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 Â 10 9 /l. He had splenomegaly but no lymphadenopathy or hepatomegaly. The tumor cells were CD5 þ , CD19 þ , CD20 þ , FMC-7 þ , 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 Â 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.

Mantle cell leukemia as a cause of leukostasis

“…In addition, we confirmed the presence of t(11;14) and t(8;10)(q13;p13) translocations which have also been well documented in the pathogenesis of mantle cell lymphoma. 4,16 This patient has some similarities to the case reported by Smith et al 12 Our patient had leukostasis syndrome, albeit with different manifestations. Whereas the previous report described oculodynia and blurred vision, our patient presented with acute respiratory distress.…”

“…Very few cases have been reported with peripheral counts exceeding 300 × 10 9 /L. 11,12 We report a case of mantle cell lymphoma presenting with a hyperleukocytosis over 650 × 10 9 /L, with pulmonary leukostasis. This case also illustrates the importance of consistently performing immunophenotype and molecular studies, because the diagnosis of mantle cell lymphoma may be overlooked if the appropriate information is not obtained.…”

Primary Gastric Mantle Cell Lymphoma

“…MCL is more likely to appear along with other gastrointestinal diseases (Chron's disease, adenocarcinoma). 1,2,9 In the vast majority of cases, gastrointestinal lymphoma can be diagnosed with endoscopic biopsy. By using additional immunological and molecular markers we can group lymphomas into subtypes according to the WHO classification and that is the cornerstone for further decision making.…”

“…However, stomach is the most comon site of extranodal lymphoma, which are almost all of B-cell lineage, including extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT lymphoma) and diffuse large B-cell lymphomas (DLBC). 2,3 Development of MCL is a result of malignant transformation of B-cell lymphocytes in mantle zone of a lymph node follicle - pregerminal center that surrounds normal germinal center follicles. This subtype of non-Hodkin's lymphoma is also caracterized by the chromosomal translocation t(11;14) (q13;q32).…”

“…Furthermore, immunohistochemical phenotype CD5 + , CD19 + , CD20 + , CD10 − , CD23 − , FMC-7 + , bcl-2 + and cyclin D1 + is specific of MCL. 2–6 We however need to emphasize that aberrant immunophenotype was also established in several cases. 4,7–8 Although nowadays we do have powerful genetic, immunohistocemical and other modern diagnostic and therapeutic methods, MCL still presents itself as a very aggressive disease with poor prognosis and median survival of 3–4 years.…”