Acquired aplastic anemia and Fanconi anemia - Brazilian Guidelines in Hematopoietic Stem Cell Transplantation. Rev. Bras. Hematol. Epub June 11, Year/Month, Html, Pdf, Epub, Total. , 0, 45, 0, April, 0, 40, 0, 40 . March, 1, 12, 0, February, 0, 4, 0, 4. January, 0, 1, 0, 1. Year/Month, Html, Pdf, Epub, Total. April, 0, 0, 0, 0. March, 0, 0, 0, 0. February, 0, 0, 0, 0. January, 0, 0, 0, 0. December, 0, 0, 0, 0.
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Acquired aplastic anemia (aAA) is a severe and rare disease, characterized by hematopoietic bone marrow failure and peripheral cytopenia. Int J Hematol. Jun;91(5) doi: /s Epub Jun 5. Clinical course of non-severe aplastic anemia in adults. Kwon JH(1) . Tratamento atual da anemia aplástica adquirida grave A sobrevida em pacientes com grave anemia aplástica melhorou muito nas .. Epub ahead of print.
Patient Handouts Summary Aplastic anemia is a rare but serious blood disorder. If you have it, your bone marrow doesn't make enough new blood cells. There are different types, including Fanconi anemia. Causes include Toxic substances, such as pesticides, arsenic, and benzene Radiation therapy and chemotherapy for cancer Certain medicines Infections such as hepatitis, Epstein-Barr virus, or HIV Autoimmune disorders Pregnancy In many people, the cause is unknown. Symptoms include fatigue, weakness, dizziness, and shortness of breath. It can cause heart problems such as an irregular heartbeat , an enlarged heart, and heart failure.
Bacigalupo et al. Hepatitis-Associated Aplastic Anemia: Hepatitis-associated aplastic anemia HAA is a variant of aplastic anemia in which aplastic anemia follows an acute seronegative hepatitis. This syndrome was first described in two patients in and from more than cases have been documented 47 , Development of AA is also described following orthotopic liver transplant LTx for viral seronegative hepatitis.
HAA syndrome has some typical clinical features: most often affects young males who presented severe pancytopenia two to three months after an episode of acute hepatitis. Hepatitis presents variability in clinical features but generally follows a benign course, showing a partial or complete resolution before AA onset. The bone marrow failure can be explosive and severe and it is usually fatal if untreated, no correlations have been observed between severity of hepatitis and AA.
The hepatitis was clinically indistin-guishable from a typical viral hepatitis, but in no study a specific virus was identified and most cases are seronegative for known hepatitis viruses, including A, B, C and G virus.
HGV and TTV have been suspected as agents of hepatitis but no study confirms this relationship 53 , All patients had evidence of severe AA and presented elevated liver enzyme levels. They were treated with immunosuppressive treatment which consisted of a four-day course of intravenous antithymocyte globulin and oral cyclosporine for 6 months, seven patients responded.
Liver enzyme returned to normal range within one month after therapy, hematologic recovery was slower, occurring after at least six months. At the presentation all patients had activated CD8 lymphocytes in peripheral blood, after immunosuppressive treatment CD8 cells decreased in all patients but one who did not respond, suggesting that liver and bone marrow associated damages are immune-mediated Osugi et al.
Another study assessed the efficacy of allogeneic bone marrow transplantation in patients with HAA Seventeen patients underwent BMT from HLA-matched donors, twelve were alive at mean follow up of 38 months; survival and transplant related mortality were similar to those patients transplanted for idiopathic AA, no cases of recurrent hepatitis occurred during the follow- up period.
A report from EBMT Severe Aplastic Anemia Working Party showed that previous hepatic damage from viral hepatitis and liver functions abnormalities did not appear to increase the toxicities from hepatocellular damage in the post transplant course The clinical characteristics and response to immunotherapy indicate a central role for immune-mediated mechanism in the pathogenesis of HAA.
The initial target organ of the immune response is the liver as suggested by the time interval between hepatitis and the onset of bone marrow failure. In acute B and C hepatitis and in autoimmune hepatitis liver histology is characterized by T cell infiltrating the parenchyma. Lu investigated the T cell repertoire of intrahepatic lymphocytes on HAA patients and reported that there is an infiltration of both clonal and many non clonal T cells with a T-cell repertoire similar to that of both viral B and C and autoimmune-hepatitis The expanded T cell clones return to a normal distribution after response to immunosuppressive treatment, suggesting the antigen or T cell clearance.
Conclusion: HAA is a life-threatening condition requiring prompt treatment. It can be considered a subset of AA as suggested by pathogenetic immune mediated mechanism and by response to immunosuppressive therapy.
The therapeutic options are allogeneic HSCT and IST, outcome and response rate are similar to those for patients with idiopathic acquired aplastic anemia.
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