Pure red cell aplasia associated with cytomegalovirus and Epstein–Barr virus infection in seven cases of Chinese children

Abstract

Pure red cell aplasia (PRCA) associated with cytomegalovirus (CMV) and Epstein-Barr virus (EBV) infection is uncommon. Here, we describe the clinical features and management of seven cases of Chinese children with PRCA associated with viral infections. The patients presented with pallor on admission. Blood cell counts and marrow smears showed anemia, reticulocytopenia, and aplasia of erythroblasts. Serological investigation and DNA polymerase chain reactions for CMV were positive in four patients and those tests for EBV were positive in other three patients. All patients received blood transfusion, corticosteroids treatment, and ganciclovir injection. Two patients had a complete response and one had a partial response after the treatments. The other three patients had a complete response to second-line therapies, including high-dose methylprednisolone, cyclosporin A, and intravenous immunoglobulin. Only one patient had no response to the therapies. Our results indicated that it might be important to combine immunosuppressive drugs with an antiviral drug in the management of PRCA associated with CMV and EBV infection.

A Concise Review of Autoimmune Cytopenias in Chronic Lymphocytic Leukemia

“…10,31 PRCA can also be associated with viral infections, such as EBV, CMV, 54 and human parvovirus B19. 55 In a large case series of PRCA in CLL (n=30), parvovirus testing was performed in 24 patients and was noted to be positive in 6 (25%) patients.…”

Successful treatment of tacrolimus‐related pure red cell aplasia and autoimmune hemolytic anemia with rituximab in a pediatric cardiac transplant patient

“…The generation of these antibodies in patients on immunosuppressants is thought to occur through immune dysregulation . Although CMV could be a potential cause of PRCA in this patient, the timing of the PRCA and the absence of significant viremia, which have been described in other cases, make CMV less likely …”

“…PRCA occurring in association with AIHA has been reported in association with hepatitis A, immunosuppressive therapy, lupus, lymphoma, Sjogren's syndrome, CMV, and EBV infection and in children with no underlying medical problems . The interval between onset of PRCA and AIHA varies widely from simultaneous occurrence to 4 years between PRCA and AIHA .…”

Combining an antiviral with rituximab in EBV-related haemophagocytic lymphohistiocytosis led to rapid viral clearance; and a comprehensive review

“…In terms of question (2), several antivirals, including rituximab, have been used for a number of conditions; for example, cases of uveitis (valganciclovir),47 interstitial pneumonitis with pancytopenia (acyclovir),48 cutaneous amyloidosis (acyclovir),49 vasculitis (eg, of CNS and or eye—acyclovir),50 51 keratoconjunctivitis (acyclovir),52 interstitial nephritis (acyclovir),53 erythema multiforme (acyclovir),54 acute retinal necrosis (acyclovir),55 transverse myelitis (ganciclovir),56 oesophagitis (acyclovir),57 epithelial cell tumours and haematological malignancies(ganciclovir),58 59 other malignancies (cidofovir)60 and other blood dyscrasias (ganciclovir) 61. A 2010 review reported favourable outcomes of antiviral treatment in most immunocompetent patients with severe manifestations (mainly neurological: meningoencephalitis, cerebellitis, Guillain-Barre, facial nerve palsy, myeloradiculitis) in which antivirals had been used (mainly acyclovir, along with steroids) 62.…”