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WHO Classification of Tumours
Splenic diffuse red pulp small B-cell lymphoma
Tumours of haematopoietic and lymphoid tissues


Definition

Splenic diffuse red pulp small B-cell lymphoma is an uncommon lymphoma with a diffuse pattern of involvement of the splenic red pulp by small monomorphous B lymphocytes. The neoplasm also involves bone marrow (BM) sinusoids and peripheral blood (PB), commonly with a villous cytology. This is a provisional entity that needs additional molecular studies for defining its main features and diagnostic markers. This diagnosis should be restricted to characteristic cases, fulfilling the major features described here and not applied to any lymphoma growing diffusely in the spleen. Chronic lymphocytic leukaemia (CLL), hairy cell leukaemia (HCL), lymphoplasmacytic lymphoma (LPL) and B-cell prolymphocytic leukaemia (PLL) should be excluded through appropriate studies. A certain diagnosis may require the examination of the spleen, but may be suggested for cases showing purely intra­sinusoidal BM involvement and villous lymphocytes in the PB. In case of doubt, the use of the term splenic B-cell lymphoma/leukaemia, unclassifiable is warranted.

> Differential diagnosis
Some degree of overlap exists with cases that fulfil the criteria for hairy cell leukaemia-variant

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Sainati L, Matutes E, Mulligan S, de Oliveira MP, Rani S, Lampert IA, Catovsky D (1990)
A variant form of hairy cell leukemia resistant to alpha-interferon: clinical and phenotypic characteristics of 17 patients.
Blood 76: 157-62




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Matutes E, Wotherspoon A, Catovsky D (2003)
The variant form of hairy-cell leukaemia.
Best Pract Res Clin Haematol 16: 41-56



. The neoplastic cells in both entities are DBA.44 (CD72)+, frequently IgG+, IgD- and have frequent chromosomal alterations. Additional studies are required to confirm these findings and to further evaluate the extent of overlap between these entities, particularly as not all studies report the same phenotypic or cytogenetic findings. A rare subtype of large B-cell lymphoma involving the sinusoids, both in the spleen and the BM, has been described by two different groups
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Mollejo M, Algara P, Mateo MS, Menárguez J, Pascual E, Fresno MF, Camacho FI, Piris MA (2003)
Large B-cell lymphoma presenting in the spleen: identification of different clinicopathologic conditions.
Am J Surg Pathol 27: 895-902




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Morice WG, Rodriguez FJ, Hoyer JD, Kurtin PJ (2005)
Diffuse large B-cell lymphoma with distinctive patterns of splenic and bone marrow involvement: clinicopathologic features of two cases.
Mod Pathol 18: 495-502



. Although these cases may be related to those described above, the diagnosis of splenic diffuse red pulp small B-cell lymphoma is restricted to an indolent lymphoma composed of small lymphocytes.

> Related Topics
Introduction: Splenic B-cell lymphoma/leukaemia, unclassifiable
Hairy cell leukaemia-variant
Introduction: Classification of lymphoid neoplasms