Nalazite se na CroRIS probnoj okolini. Ovdje evidentirani podaci neće biti pohranjeni u Informacijskom sustavu znanosti RH. Ako je ovo greška, CroRIS produkcijskoj okolini moguće je pristupi putem poveznice www.croris.hr
izvor podataka: crosbi

Transitory aplastic anemia following acute autoimmune hepatitis in a young boy (CROSBI ID 719110)

Prilog sa skupa u časopisu | sažetak izlaganja sa skupa | međunarodna recenzija

Bakoš, Matija ; Ille, Vanja ; Kovačić, Matea ; Marčinković, Nedo ; Petrović Gluščić, Ana ; Kelečić, Jadranka ; Tešović, Goran ; Aničić, Mirna Natalija ; Omerza, Lana ; Senečić Čala, Irena et al. Transitory aplastic anemia following acute autoimmune hepatitis in a young boy // Archives of disease in childhood. 2021. str. A98-A98 doi: 10.1136/archdischild-2021-europaediatrics.233

Podaci o odgovornosti

Bakoš, Matija ; Ille, Vanja ; Kovačić, Matea ; Marčinković, Nedo ; Petrović Gluščić, Ana ; Kelečić, Jadranka ; Tešović, Goran ; Aničić, Mirna Natalija ; Omerza, Lana ; Senečić Čala, Irena ; Tješić Drinković, Duška ; Vuković, Jurica

engleski

Transitory aplastic anemia following acute autoimmune hepatitis in a young boy

Introduction Acute hepatitis associated aplastic anemia (AHAAA) is a rare condition in which acute hepatitis is complicated by development of aplastic anemia. It is more common in young males, and presents with pancytopenia 2-3 months after an episode of acute hepatitis. The etiology of hepatitis mostly remains unknown and it is thought that aplastic anemia is caused by immune dysregulation provoked by perhaps infection triggered cytokine production that injures hematopoetic stem cells. Options for treating severe AHAAA are bone marrow transplantation or immunosuppressive therapy (corticosteroids, cyclosporin A, antithymocyte globulin, antilymphocite globulin). Case Report We present a case of a previously healthy 10-year old boy, admitted due to sudden onset of jaundice and nausea. Laboratory results showed highly elevated aminotransferase (AST 2089 U/L, ALT 3228 U/L), conjugated hyperbilirubinemia (total bilirubin 391 umol/L, conjugated 302 umol/L) and initially preserved synthetic liver function. Extensive workup excluded infectious, toxic and metabolic causes. Hypergammaglobulinemia was not present, but anti smooth muscle antibodies were positive in two consecutive testing (1:20). Liver biopsy showed unspecific mixed type acute inflammation. In the next few days he developed liver failure (INR 2.6) so immunosuppressive therapy for autoimmune hepatitis (AIH) was initiated (corticosteroids and azathioprine), together with symptomatic therapy (fresh frozen plasma).Due to potential adverse toxic effect (decreased activity of the enzyme thiopurinmethyltransferase) azathioprin was changed to mycophenolate mofetil. In the following weeks patient’s synthetic liver function completely recovered and aminotransferase and bilirubin levels improved. Six weeks after initial onset of his symptoms patient developed severe thrombocytopenia and leukopenia followed by mild anemia. Bone biopsy showed hypoplastic to aplastic bone marrow. Screening for opportunistic pathogens revealed positive Pneumocystis carinii, and also CMV and VZV reactivation so treatment with trimethoprim-sulfametoxazole and valganciclovir was started together with antifungal therapy for oral candidiasis. He also required repeated platelet transfusions. After two months his bone marrow started showing signs of recovery and he was discharged from hospital. At last follow up, his leukocyte counts was 4.3 x 109/L, hemoglobin 142 g/L and platelet count 57 x 109/L. Discussion Our patient followed the typical presentation of AHAAA described in the scarce literature. The notable aspect of our case is potentially spontaneous recovery of bone marrow aplasia without the need for bone marrow transplantation. Mycophenolate mofetil might be viable therapeutic step in treatment for this type of disease.

autoimmune hepatitis ; aplastic anemia ; child

nije evidentirano

nije evidentirano

nije evidentirano

nije evidentirano

nije evidentirano

nije evidentirano

Podaci o prilogu

A98-A98.

2021.

nije evidentirano

objavljeno

10.1136/archdischild-2021-europaediatrics.233

Podaci o matičnoj publikaciji

Archives of disease in childhood

0003-9888

1468-2044

Podaci o skupu

10th Congress of European Paediatric Association EPA/UNEPSA jointly held with 14 th Congress of Croatian Paediatric Society

poster

07.09.2021-09.10.2021

Zagreb, Hrvatska

Povezanost rada

Kliničke medicinske znanosti

Poveznice
Indeksiranost