Systematic review: hepatitis‐associated aplastic anaemia – a syndrome associated with abnormal immunological function

Abstract

SUMMARY BackgroundHepatitis-associated aplastic anaemia is a syndrome in which marrow failure follows the development of hepatitis.

Spectrum of anemia associated with chronic liver disease

“…The main feature of this syndrome is injury to or loss of pluripotent hematopoietic stem cells, in the absence of infiltrative disease of the bone marrow [16][17][18][19] . Hepatitis-associated aplastic anemia (HAA) has been defined as a variant of aplastic anemia, which occurs concurrently with or within 6 mo of an increase in the serum level of alanine aminotransferase to at least five times the upper limit of the reference range.…”

“…Aplastic anemia associated with liver disease is characterized by development of pancytopenia and hypocellular bone marrow in relation to the occurrence of hepatitis [16] . The main feature of this syndrome is injury to or loss of pluripotent hematopoietic stem cells, in the absence of infiltrative disease of the bone marrow [16][17][18][19] .…”

“…Hepatitis-associated aplastic anemia (HAA) has been defined as a variant of aplastic anemia, which occurs concurrently with or within 6 mo of an increase in the serum level of alanine aminotransferase to at least five times the upper limit of the reference range. Severe marrow aplasia may be induced by hepatitis viruses, such as hepatitis B virus and hepatitis C virus (HCV), and also by other viruses, such as human immunodeficiency virus, Epstein-Barr virus, transfusion-transmitted virus and echovirus [16,20] . Parvovirus B19 commonly infects pro-erythroblasts and may induce transient red-cell aplasia, particularly in patients with chronic hemolytic anemia.…”

“…The two major options for treating severe HAA are hematopoietic cell transplantation and immunosuppressive therapy. According to recent reviews, response rates to these approaches are 75%-88% and 75%-80%, respectively [16,18] . Blood and platelet infusions are often necessary before instituting specific treatment; before administration blood products should be irradiated to avoid sensitization.…”

“…Clinical presentation includes symptoms and signs related to pancytopenia, such as pallor, fatigue, hemorrhagic manifestations, progressive anemia, and bacterial infections. The diagnosis of HAA is suggested by a complete blood count, which reveals pancytopenia (including anemia) together with absolute reticulocytopenia [16] . A bone marrow biopsy typically reveals hypocellularity that affects red and white cell precursors and megakaryocytes; residual hematopoietic cells appear morphologically normal [19] .…”