differences in response to therapy based on extent of disease, presenting symptoms, prognosis, gene expression, patient age, and comorbid conditions.

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9
Q

What are “B” symptoms?

A

Fever, night sweats, and weight loss.

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10
Q

What are the common indolent lymphomas?

A

Follicular, MALT, Chronic Lymphocytic Leukemia, and Hairy Cell Leukemia.

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11
Q

What are the common aggressive lymphomas?

A

Diffuse large Cell, Mantle Cell, Hodgkin, Cutaneous T-Cell Non-Hodgkin lymphoma.

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12
Q

What is the most common stage of presentation for follicular lymphoma?

A

Stage IV with bone marrow involvement.

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13
Q

What is the grade and prognosis of follicular lymphoma?

A

Grade 1 and 2 (small cell) are indolent. Grade 3(b) behave more like DLCBL. Follicular lymphoma is considered incurable and median survival is 10 years.

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14
Q

What is the oncogene and cytogenetic defect in follicular lymphoma?

A

Bcl-2 oncogene (present in 90%), and t(14;18) defect.

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15
Q

What is the treatment of follicular lymphoma?

A

Hold treatment for grade 1 and 2 until symptomatic or organ dysfunction noted. Possibilities include Rituximab +/- chemo (R-CVP, R-CHOP, R + bendamustine). Possibility for tositumomab and ibritumomab. HSCT in patient that are young and relapsed.

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16
Q

What is the primary treatment for gastric MALT lymphoma?

A

Triple combination therapy for H. Pylori. (Metronidazole, amoxicillin or clarithromycin plus omeprazole).

17
Q

What are other autoimmune associations with MALT lymphoma?

A

AI or inflammatory disease such as Sjögren syndrome or Hashimoto thyroiditis.

18
Q

What is the most common lymphoid malignancy?

A

Chronic Lymphocytic Leukemia.

19
Q

What are the stages of CLL and what is the prognosis?

A

Increase in circulating lymphocytes (stage 0), LAD (stage 1), splenomegaly (stage 2), anemia (stage 3), or thrombocytopenia (stage 4). Prognosis is determined by stage and rate of progression.

20
Q

When should you treat CLL and what is it?

A

Only when patient presents with symptoms (fever, weight loss, sweating, and pain) or poor prognostic features. Purine nucleoside analogue (fludarabine) based chemotherapy.

21
Q

What are the most common opportunistic infections in CLL?

A

PJP, CMV, and HSV.

22
Q

What is Richter transformation?

A

transformation of indolent disease (such as CLL) to more aggressive disease.

23
Q

What are the demographics and presentation of Hairy Cell Leukemia?

A

Older adults and 5 times more common in men. Present with progressive cytopenia and splenomegaly without LAD.

24
Q

What is the treatment of hairy cell leukemia?

A

Purine analogues such as cladribine and durable complete remission occurs in >80% with only one cycle.

25
Q

What is the most common NHL?

A

Diffuse large cell lymphoma

26
Q

What are the common areas of involvement in mantle cell lymphoma?

A

Small intestine, colon, bone marrow, and peripheral blood.

27
Q

What are the are the genetic abnormalities in mantle cell lymphoma?

A

Overexpression of cyclin D1 and t(11;14) translocation.

28
Q

What is the prognosis for mantle cell lymphoma?

A

Median survival with aggressive chemotherapy is 3 years. Allogeneic HSCT remains the only curative option for advanced disease.

29
Q

What is the prognosis and treatment for Hodgkin Lymphoma?

A

Highly curable disease regardless of stage. Treatment is doxorubicin, bleomycin, vinblastin, and dacarbazine (ABVD). Add rituximab for lymphocyte predominant subtype.

30
Q

What are prognostic risk factors for Hodgkin lymphoma?

A

Age >45 years, males, WBC>15000, albumin<10.5.

31
Q

What is the initial therapy for DLBCL?

A

R-CHOP

32
Q

What are the most common forms of T-cell NHL?

A

Mycosis fungoides (affects the skin) and Sézary syndrome (affects skin and blood)

33
Q

What antigen is expressed in T-cell lymphoma?

A

CD4

34
Q

What is the typical progression in T-cell lymphoma?

A

Presents as indolent disease but eventually progresses to skin plaques, diffuse erythema, and ulcerated lesions, followed by infiltration into other organs and a leukemic phase. With progression there is cell-mediated immunodeficiency with recurrent infections.

35
Q

What is the treatment for primarily skin involved T-cell lymphoma?

A

Observation for early stage disease. Topical steroids, retinoids, psoralen and ultraviolet A light (PUVA) may be used for most advanced skin lesions.