title: Case report: mantle cell lymphoma, prolymphocytoid variant, with leukostasis syndrome.
abstract: A 76-year-old man presented with leukostasis syndrome, including oculodynia, blurred vision, and visual field defects, due to mantle cell lymphoma, prolymphocytoid variant, with marked leukocytosis, 1227 x 10(9)/l. He had splenomegaly but no lymphadenopathy or hepatomegaly. The tumor cells were CD5+, CD19+, CD20+, FMC-7+, and kappa light chain restricted. Immunohistochemistry showed expression of p53 and of cyclin D1. Fluorescent in situ hybridization demonstrated t(11;14) with translocation between CYCLIN D1 and the immunoglobulin heavy-chain genes. The patient received leukapheresis and aggressive chemotherapy, but the leukocyte count remained above 100 x 10(9)/l. The patient's condition rapidly deteriorated with lymphomatous infiltration of his lungs and soft tissues, and he expired 6 months after diagnosis. While it is known that mantle cell lymphoma may have a leukemic phase, the degree of leukocytosis in this case exceeds that previously reported in the literature and resulted in a clinical syndrome of leukostasis.
authors.affiliation: 1Department of Clinical Pathology, Royal Oak, MI, USA.
authors.forename: Marc D
authors.initials: MD
authors.lastname: Smith
authors.forename: Timothy P
authors.initials: TP
authors.lastname: Singleton
authors.forename: Savitha
authors.initials: S
authors.lastname: Balaraman
authors.forename: Ishmael
authors.initials: I
authors.lastname: Jaiyesimi
authors.forename: Barbara
authors.initials: B
authors.lastname: O'Malley
authors.forename: Abdul
authors.initials: A
authors.lastname: Al-Saadi
authors.forename: Joan C
authors.initials: JC
authors.lastname: Mattson
mesh_terms: D000368:Aged; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics; D019938:Cyclin D1 / Q000032:analysis; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D015463:Leukemia, Prolymphocytic / Q000150:complications* / Q000235:genetics / Q000378:metabolism; D007958:Leukocyte Count; D018921:Leukostasis / Q000097:blood / Q000209:etiology*; D020522:Lymphoma, Mantle-Cell / Q000150:complications* / Q000235:genetics / Q000378:metabolism; D008297:Male; D014178:Translocation, Genetic; D016159:Tumor Suppressor Protein p53 / Q000032:analysis
chemical_list: D016159:Tumor Suppressor Protein p53; D019938:Cyclin D1