title: Transformation of hairy cell leukemia to high-grade lymphoma: a case report and review of the literature.
abstract: The coexistence of hairy cell leukemia (HCL) and non-Hodgkin's lymphoma is extremely rare. In the few reports demonstrating such coexistence, the relationship between the 2 entities was mostly inconclusive. We report a case of HCL that transformed to large cell lymphoma. This case has been followed for more than 4 years with immunohistochemical, flow cytometric, and molecular genetic studies on multiple bone marrow biopsy specimens, a splenectomy specimen, and a lymph node biopsy. In our case, the immunophenotype and tartrate-resistant acid phosphatase stain confirmed that the large cell lymphoma was of HCL origin. The markedly increased Ki-67 staining (proliferation fraction) in the lymph node biopsy specimen compared to the earlier splenectomy specimen indicated the transformation of a low-grade leukemia to a high-grade lymphoma. The overexpression of p53 in the lymph node implies that p53 mutation was probably involved in the pathogenesis of HCL transformation.
authors.affiliation: Pathology and Laboratory Medicine Service, Veterans Affairs Medical Center, Denver, CO 80220, USA.
authors.forename: Tsieh
authors.initials: T
authors.lastname: Sun
authors.forename: Nichon
authors.initials: N
authors.lastname: Grupka
authors.forename: Catherine
authors.initials: C
authors.lastname: Klein
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D002471:Cell Transformation, Neoplastic; D004273:DNA, Neoplasm / Q000032:analysis; D006801:Humans; D007124:Immunoenzyme Techniques; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D019394:Ki-67 Antigen / Q000378:metabolism; D007943:Leukemia, Hairy Cell / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D008297:Male; D016609:Neoplasms, Second Primary / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D013154:Spleen / Q000378:metabolism / Q000473:pathology; D016159:Tumor Suppressor Protein p53 / Q000378:metabolism
chemical_list: D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D019394:Ki-67 Antigen; D016159:Tumor Suppressor Protein p53