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Lecture 21
Chronic Lymphoproliferative Disorders
| Question | Answer |
|---|---|
| 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. |
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UVAPATH4
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