Splenic Marginal Zone Lymphoma : Introduction

Comments:
Splenic Marginal Zone Lymphoma (SMZL) - Introduction: SMZL is an uncommon low-grade B-cell lymphoma involving spleen and bone marrow. The splenic hilar lymph nodes are also frequently involved. Peripheral blood shows villous lymphocytes with polar villous projections.

Nodular infiltrates composed of small lymphocytes with marginal zone differentiation replace the preexisting lymphoid follicles of the splenic white pulp. This corresponds grossly to a homogenous micronodular appearance as seen in this image. Despite similar terminology, SMZL is distinct from MALT or nodal marginal zone lymphomas in its clinical presentation and immunophenotypic and genetic features.

This splenectomy specimen is from a 60 y/o female who presented with splenomegaly. The spleen measured 21 cm in greatest dimension and weighed 1400 g. The final diagnosis was SMZL. The cut surface shows numerous confluent nodules measuring up to 0.4 cm in size. Image copyright : pathorama.ch.