Primary ovarian diffuse large B-cell lymphoma preceded by autoimmune hemolytic anemia

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Primary ovarian diffuse large B-cell lymphoma preceded by autoimmune hemolytic anemia Satoshi Ichikawa 1 Hideo Harigae 1

&

Asami Sasaoka 1 & Noriko Fukuhara 1 & Yoko Okitsu 1 & Yasushi Onishi 1 & Ryo Ichinohasama 2 &

Received: 15 July 2019 / Accepted: 15 October 2019 # Springer-Verlag GmbH Germany, part of Springer Nature 2019

Abstract A 62-year-old woman was diagnosed with autoimmune hemolytic anemia (AIHA), with cytology- and culture-negative pleural effusion. AIHA did not improve with steroid therapy. Computerized tomography (CT) detected a left ovarian tumor, which was resected surgically, and AIHA improved temporarily. Pathological examination yielded a diagnosis of diffuse large B-cell lymphoma (DLBCL). Positron emission tomography with CT suggested systemic infiltration of lymphoma. Rituximabcontaining chemotherapy (R-CHOP regimen) yielded rapid decreases in lymphoma lesions and pleural effusion with improvement of AIHA. After six courses of R-CHOP, complete response (CR) was confirmed and AIHA remitted completely. Two months after confirmation of CR, however, lymphoma and AIHA relapsed simultaneously. To our knowledge, this is the first case report describing ovarian DLBCL preceded by AIHA. Keywords Autoimmune hemolytic anemia . Diffuse large B-cell lymphoma . Primary ovarian lymphoma

Introduction Autoimmune hemolytic anemia (AIHA) is a disorder caused by destruction of red blood cells by autoantibodies and may be either a primary or a secondary disease. Non-Hodgkin’s lymphomas (NHL), such as chronic lymphocytic leukemia (CLL) or angioimmunoblastic T-cell lymphoma (AITL), are wellknown causes of secondary AIHA; diffuse large B-cell lymphoma (DLBCL) is a rare histopathological subtype associated with AIHA [1, 2]. Case series describing the clinicopathological features of NHL with AIHA have been reported [3, 4]. The diagnosis of AIHA was shown to often precede diagnosis of NHL, at diagnosis the disease is often at an advanced stage, and DLBCL associated with AIHA is refractory to conventional chemotherapy and has poor prognosis. Primary ovarian lymphoma is a rare disease, although secondary ovarian invasion by lymphoma is not uncommon [5]. * Satoshi Ichikawa [email protected] 1

Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-cho Sendai 980-8574 Japan

2

Department of Hematopathology, Tohoku University Hospital, Sendai Japan

According to a report aggregating data regarding primary ovarian lymphoma, DLBCL is the most common histological subtype [6]. In addition, it has been suggested that primary ovarian DLBCL could be an aggressive disease with poor prognosis [7, 8]. Here, we report a case of AIHA preceding a diagnosis of primary ovarian DLBCL. We reached a diagnosis of DLBCL through searching for the cause of treatment-resistant AIHA. The importance of investigation of underlying diseases should be emphasized in such cases of refractory AIHA. To our knowledge, this is the first case report describing ovarian DLBCL which was preceded by AIHA