title: A case of recurrent autoimmune hemolytic anemia during remission associated with acute pure red cell aplasia and hemophagocytic syndrome due to human parvovirus B19 infection successfully treated by steroid pulse therapy with a review of the literature.
abstract: The patient was a 47-year-old man diagnosed as having autoimmune hemolytic anemia (AIHA) in April 2011. He also had a congenital chromosomal abnormality, a balanced translocation. Treatment with prednisolone (PSL) 60 mg/day resulted in resolution of the AIHA, and the treatment was completed in November 2011. While the patient no longer had anemia, the direct and indirect Coombs tests remained positive. In May 2013, he developed recurrent AIHA associated with acute pure red cell aplasia (PRCA) and hemophagocytic syndrome (HPS) caused by human parvovirus B19 (HPV B19) infection. Tests for anti-erythropoietin and anti-erythropoietin receptor antibodies were positive. Steroid pulse therapy resulted in resolution of the AIHA, PRCA, as well as HPS. The serum test for anti-erythropoietin antibodies also became negative after the treatment. However, although the serum was positive for anti-HPV B19 IgG antibodies, the patient continued to have a low CD4 lymphocyte count (CD4, <300/μL) and persistent HPV B19 infection (HPV B19 DNA remained positive), suggesting the risk of recurrence and bone marrow failure.
authors: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors: Yasunobu
authors: Y
authors: Sekiguchi
authors: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors: Asami
authors: A
authors: Shimada
authors: Shibanishi Clinic Japan.
authors: Hidenori
authors: H
authors: Imai
authors: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors: Mutsumi
authors: M
authors: Wakabayashi
authors: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors: Keiji
authors: K
authors: Sugimoto
authors: Department of Clinical Laboratory, Juntendo University Urayasu Hospital Japan.
authors: Noriko
authors: N
authors: Nakamura
authors: Department of Clinical Laboratory, Juntendo University Urayasu Hospital Japan.
authors: Tomohiro
authors: T
authors: Sawada
authors: Department of Hematology, Juntendo University Hospital Japan.
authors: Norio
authors: N
authors: Komatsu
authors: Department of Hematology, Juntendo University Urayasu Hospital Japan.
authors: Masaaki
authors: M
authors: Noguchi
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D015415:Biomarkers / Q000097:blood; D001856:Bone Marrow Examination; D018791:CD4 Lymphocyte Count; D002648:Child; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D008297:Male; D008875:Middle Aged; D010322:Parvoviridae Infections / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D016732:Parvovirus B19, Human / Q000276:immunology / Q000472:pathogenicity*; D011239:Prednisolone / Q000008:administration & dosage*; D020551:Pulse Therapy, Drug; D012008:Recurrence; D012010:Red-Cell Aplasia, Pure / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D012074:Remission Induction; D012698:Serologic Tests; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; D011239:Prednisolone
keywords: Autoimmune hemolytic anemia; CD4 lymphocyte count; hemophagocytic syndrome; human parvovirus B19; pure red cell aplasia