Severe acute anemia attributable to concomitant occurrence of AIHA with PRCA induced by parvovirus B19 infection

Abstract

A 10-year-old, previously healthy girl presented with asthenia and fever of 3 days' duration. Initial investigations revealed mild leukopenia and severe hemolytic anemia (hemoglobin 5 4.9 mg/dL) with a markedly positive direct Coombs test. However, the absolute reticulocyte count was unexpectedly low, necessitating a bone marrow aspiration. It revealed marked hypoplasia of erythroid lineage that was mainly represented by scattered giant proerythroblasts showing intranuclear inclusions highly suggestive of underlying parvovirus B19 infection (panel A; May-Grunwald Giemsa stain, original magnification 31000). Activated macrophages showing hemophagocytosis were also noted as highlighted on the iron stain preparation (panel B; Perls' Prussian blue stain, original magnification 31000). After common causes of infection and hemolysis were ruled out, the diagnosis was confirmed by serology showing significantly increased anti-human parvovirus B19 immunoglobulin M and by qualitative polymerase chain reaction performed on a bone marrow sample. Therefore, the patient was diagnosed with autoimmune hemolytic anemia (AIHA) associated with pure red cell aplasia (PRCA) caused by parvovirus B19 infection. She was treated with steroids and intravenous immunoglobulin and had a complete recovery.PRCA induced by parvovirus infection is a well-known entity. However, concomitant occurrence of AIHA has been rarely reported in such a context. This association should be considered when a patient presents with a severe acute anemia in the absence of a known cause of shortened erythrocyte lifetime.For additional images, visit the ASH Image Bank, a reference and teaching tool that is continually updated with new atlas and case study images. For more information, visit http://imagebank.hematology.org.

New Insights in Autoimmune Hemolytic Anemia: From Pathogenesis to Therapy

“…During infections, there is the so-called "molecular mimicry", (i.e., a cross-reaction between antigen determinants of the infectious agent and self molecules). Several infections have been associated with AIHA (parvovirus B19, hepatotropic virus, HIV, mycoplasma pneumonia, mycobacterium tuberculosis, brucella, syphilis) [6,[31][32][33][34][35][36] including, more recently, COVID-19 pneumonia [37][38][39]. Drugs can induce the production of autoantibodies by modifying the RBC membrane by adsorption to the membrane or by binding to the membrane as immunocomplexes.…”

Pure red cell aplasia accompanied by COVID-19 successfully treated using cyclosporine

“…The pathogenesis of PRCA is idiopathic in 39% of cases, thymoma in 23%, and lymphoproliferative disorder in 14% [4]. In this case, we excluded infections with B19 parvovirus [5], acute hepatitis A [6], human immunodeficiency virus, human T-cell leukemia virus type 1, and Epstein-Barr virus based on serological tests and PCR. As the p-antigen on RBC progenitors is known to act as the receptor for B19 parvovirus, we speculate that cytotoxic T cells might destroy erythroid progenitors in the bone marrow [7].…”