Hepatitis Associated Aplastic Anemia: A review

Abstract

Hepatitis-associated aplastic anemia (HAAA) is an uncommon but distinct variant of aplastic anemia in which pancytopenia appears two to three months after an acute attack of hepatitis. HAAA occurs most frequently in young male children and is lethal if leave untreated. The etiology of this syndrome is proposed to be attributed to various hepatitis and non hepatitis viruses. Several hepatitis viruses such as HAV, HBV, HCV, HDV, HEV and HGV have been associated with this set of symptoms. Viruses other than the hepatitis viruses such as parvovirus B19, Cytomegalovirus, Epstein bar virus, Transfusion Transmitted virus (TTV) and non-A-E hepatitis virus (unknown viruses) has also been documented to develop the syndrome. Considerable evidences including the clinical features, severe imbalance of the T cell immune system and effective response to immunosuppressive therapy strongly present HAAA as an immune mediated mechanism. However, no association of HAAA has been found with blood transfusions, drugs and toxins. Besides hepatitis and non hepatitis viruses and immunopathogenesis phenomenon as causative agents of the disorder, telomerase mutation, a genetic factor has also been predisposed for the development of aplastic anemia. Diagnosis includes clinical manifestations, blood profiling, viral serological markers testing, immune functioning and bone marrow hypocellularity examination. Patients presenting the features of HAAA have been mostly treated with bone marrow or hematopoietic cell transplantation from HLA matched donor, and if not available then by immunosuppressive therapy. New therapeutic approaches involve the administration of steroids especially the glucocorticoids to augment the immunosuppressive therapy response. Pancytopenia following an episode of acute hepatitis response better to hematopoietic cell transplantation than immunosuppressive therapy.

Nonregenerative Anemia

“…Deficiencies in cortisol and androgens 93 GI hemorrhage 87 Diabetes mellitus Inflammation 187 GI dysfunction 155 Renal injury (diabetic nephropathy) 196 Relative EPO deficiency secondary to neuropathology 21 Destabilization and degradation of hypoxia-inducible factor-1a secondary to hyperglycemia 32 Decreased iron availability secondary to glycation of transferrin 59 Hepatic disease GI hemorrhage secondary to portal hypertension Inflammation Certain viral diseases 140 In patients with portovascular anomalies, the mechanism of anemia is unknown, but iron sequestration causing a functional iron deficiency is suspected 156 Nutritional and GI disease Iron deficiency secondary to chronic GI blood loss (eg, strongylid infection, 75 multiple cytopenias, FeLV-C is the causative agent of FeLVassociated PRCA. The host's cellular receptor for FeLV-C (which binds to the FeLV-C's unique surface envelope glycoprotein) may be necessary for erythropoiesis.…”

Diagnosis of acquired aplastic anemia

“…[31][32][33][34][35] It typically occurs in young, healthy males with severe but self-limited liver inflammation. A common inciting infectious cause could be involved both in the liver disease and in the BM failure.…”

Hepatitis E virus-associated aplastic anaemia: The first case of its kind

“…3,4 Aplastic anaemia (AA) complicating hepatitis is a rare but well-documented phenomenon and there are a number of reports in the literature describing such cases with AA. 5,6 Hepatitis-associated aplastic anaemia (HAAA) is a severe disorder with a high mortality rate (85%). 7 HAAA is a variant of AA in which AA follows an acute attack of hepatitis.…”