title: Hand mirror cell lymphoid leukemia in adults. A distinct clinicopathologic syndrome. Case report and literature review.
abstract: Hand mirror cell (HMC) lymphoid leukemia is an unusual variant of acute lymphocytic leukemia (ALL) in which the bone marrow lymphoblasts manifest distinctive hand mirror morphologic features. Reported here is a 66-year-old woman with HMC lymphoid leukemia whose clinical course was characterized by 12 months of initial disease stability while she was receiving no chemotherapy; a prompt response to cyclophosphamide, vincristine, and prednisone therapy once instituted; and a hyperleukocytic episode (leukocyte count 607,000/mm3), which resulted in her death after 22 months of disease. This patient and 13 other reported adults (15 years and older) with HMC lymphoid leukemia (greater than 40% bone marrow HMC) are reviewed. HMC lymphoid leukemia appears to differ from typical adult ALL in that it has a female predominance, a relatively indolent early clinical course that lasts 1 year or longer, and it manifests the possibility of survival for 1 or 2 years despite the failure to achieve a complete remission with chemotherapy. Phenotypically, the HMC leukemic cells from all adults evaluated were null cells, Ia-positive, TdT-positive, and stained positively with acid phosphates, which suggests that HMC lymphoid leukemia is a variant of non-T, non-B-ALL. HMC lymphoid leukemia in adults appears to be a distinctive clinicopathologic entity.
authors.forename: E M
authors.initials: EM
authors.lastname: Mazur
authors.forename: E G
authors.initials: EG
authors.lastname: Wittels
authors.forename: F J
authors.initials: FJ
authors.lastname: Schiffman
authors.forename: K
authors.initials: K
authors.lastname: South
authors.forename: R J
authors.initials: RJ
authors.lastname: Horner
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D007945:Leukemia, Lymphoid / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*