title: 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.
abstract: 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.
authors.affiliation: Department of Hematology and Oncology, Oji General Hospital, 3-4-8 Wakakusa-Cho, Tomakomai, 053-8506, Japan, akihito.fujimi@ojihosp.or.jp.
authors.forename: Akihito
authors.initials: A
authors.lastname: Fujimi
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Kamihara
authors.forename: Yuji
authors.initials: Y
authors.lastname: Kanisawa
authors.forename: Akari
authors.initials: A
authors.lastname: Hashimoto
authors.forename: Chisa
authors.initials: C
authors.lastname: Nakajima
authors.forename: Naotaka
authors.initials: N
authors.lastname: Hayasaka
authors.forename: Naoki
authors.initials: N
authors.lastname: Uemura
authors.forename: Toshinori
authors.initials: T
authors.lastname: Okuda
authors.forename: Shinya
authors.initials: S
authors.lastname: Minami
authors.forename: Satoshi
authors.initials: S
authors.lastname: Iyama
authors.forename: Koichi
authors.initials: K
authors.lastname: Takada
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Sato
authors.forename: Akinori
authors.initials: A
authors.lastname: Hara
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Iwata
authors.forename: Kengo
authors.initials: K
authors.lastname: Furuichi
authors.forename: Takashi
authors.initials: T
authors.lastname: Wada
authors.forename: Junji
authors.initials: J
authors.lastname: Kato
mesh_terms: D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000276:immunology*; D000911:Antibodies, Monoclonal / Q000009:adverse effects*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D005260:Female; D019788:Fluorodeoxyglucose F18; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D009367:Neoplasm Staging; D049268:Positron-Emission Tomography; D017467:Receptors, Erythropoietin / Q000037:antagonists & inhibitors*; D012010:Red-Cell Aplasia, Pure / Q000139:chemically induced* / Q000150:complications* / Q000188:drug therapy; D013601:T-Lymphocytes / Q000473:pathology; D014057:Tomography, X-Ray Computed
chemical_list: D000911:Antibodies, Monoclonal; D017467:Receptors, Erythropoietin; D019788:Fluorodeoxyglucose F18
references.citation: Int J Hematol. 2012 Nov;96(5):594-9
references.pmid: 23099924
references.citation: Leuk Lymphoma. 2007 Jun;48(6):1139-49
references.pmid: 17577777
references.citation: Acta Haematol. 2005;113(3):194-7
references.pmid: 15870490
references.citation: Am J Hematol. 2009 Feb;84(2):98-101
references.pmid: 19105232
references.citation: Leuk Lymphoma. 2003 May;44(5):889-90
references.pmid: 12802933
references.citation: Haematologica. 2007 Aug;92(8):1021-8
references.pmid: 17640861
references.citation: Am J Hematol. 2009 Mar;84(3):144-8
references.pmid: 19195037
references.citation: Leuk Lymphoma. 1995 Jul;18(3-4):311-6
references.pmid: 8535198
references.citation: Br J Haematol. 1987 Oct;67(2):235-9
references.pmid: 3499930
references.citation: Semin Hematol. 2010 Apr;47(2):124-32
references.pmid: 20350659
references.citation: Br J Haematol. 2000 Dec;111(4):1010-22
references.pmid: 11167735
references.citation: Leuk Res. 1990;14(3):279-86
references.pmid: 1690829
references.citation: Blood. 2008 Feb 15;111(4):1820-6
references.pmid: 18055869
references.citation: Pediatr Blood Cancer. 2006 Oct 15;47(5 Suppl):706-9
references.pmid: 16933263
references.citation: N Engl J Med. 1999 Jan 28;340(4):278-84
references.pmid: 9920952
references.citation: Br J Haematol. 2013 Jan;160(2):244-50
references.pmid: 23151030