Lymphoid Malignancies Flashcards
(35 cards)
What is the basic work up for lymphadenopathy?
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.
What common viruses are linked to various lymphoma types?
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.
Which bacteria predisposes to lymphoma?
H. Pylori can cause MALT lymphoma (mucosa-associated lymphoid tissue).
What malignancy is Hodgkin’s patient that are treated at risk for?
NHL
Which pathologic testing is performed when lymphoma is suspected?
Molecular (cytogenetics, FISH, gene expression), histopathology, and immunophenotyping.
What is the prognosis for germinal center B-cell phenotype lymphoma vs. activated B-cell phenotype?
Germinal center has a more favorable prognosis than activated B-cell phenotype.
What are the progression and cure status of indolent vs. aggressive lymphomas?
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.
What determines prognosis is aggressive lymphomas?
Differences in response to therapy based on extent of disease, presenting symptoms, prognosis, gene expression, patient age, and comorbid conditions.
What are “B” symptoms?
Fever, night sweats, and weight loss.
What are the common indolent lymphomas?
Follicular, MALT, Chronic Lymphocytic Leukemia, and Hairy Cell Leukemia.
What are the common aggressive lymphomas?
Diffuse large Cell, Mantle Cell, Hodgkin, Cutaneous T-Cell Non-Hodgkin lymphoma.
What is the most common stage of presentation for follicular lymphoma?
Stage IV with bone marrow involvement.
What is the grade and prognosis of follicular lymphoma?
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.
What is the oncogene and cytogenetic defect in follicular lymphoma?
Bcl-2 oncogene (present in 90%), and t(14;18) defect.
What is the treatment of follicular lymphoma?
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.
What is the primary treatment for gastric MALT lymphoma?
Triple combination therapy for H. Pylori. (Metronidazole, amoxicillin or clarithromycin plus omeprazole).
What are other autoimmune associations with MALT lymphoma?
AI or inflammatory disease such as Sjögren syndrome or Hashimoto thyroiditis.
What is the most common lymphoid malignancy?
Chronic Lymphocytic Leukemia.
What are the stages of CLL and what is the prognosis?
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.
When should you treat CLL and what is it?
Only when patient presents with symptoms (fever, weight loss, sweating, and pain) or poor prognostic features. Purine nucleoside analogue (fludarabine) based chemotherapy.
What are the most common opportunistic infections in CLL?
PJP, CMV, and HSV.
What is Richter transformation?
transformation of indolent disease (such as CLL) to more aggressive disease.
What are the demographics and presentation of Hairy Cell Leukemia?
Older adults and 5 times more common in men. Present with progressive cytopenia and splenomegaly without LAD.
What is the treatment of hairy cell leukemia?
Purine analogues such as cladribine and durable complete remission occurs in >80% with only one cycle.
