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32 Cards in this Set
- Front
- Back
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what proportion of NHL are FL?
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20%-25%
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what is the median survival in follicular lymphoma?
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8-12 years
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what is the M:F ratio in follicular lymphoma?
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M:F = 1:1
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what is the characteristic genetic finding in FL?
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t(14;18) with overexpression of the bcl2 protoncogene
mnemonic: follicular - fourteen plus unlucky number equals 18 |
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what is the course in FL?
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indolent lymphoma with >80% in stage III/IV at presentation
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what are the therapy principles in FL?
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localized disease (stage I or II) radiation therapy with curative intent
advanced stage palliative with variable chemotherapy schemes, rituximab alone or in combination |
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what disorders are associated with MZL?
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continued massive antigen stimulation
MALT of the salivary gland associated with Sjoegren syndrome MALT of the thyroid associated with Hashimoto thyroiditis MALT of the GIT associated with Crohn disease or celiac disease MALT of the stomach associated with H. pylori infection |
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where are nongastric MALT commonly localized?
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head and neck, lung and orbit
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what are the therapy principles for nongastric MALT lymphomas?
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locoregional radiation and/or surgery for stage IE-II disease
relapse or stage III/IV disease is treated with regimens used for follicular lymphoma, such as R-CVP or FND (fludarabine, mitoxantrone, dexamethasone) |
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what are the therapy principles for gastric MALT lymphomas?
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eradication of H. pylori
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what are the essentials of diagnosis in hairy cell leukemia?
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pancytopenia, often massive splenomegaly and presence of hairy cells on blood smear and especially in the bone marrow
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what is the median age at diagnosis in HCL?
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55 years
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what is the M:F ratio in HCL?
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striking 5:1 male predominance
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what is the frequency of lymphadenopathy and hepatosplenomegaly in HCL?
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lymphadenopathy is uncommon, hepatomegaly is present in 50%, while splenomegaly is almost invariably present
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what is the course of HCL?
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usually an indolent disorder whose course is dominated by pancytopenia and recurrent infections
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what are the blood findings in HCL?
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hallmark is pancytopenia
nearly all with striking monocytopenia |
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what are the bone marrow findings in HCL?
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usually dry tap, biopsy shows hypercellular marrow with infiltration by hairy cells
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what stain is used to differentiate hairy cells from other lymphocytes in HCL?
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TRAP (tartrate-resistant acid phosphatase)
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what are the immunophenotypic findings in HCL?
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B-cell markers CD19 and CD20, as well as CD25
CD103 is an important differentiating markers from other hematologic malignancies, except enteropathy-associated T-cell lymphoma (EATL) |
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in what other hematopoietic disorders is CD103 also expressed?
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EATL (enteropathy associated T-cell lymphoma)
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what region of the spleen is typically infiltrated in HCL?
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red pulp in contrast to the usual predilection of lymphomas to involve the white pulp of the spleen
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what is the differential diagnosis to HCL?
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other lymphoproliferative disease, such as Waldenström macroglobulenemia and other NHL
other causes of pancytopenia, such as hypersplenism due to any cause, aplastic anemia and paroxysmal nocturnal hemoglobinuria |
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what are the therapy principles in HCL?
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as in other indolent lymphomas initially expectant
therapy indications are recurrent infections, cytopenias or B symptoms therapy of choice is the antimetabolite cladribine (2-CDA) with good long-term prognosis |
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which cytostatic drugs are of little effect in HCL?
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alkylating agents
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what are the entities of marginal zone lymphoma?
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extranodal MZL, such as MALT, BALT and SALT
nodal MZL splenic MZL |
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what additional criteria is used in the prognostic index FLIPI?
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hemoglobin <120 g/l
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what are characteristics of splenic marginal zone lymphoma?
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splenomegaly without lymphadenopathy, but common bone marrow involvement
cytopenias secondary to hypersplenism are common |
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what are the key points in hairy cell leukemia?
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typical presentation is an elderly male with pancytopenia, notably monocytopenia and splenomegaly
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what is the characteristic finding in the blood smear in HCL?
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atypical lymphoid cells that stain for tartrate-resistant acid phosphatase (TRAP)
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what is the prognosis in HCL?
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exceptionally sensitive to "gentle" chemotherapy with often long-lasting remission
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what is the cell of origin in HCL?
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memory B cell
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what are the key points in FL?
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older adults with generalized lymphadenopathy and marrow involvement
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