Case report: mantle cell lymphoma, prolymphocytoid variant, with leukostasis syndrome.
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.
Smith
Marc D
MD
1Department of Clinical Pathology, Royal Oak, MI, USA.
Singleton
Timothy P
TP
Balaraman
Savitha
S
Jaiyesimi
Ishmael
I
O'Malley
Barbara
B
Al-Saadi
Abdul
A
Mattson
Joan C
JC
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
D016159:Tumor Suppressor Protein p53; D019938:Cyclin D1