RESUMO
This retrospective study has aimed at determining the prevalence, aetiology and clinical evolution of chronic liver disease (CLD) after allogeneic bone marrow transplantation (BMT). A total of 106 patients who had been transplanted in a single institution and who had survived for at least 2 years after BMT were studied. The prevalence of CLD was 57.5% (61/106). In 47.3% of cases more than one aetiopathogenic agent coexisted. The causes of CLD were iron overload (52.4%), chronic hepatitis C (47.5%), chronic graft-versus-host disease (C-GVHD) (37.7%), hepatitis B (6.5%), non-alcoholic steatohepatitis (NASH) (4.9%), autoimmune hepatitis (AIH) (4.9%) and unknown two (3.3%). Twenty-three patients with iron overload underwent venesections which were well tolerated. An improvement in liver function tests (LFTs) was observed in 21 (91%) patients. All six patients with siderosis as the only cause of CLD normalized LFT as well as three patients with HCV infection. Clinical evolution was satisfactory for patients with GVHD, AIH, NASH and hepatitis B. At the last visit 23 patients continued with abnormal LFTs, and 19 of them were infected by the HCV. A sustained biochemical and virologic response was achieved in only one case out of six patients with CHC who received interferon. We have found that CLD is a common complication in long-term BMT survivors. The aetiology is often multifactorial, iron overload, CHC and C-GVHD being the main causes. The CLD followed a rather 'benign' and slow course in our patients as none of them developed symptoms or signs of liver failure and we did not observe an increase in morbidity or mortality in these patients, but a longer follow-up is necessary in HCV infected patients based on the natural history of this infection in other populations.
Assuntos
Transplante de Medula Óssea , Hepatopatias/etiologia , Complicações Pós-Operatórias/etiologia , Adolescente , Adulto , Alanina Transaminase/sangue , Criança , Pré-Escolar , Doença Crônica , Feminino , Seguimentos , Doença Enxerto-Hospedeiro/etiologia , Hepatite B/etiologia , Hepatite C Crônica/etiologia , Hepatite Autoimune/etiologia , Humanos , Sobrecarga de Ferro/etiologia , Hepatopatias/enzimologia , Hepatopatias/epidemiologia , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Prevalência , Estudos Retrospectivos , Transplante HomólogoRESUMO
A patient with chronic myeloid leukemia (CML) treated with interferon alpha (IFN alpha) and who developed autoimmune hepatitis (AIH) is described. The patient was treated with IFN alpha 2a, a complete cytogenetic response was achieved 5 months later, and this response has lasted now more than 7 years. Autoimmune hypothyroidism appeared at 18 months of treatment, and 1 year later severe type I autoimmune hepatitis developed. To our knowledge this is the first report of such complication in an IFN alpha-treated CML patient.
Assuntos
Doenças Autoimunes/etiologia , Hepatite/etiologia , Hepatite/imunologia , Interferon-alfa/efeitos adversos , Interferon-alfa/uso terapêutico , Leucemia Mielogênica Crônica BCR-ABL Positiva/complicações , Leucemia Mielogênica Crônica BCR-ABL Positiva/terapia , Southern Blotting , Feminino , Humanos , Cariotipagem , Pessoa de Meia-Idade , Índice de Gravidade de DoençaRESUMO
Caroli's syndrome is included in the fibropolycystic abnormalities of the bile ducts. The disease is usually manifested by episodes of cholangitis. We report a case which presented as acute pancreatitis in the adulthood. The diagnosis was obtained by ultrasonography, CT scan, ERCP and liver biopsy. A choledochojejunostomy was performed with poor outcome and the patient is in the waiting list for liver transplantation.
