Splenic marginal zone lymphoma: a case report and literature review
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(2020) 18:259
CASE REPORT
Open Access
Splenic marginal zone lymphoma: a case report and literature review Shiyu Zhang1,2,3,4, Zefeng Xuan1,2,3,4, Liang Zhang1,2,3,4, Jiahua Lu1,2,3,4, Penghong Song1,2,3,4* and Shusen Zheng1,2,3,4*
Abstract Background: Splenic marginal zone lymphoma (SMZL) is a rare non-Hodgkin lymphoma, and much little is known about its clinical characteristics and management strategies. Here we present a case of SMZL and review relevant literature to provide a better recognition of this disease entity. Case presentation: A 49-year-old Chinese female was admitted to our hospital with complaints of abdominal distension and acid reflux. Physical examinations and imaging investigations suggested the presence of splenomegaly. Laboratory workups revealed mildly reduced white blood cell count otherwise was not remarkable. The patient underwent splenectomy. Histological examination combined with immunohistochemical analysis of the resected spleen confirmed the diagnosis of SMZL. The patient recovered uneventfully during follow-ups. Conclusions: Due to the rarity and unspecific clinical features, SMZL is extremely challenging to be diagnosed preoperatively. Patients with SMZL are generally associated with favorable prognosis. Combining the staging characteristics of non-Hodgkin’s lymphoma and splenic primary lymphoma may assist in clinical staging management of SMZL. Keywords: Splenic marginal zone lymphoma, Management, Staging, Case report
Background Lymphoma is a malignant tumor originating from the lymphatic hematopoietic system. According to the different cell sources, it is divided into non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL). Primary splenic lymphoma is a rare type of malignant lymphoma, which involves only the spleen and splenic hilar lymph nodes. However, splenic marginal zone lymphoma (SMZL) is even rarer. SMZL represents a rare chronic B lymphocyte proliferative disease, which only accounts for about 1–2% of nonHodgkin’s lymphoma [1]. The mean age of SMZL onset is about 65 years. There was no significant difference between men and women. About 25% of patients with early SMZL * Correspondence: [email protected]; [email protected] 1 The First Affiliated Hospital, Zhejiang University School of Medicine, Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Zhejiang Province, China Full list of author information is available at the end of the article
have no significant special discomfort; some people might only show the upper abdominal discomfort, such as abdominal pain and distention; and other patients may be accompanied by splenomegaly, emaciation, fatigue, weight loss, or other manifestations. The cell origin of SMZL remains not fully clear; some scholars believe that it originates from memory B lymphocytes in the marginal zone of secondary lymphoid follicles [2]. The pathogenesis of the disease is still in the process of exploration. Many studies believe that its pathogenesis may be closely related to 7q distortion [3]. Although it is an indole
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