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Published on: March 30, 2018
Mantle cell leukemia, prolymphocytoid type: a rarely described form
1Division of Hematopathology, Department of Pathology, St. Louis University Health Sciences Center, St. Louis, Missouri, USA. dunphyc@slucare1.sluh.edu
Insights
This report details a rare case of mantle cell leukemia, prolymphocytoid type, in an elderly woman. Complete flow cytometry data and diagnostic methods are presented to raise awareness of this uncommon leukemic manifestation.
Area of Science:
- Hematology
- Oncology
- Flow Cytometry
Background:
- Mantle cell lymphoma (MCL) can present with leukemic manifestations, though typically in advanced stages.
- Peripheral blood findings in MCL leukemia usually involve cells resembling mantle cells or, less commonly, a blastic morphology.
Observation:
- A prolymphocytic leukemia with the characteristic t(11;14) translocation has been previously noted, but lacked comprehensive flow cytometry data.
- This report focuses on a specific subtype: mantle cell leukemia, prolymphocytoid type, with complete flow cytometric immunophenotypic profiling.
Findings:
- A 90-year-old female presented with an elevated white blood cell count, leading to the diagnosis of mantle cell leukemia, prolymphocytoid type.
- Diagnosis was confirmed through a combination of peripheral blood flow cytometry, cytomorphology, and bone marrow cyclin D1 immunohistochemistry.
- This case provides complete flow cytometric data for this rare entity.
Implications:
- The description of this rare entity aims to increase clinician awareness of mantle cell leukemia, prolymphocytoid type.
- Complete immunophenotypic data aids in accurate diagnosis and classification of rare leukemic subtypes.
- Understanding rare presentations of MCL is crucial for timely and appropriate patient management.
Abstract:
Leukemic manifestations of mantle cell lymphoma are seen in a minority of cases, usually associated with extensive tumor. Usually the neoplastic cells in the peripheral blood resemble mantle cells with a mature chromatin pattern and irregular nuclear contours, or less frequently with a more "blastic" chromatin pattern. A prolymphocytic leukemia with t(11;14)(q13;q32) has previously been reported; however, a complete flow cytometric immunophenotypic profile was lacking. Mantle cell leukemia, prolymphocytoid type with complete flow cytometric data has not previously been described and is the purpose of this report. We report such a case in a 90 year-old female who presented with an elevated white blood cell count. The diagnosis was based on flow cytometric immunophenotyping and the cytomorphology of the peripheral blood combined with cyclin D1 immunohistochemical staining of the bone marrow. We describe our findings and her clinical course in order to heighten awareness of this previously rarely described entity.
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