Naslov
Transitory aplastic anemia following acute autoimmune hepatitis in a young boy

Autori
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

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Archives of disease in childhood, 106 (2021), Suppl 2 / - , 2021, A98-A98

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

Mjesto i datum
Zagreb, Hrvatska, 07.10.2021. - 09.10.2021

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
autoimmune hepatitis ; aplastic anemia ; child

Sažetak
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.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA