Hepatitis-Associated Aplastic Anemia: Etiology, Clinical Characteristics and Outcome

Abstract

Hepatitis-associated aplastic anemia (HAA) is a form of acquired aplastic anemia (AA) in which bone marrow failure develops after an acute attack of hepatitis. Bone marrow failure leading to AA is generally severe in cases of HAA and fatal if left untreated. This retrospective multicenter study investigated clinical and laboratory characteristics, possible causes, treatment, and outcome of HAA in children. Twenty patients from 8 centers were included in the study. Aspartate aminotransferase and alanine aminotransferase were < 3 to 5×upper limit of normal (ULN) in 2 patients, < 5 to 10×ULN in 2 patients, and > 10×ULN in 16 patients. Acute liver failure developed in 5 (29%) patients. Pancytopenia was simultaneously present in 6 of 20 (30%) patients. Eleven of the 20 patients (55%) were alive, in remission and transfusion free. Those who were alive either had undergone hematopoietic stem cell transplantation and/or immunosuppressive treatment, except 1 patient who had received no treatment. Patients with the diagnosis of acute hepatitis should be evaluated and followed up carefully for presence of cytopenia, so that definitive treatment of AA can be initiated in a timely and appropriate manner when needed.

Identifying the Aetiology of Acute Liver Failure Is Crucial to Impact Positively on Outcome

“…According to standardised protocols, all patients were considered for allogeneic hematopoietic stem cell transplantation, which was available only in 1 patient. The other 5 patients were treated with immunosuppressive therapy; at last follow up they are alive and well [ 18 ].…”

“…Of interest, in this cohort, 16% of patients with ID-ALF (all included in G2) developed AA by 90 days after the diagnosis of ALF. Aplastic anaemia was diagnosed by bone marrow biopsy in all (100%), and it was classified as severe AA according to the standard classification [ 18 ].…”

“…Aplastic anaemia . The diagnosis was made according to standardised criteria following bone marrow aspiration and bone biopsy [ 18 ].…”

Toxic hepatitis-associated aplastic anaemia after dual homeopathic remedies and Gymnema sylvestre use

“…Various immune mechanisms triggered by various acute insult have been elucidated as the cause of bone marrow failure following an episode of severe acute hepatitis. These include increased activation and a broad skewing pattern of T cell fractions such as circulating cytotoxic T cells which infiltrate and accumulate in the liver (with notable inflammation on liver biopsy), defective monocyte to macrophage differentiation, CD8+ Kupffer cell-mediated liver and bone marrow toxicity, increase in interferon-gamma and interleukin-2-mediated organ damage in liver and bone marrow—all of which are driven by hapten/antibody-mediated inflammatory cascade after the initial insult 16 17. Similar immune mechanisms, immune dysfunction and adaptive and innate immune changes (direct or indirect toxicity due to antibody or secondary metabolite generation) have been described in DILI, possibly explaining common pathophysiological mechanisms that result in HAAA syndrome development 18–20.…”

Suspected Adenovirus Causing an Emerging HEPATITIS among Children below 10 Years: A Review

“…The virus begins to block host macromolecular synthesis and mRNA transport to the cytoplasm after many replication cycles. These cellular disruptions damage host cells and may produce symptoms of disease in their hosts, such as respiratory stress [ 35 ]. Studies have suggested that the penton protein of adenoviruses seems to be virulent, in addition to the symptoms caused by cell lysis and the inhibition of cellular synthesis.…”