| Popis: |
Background: Hairy cell leukemia (HCL) is a slowly progressive lymphoproliferative disorder that tends to afflict middle-aged adults, especially men. Blastic transformation of this form of leukemia is extremely rare. To date, a single case has been reported. Methods and Results: A case of HCL, evolving with blastic transformation after a 9- year clinical course, is reported. Routine histology, cytochemistry, flow cytometry immunophenotyping, and Southern blot analysis for B- and T-cell gene rearrangements were used in the evaluation. Although morphology at the time of presentation was characteristic of HCL, the cells were initially tartrate-resistant acid phosphatase (TRAP) negative. During a clinical course over several years, the hairy cells became progressively TRAP positive. The morphology of the leukemic cells changed 9 years after initial diagnosis, with blastic transformation and retaining strong TRAP positivity. Immunophenotypic analysis showed evolution from a characteristic hairy cell leukemic phenotype to a phenotype indicative of marked immaturity. Genotypic analysis showed an evolving pattern of immunoglobulin gene rearrangements, paralleling the morphology and phenotypic evolution and ruling out a second B-cell malignancy. Conclusions: This case report of blastic transformation in a patient with HCL is only the second such case identified in the medical literature to date. |
