Leukemic Mantle Cell Lymphoma: Clinical and Pathologic Spectrum of Twenty-Three Cases

Abstract

Twenty-three patients with marked leukemic involvement by mantle cell lymphoma (MCL) are described. Each patient had an absolute lymphocyte count more than 10 ؋ 10 9 /L. The diagnosis of MCL was supported by compatible immunophenotypic findings and the t(11;14)(q13;q32) in all cases. Morphologically, these cases exhibited a spectrum of findings that we divided into two groups using a cutoff of 20% large or blastoid cells (log rank test, P ‫؍‬ .004). Patients with small-cell (<20%) morphologic features survived longer than patients with large/blastoid (>20%) morphologic features, (P ‫؍‬ .003, log rank test). The most common additional karyotypic abnormality identified in this study involved chromosome 17, in 13 of 23 (56.5%) cases, which correlated with p53 overexpression but not with cytologic features. We conclude that cytologic features of MCL predict the prognosis of patients with marked leukemic involvement. Chromosome 17 abnormalities are common in leukemic MCL, may be involved in pathogenesis, and are associated with p53 expression.

Case report: mantle cell lymphoma, prolymphocytoid variant, with leukostasis syndrome

“…There have been four prior reports of prolymphocytoid or nucleolated mantle cell lymphoma. [2][3][4][5] The previously reported cases had the t(11;14) by either conventional cytogenetics or FISH. Mantle cell lymphoma had a characteristic immunophenotype: CD5 þ , CD19 þ , and CD20 þ , and usually negative for CD23.…”

“…The medical literature has reports of another variant termed prolymphocytoid or nucleolated. [2][3][4][5] The identification of mantle cell lymphoma is important because of its aggressive clinical course. It is incurable with standard treatment regimens and has a median survival of only 3-5 years.…”

“…[6][7][8] Reports of mantle cell leukemia with prolymphocytoid or nucleolated morphology, as seen in the current case, are rare. [2][3][4][5] We are aware of only one prior report describing mantle cell lymphoma with a leukocyte count over 300 Â 10 9 /l (689 Â 10 9 /l). 4 Here, we describe a case of mantle cell lymphoma, prolymphocytoid variant, with marked leukocytosis of 1227 Â 10 9 /l and with a leukostasis syndrome.…”

Mantle Cell Lymphoma: Decision Making for Transplant

“…Diagnosis of MCL can be made by lymph node or bone marrow biopsy, or analysis of malignant cells obtained from peripheral blood, if the disease is in the leukemic phase 9,10 . Differential diagnosis with CLL is important since both MCL and CLL cell have coexpression of CD5 and CD19/20 11 .…”

“…Prognosis can be estimated by using MIPI (mantle cell lymphoma international prognostic index, Figure 1) 26 which seems to be more efficient than international prognostic index (IPI) 27 or follicular lymphoma international prognostic index (FLIPI) 28, 29 , which includes leukemic phase 10,30,31 , besides other clinical parameters used in the IPI. For each prognostic factor, 0 to 3 points were given to each patient and points were summed up to a maximum of 11.…”