title: B-cell prolymphocytic leukemia carrying t(8;14)(q24;q32), associated with both autoimmune hemolytic anemia and pure red cell aplasia.
abstract: An 80-year-old man was referred to our department because of lymphocytosis. His white cell count was 17.1 × 10(3)/μL, with 64% prolymphocytes. He did not exhibit splenomegaly or lymphadenopathy. Prolymphocytes were CD5(+), CD10(-), CD19(+), CD20(+), CD21(+weak), CD22(+), CD23(-), and HLA-DR(+), and expressed μδ/λ cell-surface immunoglobulins. G-banding and fluorescence in situ hybridization using c-MYC and immunoglobulin heavy-chain (IgH) gene probe revealed that leukemia cells carried the t(8;14)(q24;q32)/c-MYC-IgH fusion gene, and breakage and reunion occurred within the non-coding region of c-MYC exon 1 as well as the α switch region of IgH. Nine months after the initial presentation, the patient's hemoglobin level fell to 5.7 g/dL. Coombs' test was positive and marked hypoplasia of erythroid precursors was detected in his bone marrow. The patient was treated with prednisolone followed by 4 weekly doses of rituximab, which led to resolution of the anemia and complete response of the underlying leukemia. The role of t(8;14)(q24;q32)/c-MYC-IgH in the pathogenesis of B-cell prolymphocytic leukemia (B-PLL) may not be identical to that in aggressive lymphoid neoplasms, and, in the present case, autoantibodies targeting both mature red cells and erythroid precursors may have been concurrently produced in the setting of B-PLL.
authors.affiliation: Department of Hematology, Tenri Hospital.
authors.forename: Futoshi
authors.initials: F
authors.lastname: Iioka
authors.forename: Takashi
authors.initials: T
authors.lastname: Akasaka
authors.forename: Masahiko
authors.initials: M
authors.lastname: Hayashida
authors.forename: Atsuko
authors.initials: A
authors.lastname: Okumura
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Ohno
mesh_terms: D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000235:genetics* / Q000473:pathology; D002883:Chromosomes, Human, Pair 14; D002898:Chromosomes, Human, Pair 8; D006801:Humans; D054403:Leukemia, Prolymphocytic, B-Cell / Q000097:blood* / Q000235:genetics*; D008297:Male; D012010:Red-Cell Aplasia, Pure / Q000097:blood / Q000235:genetics* / Q000473:pathology; D014178:Translocation, Genetic