1. ¹Department of Clinical Pathology, Royal Oak, MI, USA
2. ²Department of Hematology/Oncology, Royal Oak, MI, USA
3. ³Department of Anatomic Pathology, William Beaumont Hospital, Royal Oak, MI, USA

Correspondence: Dr JC Mattson, MD, Department of Clinical Pathology, William Beaumont Hospital, 3601 W 13 Mile Road, Royal Oak, MI 48073, USA. E-mail: jmattson@beaumont.edu

Received 19 December 2003; Accepted 29 January 2004.

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 10⁹/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 100 10⁹/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.