University of Virginia Health System

Chronic Mantle Cell Leukemia

 
Mantle cell leukemia- Peripheral blood smear, Wright-Giemsa stain, 1000x

 Description:

Although the clonal cells tend to have more indented nuclei, they cannot be distinguished from B-CLL cells or normal lymphocytes by light microscopy. The cells are small, ranging in size from that of a small normal lymphocyte to 1 1/2 times larger. The cytoplasm usually is scant, but does vary somewhat, and is grey to grey-blue in color. The nuclear:cytoplasmic ratio is high, and the nucleus may be round or slightly oval, and is often irregular in appearance. The characteristic nuclear indentations (clefts) are usually present, but vary in frequency. The nuclear chromatin is clumped, and nucleoli usually are not visible.

The nuclear clefting seen in follicular lymphoma cells is usually more pronounced, and the cells tend to be larger with more abundant cytoplasm.

A subset of mantle cell lymphoproliferative disorders present as a leukemia (10% or more,) often with splenomegaly and without lymphadenopathy. A leukemic phase may develop during the course of the more typical presentation of mantle cell lymphoma in up to 20% or more.

Immunophenotype:

The clonal lymphocytes are CD19+, CD5+, FMC7+, and CyclinD1+, but CD23-, in constrast to B-CLL cells, and CD10 negative, in contrast to follicular lymphoma cells. The surface immunoglobulins are IgM and IgD. The colabeling of a CD19+ cell with CD5 and FMC7 is essentially diagnostic. FMC7 positivity is present on other clonal B cell disorders such as hairy cell leukemia, but these disorders do not express CD5.

Cytogenetics:

The signature cytogenetic abnormality is the t(11;14) which is present in essentially all cases as determined by FISH analysis. The translocation is between the IgH gene on chromosome 14 and the Cyclin D1 (bcl-1, PRAD1) gene on chromosome 11. Some cases have 11q abnormalities. In the more aggressive variants (e.g. blastic variant) other abnormalities are frequently seen, and often are similar to those seen in CLL.

Clinical Manifestations and Prognosis:

Those that present as a chronic leukemia with or without splenomegaly, and without lymphadenopathy often pursue a more chronic course not unlike most cases of CLL. The blastic variant is aggressive, similar to other acute leukemias (see under ALL.)

General References:

• Jaffe ES, Harris NL, Stein H, Vardiman JW, eds. Pathology and genetics of tumours of hematopoietic and lymphoid tissues. Vol. 3 of World Health Organization classification of tumors, Lyon, France: IARC Press, 2001
• Banks P, Chan J, Cleary M, et al. Mantle cell lymphoma: A proposal for unification of morphologic, immunologic, and molecular data. Am J Surg Pathol 16:637, 1992

For all publication requests, please complete the image permission form and we will respond to your request shortly.