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Mature T cell leukemias which cannot be adequately classified under the new WHO classification of lymphoid neoplasms

Leukemia volume 16, pages 2457–2458 (2002)Cite this article

We would like to draw attention to rare peripheral blood-and/or marrow-based leukemias of mature T cells which do not fit well within any of the entities included in the new WHO Classification of Lymphoid Neoplasms, and therefore cannot be adequately classified using this system. These have all been CD4-positive, mature T cell lymphoproliferative processes without either the prolymphocytoid morphology or associated hepatosplenomegaly and/or generalized lymphadenopathy characteristic of T cell prolymphocytic leukemia (T-PLL), and without clinical or morphologic features to suggest adult T cell leukemia/lymphoma, mycosis fungoides/Sézary syndrome, T cell large granular lymphocytic leukemia, or an underlying peripheral T cell lymphoma. Because of the rarity of these cases, we have chosen to describe them via this letter, rather than wait the 5 to 10 years likely to be required to accumulate an adequate series for a full-length manuscript.

Under the Revised European–American Lymphoma (REAL) classification previously employed by most hematopathologists,1 these cases would have fallen within the spectrum of processes known as T cell chronic lymphocytic leukemia/prolymphocytic leukemia (T-CLL/PLL), and therefore could have been readily classified for the clinicians treating these patients. However, under the WHO classification the T-CLL component of the REAL's T-CLL/PLL category has been eliminated,2,3 leaving hematopathologists with only the entity known as T cell prolymphocytic leukemia (T-PLL) for the classification of cases with the clinical and morphologic features described above. Because these cases are more similar to those in a 1995 series of 25 cases of T-CLL4 than they are to T-PLL, we feel it is important to include an entity comparable to T-CLL in any comprehensive lymphoma classification system. Therefore, the REAL classification appears superior to the WHO system in dealing with these rare cases. While we agree with the WHO's decision to separate T-PLL from T-CLL in light of the cytogenetic evidence arguing that T-PLL is indeed a distinct entity, based on our experience we cannot support the decision to eliminate a T-CLL-like entity.

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  1. Department of Laboratory Medicine, University of Washington Medical Center, Seattle, WA, USA

    SJ Kussick, BL Wood & DE Sabath

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Kussick, S., Wood, B. & Sabath, D. Mature T cell leukemias which cannot be adequately classified under the new WHO classification of lymphoid neoplasms. Leukemia 16, 2457–2458 (2002). https://doi.org/10.1038/sj.leu.2402715

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