An insidious presentation of splenic marginal zone lymphoma.
Herein, we report on a patient with splenic marginal zone lymphoma who initially presented without splenomegaly and bone marrow (BM) or peripheral blood involvement. At first, the patient showed moderate leukothrombocytopenia; she was completely asymptomatic, and BM examination excluded a hematologic disease. After 7 months, spleen enlargement was detected without determining any symptoms or worsening of the bilinear cytopenia. Bone marrow histologic, immunohistochemic, cytologic, and immunophenotypic examinations were normal. Splenectomy was performed, and a diagnosis of splenic marginal zone B-cell lymphoma was established. A monoclonal IgVH gene rearrangement was identified in the spleen tissue (VH3 gene family) and subsequently detected in the BM mononuclear cells. Because of the large surgical debulking and the absence of histologic, cytologic, and immunophenotypic BM involvement, no further treatment was proposed. After the splenectomy, the blood cell count normalized, and neither BM nor peripheral blood involvement appeared after 12 months of follow-up.
Burcheri
Sara
S
Division of Hematology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Viale Golgi 19, 27100 Pavia, Italy. sara.burcheri@tin.it
Arcaini
Luca
L
Della Porta
Matteo
M
Paulli
Marco
M
Boveri
Emanuela
E
Zibellini
Silvia
S
Rumi
Elisa
E
Algarotti
Alessandra
A
Passamouti
Francesco
F
Lazzarino
Mario
M
D054637:Dual-Specificity Phosphatases / Q000235:genetics; D005260:Female; D015321:Gene Rearrangement / Q000235:genetics; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D008875:Middle Aged; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery
C442320:DUSP5 protein, human; D054637:Dual-Specificity Phosphatases