Anti-erythropoietin receptor antibody-associated pure red cell aplasia accompanied by Coombs-negative autoimmune hemolytic anemia in a patient with T cell/histiocyte-rich large B cell lymphoma

• Published in: International journal of hematology 2014-11, Vol.100 (5), p.490-493
• Main Authors: Fujimi, Akihito, Kamihara, Yusuke, Kanisawa, Yuji, Hashimoto, Akari, Nakajima, Chisa, Hayasaka, Naotaka, Uemura, Naoki, Okuda, Toshinori, Minami, Shinya, Iyama, Satoshi, Takada, Koichi, Sato, Tsutomu, Hara, Akinori, Iwata, Yasunori, Furuichi, Kengo, Wada, Takashi, Kato, Junji
• Format: Article
• Language: English
• Subjects: Aged; Anemia, Hemolytic, Autoimmune - complications; Anemia, Hemolytic, Autoimmune - immunology; Anemias. Hemoglobinopathies; Antibodies, Monoclonal - adverse effects; Antineoplastic Combined Chemotherapy Protocols - adverse effects; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Case Report; Diseases of red blood cells; Female; Fluorodeoxyglucose F18; Hematologic and hematopoietic diseases; Hematology; Histiocytes - pathology; Humans; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, Large B-Cell, Diffuse - complications; Lymphoma, Large B-Cell, Diffuse - diagnosis; Lymphoma, Large B-Cell, Diffuse - drug therapy; Lymphoma, Large B-Cell, Diffuse - pathology; Medical sciences; Medicine; Medicine & Public Health; Neoplasm Staging; Oncology; Parvovirus B19; Positron-Emission Tomography; Receptors, Erythropoietin - antagonists & inhibitors; Red-Cell Aplasia, Pure - chemically induced; Red-Cell Aplasia, Pure - complications; Red-Cell Aplasia, Pure - drug therapy; T-Lymphocytes - pathology; Tomography, X-Ray Computed
• ISSN: 0925-5710; 1865-3774
• DOI: 10.1007/s12185-014-1652-5

A 79-year-old female diagnosed with T cell/histiocyte-rich large B cell lymphoma in complete remission after six cycles of rituximab-combined chemotherapy developed severe anemia, reticulocytopenia, and bone marrow erythroid hypoplasia. She was diagnosed with pure red cell aplasia (PRCA) accompanied by Coombs-negative autoimmune hemolytic anemia evidenced by a lack of glycophorin-A-positive cells in the bone marrow, haptoglobin under the detection level, and a high titer of RBC-bound IgG. Anti-erythropoietin receptor (EPOR) antibody was detected in the serum, and oligoclonal α/β and γ/δ T cells were also detected in her peripheral blood by Southern blotting analysis. Parvovirus B19 DNA was not detected by PCR. Although the treatment with rituximab had limited efficacy (specifically, only for hemolysis), subsequent cyclosporine therapy led to prompt recovery of erythropoiesis with the disappearance of anti-EPOR antibody and oligoclonal T cells. This is the first case report of anti-EPOR antibody-associated PRCA in a patient with malignant lymphoma treated successfully with cyclosporine.