Pure Red Cell Aplasia Responsive to Interferon-α in a Patient with Hepatitis C Virus Infection

Abstract

A 51-year-old man presented with severe anemia, mild splenomegaly and elevated serum aspartate aminotransferase and serum alanine aminotransferase levels. The bone marrow findings were consistent with pure red cell aplasia (PRCA) with a ‘maturation arrest’ at the level of pronormoblast. The patient has been transfusion-dependent for 8 months. Following diagnosis of chronic active hepatitis due to hepatitis C virus (HCV), therapy with interferon-α was initiated. Two weeks later, the hemoglobin level stabilized, and he has not required any transfusion ever since. In spite of ongoing HCV viremia, cessation of interferon therapy, and deterioration of the liver function tests, the patient, followed for 2 years, maintains a high-normal hemoglobin level. To the best of our knowledge, this is the first report of prolonged PRCA corrected by interferon-α therapy, with or without an ongoing HCV infection. We speculate that the ‘maturation arrest’ of the erythroid lineage seen in the bone marrow was the result of an immune mechanism, possibly induced by the HCV, and that the elimination of this mechanism, rather than the elimination of the HCV, provided the opportunity for regeneration of erythropoiesis.

Pure red cell aplasia caused by ribavirin and interferon treatment

“…The bone marrow of afflicted individuals has numerous giant pronormoblasts and few mature erythroid progenitors. The result is a transient period of asymptomatic aplasia in the majority of infected individuals, but immunosuppressed patients and those with chronic hemolytic anemia may develop life-threatening aplastic crises [18]. Various other infections including HCV, hepatitis A and B, HIV, CMV, and disseminated tuberculosis have been associated with development of PRCA [19][20][21][22][23][24][25].…”

Anti-erythropoietin antibody–mediated pure red cell aplasia in a living donor liver transplant recipient treated for hepatitis C virus

“…This phenomenon is even more striking when coupled with the observation that although there are no standard guidelines for the treatment of anti‐EPO antibody–mediated PRCA, immunosuppressive medications are often the mainstay of therapy 22. Retrospective data from European centers have documented varying levels of clinical recovery in patients with PRCA treated with combinations of corticosteroids, cyclophosphamide, intravenous immunoglobulin, cyclosporin, rituximab, or mycophenolate mofetil 23. As an interesting contrast to our case, IFN has been used successfully in the treatment of idiopathic pure red cell aplasia 24…”

A sustained response to low dose interferon‐α in a case of refractory pure red cell aplasia

“…In our patient, INF‐ α induced rapid remission and a sustained normal hematocrit. To date, there has been one other case report of PRCA responsive to INF‐ α therapy, in a patient with hepatitis C infection (12). The patient's PRCA went into lasting remission, and the authors speculated an immune mechanism separate from his hepatitis.…”

“…Furthermore, Davidovitz et al. (12), observed correction of anemia in a patient with PRCA and hepatitis C after IFN‐ α therapy. We describe here another patient with refractory PRCA in whom IFN‐ α therapy led to a normal hematocrit and transfusion independence.…”