Splenic marginal zone lymphoma complicated by cold agglutinin disease.
Splenic marginal zone lymphoma (SMZL) is a rare low-grade B-cell lymphoma accounting for less than 1% of lymphoid neoplasms and is often associated with autoimmune disorders. A 48-year-old woman presented with severe anemia due to steroid-refractory cold agglutinin disease (CAD), and was referred to our hospital for management of progressive systemic illness and high fever. On admission, she showed elevated serum soluble IL-2R and mild splenomegaly. PET/CT revealed FDG accumulation in the spleen and bone. She was pathologically diagnosed as having splenic marginal zone lymphoma by splenectomy and received 8 cycles of rituximab every 2 weeks, resulting in marked improvement of anemia. Lymphoma cells were positive for CD20/CD11c/CD13, phenotypically compatible with SMZL. We report herein the characteristic features of SMZL, and appropriate procedures for diagnosis and treatment.
Department of Hematology and Oncology, Institute of Medical Science, The University of Tokyo.
Kiyosumi
K
Ochi
Kazuaki
K
Yokoyama
Nobuhiro
N
Ohno
Yasunori
Y
Ota
Arinobu
A
Tojo
D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000188:drug therapy*; D008875:Middle Aged; D000069283:Rituximab / Q000627:therapeutic use*; D013156:Splenectomy / Q000379:methods; D013160:Splenic Neoplasms / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D016896:Treatment Outcome
D000069283:Rituximab