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WHO Classification of Tumours
Splenic B-cell lymphoma/leukaemia, unclassifiable
Tumours of haematopoietic and lymphoid tissues


Definition

There are a number of variably well defined entities that represent small B-cell clonal lymphoproliferations involving the spleen, but which do not fall into any of the other types of B-cell lymphoid neoplasms recognized in the WHO classification. The best defined of these relatively rare provisional entities are:

> Splenic diffuse red pulp small B-cell lymphoma (SDRPBCL)

> Hairy cell leukaemia-variant (HCL-v)

The relationship of splenic diffuse red pulp small B-cell lymphoma to hairy cell leukaemia variant and other primary splenic B-cell lymphomas remains uncertain, their precise diagnostic criteria are not fully established and the most appropriate terminology is unsettled.
Other splenic small B-cell lymphomas not fulfilling the criteria for either of these provisional entities should be diagnosed as splenic B-cell lymphoma/ leukaemia, unclassifiable until more is known

Click for details
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



. Therapeutic options for these patients are not well known because of the rarity of the disease and lack of prospective series.

> Related Topics
Introduction: Classification of lymphoid neoplasms