Lymphomas of the marginal spleen zone are an entity recently considere
d as separate by the International Lymphoma Study Group, There are B-c
ell non Hodgkin's lymphomas (NHL) of low grade malignancy with a chara
cteristic phenotype that allows to differentiate from mantle lymphomas
and other B-cell lymphoproliferative syndromes, The case of a 69-year
-old female patient admitted for abdominal pain due to large splenomeg
aly is reported. Pancytopenia and the presence of atypical large-sized
lymphocytes with extensive cytoplasm and a rounded nucleus with inden
tations, reticulated appearing chromatin and one or several nucleoli w
ere of note in the hemogram, Microscopic examination of the bone marro
w demonstrated moderate-degree lymphocytary infiltration with grade I
reticulin fibrosis, Laparotomy with splenectomy was performed, White p
ulp invasion with multifocal infiltration of the red pulp by lymphocyt
es of the same characteristics as those observed in the peripheral blo
od and bone marrow were observed on microscopic bone marrow examinatio
n, Immunophenotypic study of these lymphocytes was positive for CD19,
CD20 and CD22 while being negative for CD5, CD10, CD23, CD25, CD11c an
d FMC7, the phenotype belonging to the lymphocytes of marginal spleen
zone. Following splenectomy the patient recovered hemoperipheral count
s and did not undergo additional treatment. The patient died due to se
ptic shock of respiratory origin 4 months later. The clinical, morphol
ogic and immunophenotypic features of marginal spleen zone lymphomas a
re reported with emphasis on the differences with other B-cell non Hod
gkin's lymphomas of low malignancy.