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Lecture 21

Chronic Lymphoproliferative Disorders

QuestionAnswer
What is the normal ratio of CD4:CD8? 2:1
What is the typical ratio of kappa:lambda expression on B cells? 2-3:1
Immunophenotype of Helper T cells CD 3+ and CD 4+
Immunophenotype of Suppressor T cells CD 3+ and CD 8+
Immunophenotype of B cells CD 19+, CD 20+, and CD 5+
Immunophenotype of peripheral blood stem cells CD 34+
Immunophenotype of natural killer cells CD 56+, CD 3-
Immunophenotype profiles of CLL kappa or lambda Surface Ig, CD19, CD20, CD5, and CD23
Immunophenotype profiles of MCL kappa or lambda Surface Ig, CD19, CD20, CD5, FMC-7, Cyclin D1
Immunophenotype profiles of HCL kappa or lambda Surface Ig, CD19, CD20, FMC-7, CD103, and CD11c
Immunophenotypic profile of SLVL (Splenic lymphoma with villous lymphocytes) MBL (Monocytoid B cell lymphoma) MZL (Marginal zone lymphoma) kappa or lambda surface IgG, CD19, CD20, FMC-7, and CD11c
Immunophenotypic profile of FL (Follicular lymphoma) kappa or lambda surface IgG, CD19, CD20, and CD10
Immunophenotypic profile of LPL (Lymphoplasmacytic lymphoma) kappa or lambda surface IgG, CD19 and CD20
Immunophenotypic profile of LGL-NK (Large granular lymphocyte syndrome NK cell) CD56
Immunophenotypic profile of LGL-T (Large granular lymphocyte syndrome T cell) CD3, CD8, and CD57
Immunophenotypic profile of PLL-B (prolymphocytic leukemia B cell) kappa or lambda surface IgG, CD19, CD20, and FMC-7
Immunophenotypic profile of PLL-T ((prolymphocytic leukemia T cell) CD5, CD3, CD4
Immunophenotypic profile of Mycosis fungoides-Sezary's cell CD5, CD3, CD4
What are the prognostic factors of CLL? 1)Age 2)Male sex 3)Performance status 4)Stage 5)Cytogenetic profiles 6)Mutational status of Ig Vh region 7)Prolymphocytic transformation 8)Transformation to Richter's, Hodgkin lylmphoma, ALL 9) serum beta2microglobulin level, Bcl2, Bcl6, telomere length
What is the most common leukemia in the Western world? Chronic lymphocyte leukemia (CLL)
What is the median age of diagnosis of CLL? 65 years
What are the most frequent sites of secondary cancers in CLL? Skin, lungs, and GI tract
What are the autoimmune complications of CLL? Hemolytic anemia, thrombocytopenia, neutropenia, and pure red cell aplasia
What organisms are the most frequent causes of recurrent infections in CLL? Streptococcus penumoniae, staphylococcus, Haemophilius influenzae, candida, asperigllus, varicella zoster virus
What are the possible disease transformations of CLL? Prolymphocytic leukemia, Large-cell lymphoma (Richter's syndrome), Acute leukemia, Multiple myeloma
(T or F) Fever should not be attributed to CLL and should be considered a manifestation of infection or drug-induced. True.
What is the historical diagnostic lymphocyte count criteria for CLL? Lymphocytosis of small lymphocytes of greater than 5,000/uL
What is the Rai Stage 0 criteria for clinical staging of CLL? Lymphocytosis only (of peripheral blood and bone marrow)
What is the Rai Stage I criteria for clinical staging of CLL? Lymphocytosis plus enlarged nodes
What is the Rai Stage II criteria for clinical staging of CLL? Lymphocytosis plus enlarged spleen and/or liver (nodes may not be enlarged)
What is the Rai Stage III criteria for clinical staging of CLL? Lymphocytosis plus anemia (Hb<11 g/dL). Nodes, spleen, and liver may or may not be enlarged.
What is the Rai Stage IV criteria for clinical staging of CLL? Lymphocytosis plus thrombocytopenia (plt<100,000/mm3). Enlargement of nodes, spleen, and liver; anemia may or may not be present.
What is the most frequent cytogenetic abnormality associated with CLL? 13q deletion
Which cytogenetic abnormality of CLL has the worst prognosis? 17q deletion
(T or F) Somatic hypermutation of the immunoglobulin heavy-chain variable region has a good prognosis. True.
What is the relationship between the degree of somatic hypermutation of the immunoglobulin heavy-chain variable region with ZAP-70 expression? The degree of somatic mutation has an inverse relationship with the degree of ZAP-70 expression by the clonal B cells.
(T or F) Low expression of CD38 (<30%) confers a better prognosis in CLL? True.
(T or F) The presence of Bcl-6 mutation is a negative prognostic factor for CLL. False. The higher the expression of Bcl-2, the worse the prognosis. The presence of Bcl-6 mutation indicates a better prognosis in CLL
What is the treatment for hypogammaglobulinemia with recurrent infection in the setting of CLL? Give monthly IV Ig
What is the first line of therapy for autoimmune complications of CLL? Prednisone
What purine nucleosides are used for treatment of CLL? (1) Fludarabine (2) 2-Chlorodeoxyadenosine (3) 2-deoxycoformycin
A hematologic disorder presenting with spelnomegaly without lymphadenopathy and the presence of small lymphocytes with hairy projections only at one pole of the cells. Splenic lymphoma with Villous Lymphocytes
A syndrome characterized by rheumatoid arthritis, splenomegaly, and neutropenia. Felty's syndrome
What is the management of Large Granular Lymphocyte Syndrome? No treatment is necessary in most cases. Prompt treatment of infections is important. Those with frequent infections may require growth factor suppor twith G-CSF. More aggressive forms may require combination chemotherapy, but with poor response.
Created by: UVAPATH4