RESUMO
Amyloidosis is the extracellular deposition of unique protein fibrils in different tissue organs. It is most commonly associated with B-cell malignancy such as multiple myeloma or Waldenstrom macroglobulinaemia. It involves the liver, heart, kidney, peripheral nerves and soft tissues. Liver however is affected, but clinically apparent disease is very rare. Hepatomegaly and mild elevation of alkaline phosphatase is the most common presentation in patients with liver involvement. Acute hepatic failure is a rare presentation with myeloma-induced amyloidosis. The diagnosis can be difficult requiring biopsy or sometimes special staining of the tissue. Management is still very challenging.
Assuntos
Insuficiência Hepática Crônica Agudizada/etiologia , Amiloidose/complicações , Mieloma Múltiplo/complicações , Adulto , Biópsia , Diagnóstico Diferencial , Humanos , Masculino , RadiografiaRESUMO
Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital vascular anomaly syndrome characterised by multifocal venous vascular malformations. It involves skin, central nervous systems, liver, muscles and gastrointestinal (GI) tract resulting in intestinal haemorrhage and anaemia. Patients with BRBNS experience severe chronic anaemia without any diagnosis requiring multiple transfusions and hospitalisations. BRBNS has a propensity for severe life-threatening bleeding. Skin and GI tract are the most commonly affected organs.