Gastric mucosa-associated lymphoid tissue lymphoma complicated with hemophagocytic syndrome in an elderly woman

Abstract

Dear Editor, Hemophagocytic syndrome (HPS) has been described in association with a variety of lymphomas, most commonly Tor NK-cell-type tumors [1]. More rarely, HPS occurs in association with aggressive B-cell tumors, including diffuse large B-cell lymphoma and intravascular large B-cell lymphoma [2, 3]. Although these lymphoma-associated forms of HPS generally show a poor prognosis [1], appropriate therapy for the underlying lymphoma can lead to the resolution of this condition [2]. Here, we report an elderly patient with mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach, who developed HPS during the course of the disease. An 80-year-old woman, who had been treated for cognitive disorder in a psychiatric clinic, presented with upper abdominal discomfort. Endoscopy of the upper gastrointestinal tract revealed multiple mucosal lesions from the lower body to the antrum of the stomach, including superficial erosions/ulcers and “gastritis-like” erythema (Fig. 1a). A biopsy demonstrated mucosal infiltrates of small lymphoid cells showing a monocytoid appearance associated with lymphoepithelial lesions, being compatible with gastric MALT lymphoma (Fig. 1b). Tests for Helicobacter pylori were positive. Triple therapy comprising lansoprazole, amoxicillin, and clarithromycin was initiated; however, the patient soon discontinued treatment due to the development of an allergic skin rash. As the patient’s symptoms persisted, she was finally admitted to our hospital 4 months after the initial presentation. On examination, she was febrile and icteric, and the abdomen showed hepatosplenomegaly. There was no superficial lymphadenopathy. The blood counts were: hemoglobin, 11.5 g/dl; white blood cell count, 2,500/μl with 86% neutrophils; and platelet count, 117×10/μl. The blood chemistry values included: total bilirubin, 9.2 mg/dl; asparate aminotransferase, 1,446 IU/l; alanine aminotransferase, 387 IU/l; alkaline phosphatase, 1,439 IU/l; lactate dehydrogenase, 1,851 IU/l; and γ-glutamyl transpeptidase, 143 IU/l. The level of triglyceride was 294 mg/dl, fibrinogen—83 mg/dl, fibrinogen/fibrin degradation product—15.5 μg/ml, ferritin—2,929 ng/ml, and soluble interleukin-2 receptor was as high as 17,388 U/ml. A computed tomographic scan and ultrasonography of the abdomen revealed hepatosplenomegaly and wall thickening of the gall bladder (Fig. 1c); the latter finding suggested portal cellular infiltration [4]. Bone marrow specimens obtained from the iliac crest revealed hypocellular marrow with increased numbers of macrophages ingesting blood cells (Fig. 1d); no lymphoma cells were apparent on microscopic examination or flow cytometric analysis. A diagnosis of gastric MALT lymphoma complicated with HPS was made. The patient was treated with chemotherapy consisting of vincristine, cyclophosphamide, prednisolone, and doxorubicin, in combination with rituximab. HPS responded well to the treatment; H. Ohno (*) Department of Hematology, Takeda General Hospital, 28-1, Mori-minami-cho, Ishida, Fushimi-ku, Kyoto 601-1495, Japan e-mail: hitoshi-ono@fm2.seikyou.ne.jp