A patient of more than 70 years, presented with generalized lymphadenopathy. WBC count was 20,000/ mm3 and blood film showed >70% mature looking lymphocytes. Next investigation that should he done:
Correct Answer: Peripheral Immunophenotyping
Description: Ans. b. Peripheral Immunophenotyping (Ref: Robbins 9/e p593, 8/e p604; Wintrobes 12/e p2077-2078; Harrison 19/e p701, 18/e p926-927)A patient of more than 70 years, presented with generalized lymphadenopathy. WBC count was 20,000/mm3 and blood film showed >70% mature looking lymphocytes. This clinical situation is suggestive of CLL.Harrison says "Differential diagnosis of typical B cell CLL is extensive. Immunophenotyping will eliminate the other T-cell disorders and can often help sort other B-cell malignancies."B-cell CLLB-cell CLL is neoplasm of small round B-cells that usually have scant surface Ig and a CD 5+/CD 23+Q.May occur in familial clusters with a decreasing age of onset from generation to generation.Lymph node architecture is totally effaced with loss of lymphoid follicles and obliteration of sinuses by an infiltrate of monomorphic small round lymphocytes with condensed chromatin and scant cytoplasmQ.Morphological features that correlate with worse prognosis:Capsular invasionQIncreased number of large lymphocytes in the node or increased number of (>10%) prolymphocytesQ vs mature looking lymphocytes.Richter SyndromeEvolution of CLL to large cell lymphomaQOccurs in <5% of patientsMay arise as transformation of the neoplastic small B cell or as unrelated B-cell cloneImmunophenotypingNeoplastic lymphocytes co-express the nominal T-cell antigen CD-5 with B-cell markers CD-19, 20, 21, 11C and 23Q.The tumor cell are negative for FMC-7, CD-10 and cyclin-D1Q.CD-23 expression and FMC-7 and cyclin-D1 negativity are helpful in phenotypic separation of B-cell from mantle cell lymphoma, another CD-5+ small B-cell neoplasm.
Category:
Pathology
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