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Medical Science Monitor Basic Research


High-Grade Transformation in a Splenic Marginal Zone Lymphoma with a Cerebral Manifestation

Unusual clinical course, Challenging differential diagnosis

Xiaoning Gao, Jie Li, Ji Lin, Daihong Liu, Li Yu, Quanshun Wang

China (mainland) Department of Hematology, Chinese PLA General Hospital, Medical School of Chinese PLA, Beijing, China (mainland)

Am J Case Rep 2017; 18:611-616

DOI: 10.12659/AJCR.903679

Available online: 2017-06-01

Published: 2017-06-01


BACKGROUND: Splenic marginal zone lymphomas (SMZLs) are generally uncommon, indolent lymphomas that typically affect older adults, but the development of the transformation to high-grade lymphoma may occur in a small proportion of patients and represents a rare event with blastic cell infiltration in the lymph nodes and bone marrow.
CASE REPORT: Here, we present a young adult patient who was diagnosed with a SMZL and developed a high-grade transformation to diffuse large B cell lymphoma (DLBCL) with central nervous system involvement. The patient was a 31-year-old woman whose hematologic medical history began with severe anemia and thrombocytopenia associated with atypical lymphoid infiltrate in the bone marrow and massive splenomegaly. A splenectomy was performed and revealed the SMZL. She was first treated with the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) and subsequently with the R-FMD regimen (rituximab, fludarabine, mitoxantrone, and dexamethasone) because the peripheral blood leukocytes were remarkably increased postoperatively. Six months after the splenectomy, she complained of headaches. A magnetic resonance imaging scan of her brain revealed intracerebral tumorous lesions from which a biopsy was taken. On morphological and immunohistochemical examination, the tumor fulfilled the criteria for a DLBCL. Treatment with pulse-dose intravenous methylprednisolone followed by high-dose methotrexate was promptly initiated, but the patient’s condition continued to deteriorate and she died of the disease 13 months after the splenectomy.
CONCLUSIONS: Although there is a general tendency for SMZL to display low aggressiveness, central nervous system involvement associated with a histological transformation to high-grade lymphoma, as presented here, can occur in advanced stage of the disease.

Keywords: Diagnosis, Lymphoma, B-Cell, Marginal Zone, Lymphoma, Large B-Cell, Diffuse