Paroksizmalna nočna hemoglobinurija po aplastični anemiji - prikaz primera = Paroxysmal noctural haemoglobinuria emerging after aplastic anaemia - case report
Mlakar, Uroš, 1948-
Background. Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal haematopoietic stem cell disorder characterised by intravascular haemolysis, bone marrow failu- re and increased tendency ...to thrombosis. Due to mutation ofthe PIG-A gene, abnormal blood cells lack a series of glycosylphosphatidyl inositol-anchored proteins, which results in complement mediated haemolysis. The coexistence of bone marrow failure that paradoxically produces growth advantage for the PNH clone explains the close association between PNH and aplastic anaemia. Methods, results. A 24-year old male patient was found to have severe aplastic anaemia. He was treated with antithymocyte globulin, cyclosporine A and glucocorticoids. In parallel with a clinical and haematological improvement of aplastic anaemia, attacks of abdominal pain occurred. In one of such episodes when abdominal pain was accompanied by massive intravascular haemolysis, the diagnosis of PNH was established by the positive Ham test. The treatment was symptomatic. Because of the risk of thrombotic complication the patient is on antithrombotic prophylaxis with low-molecular weight heparin. Conclusions. Due to the variability of the symptoms at presentation and its rarity, PNH is frequently diagnosed after considerable delay. The diagnosis of PNH is made using the Ham test or flow cytometry. The diagnostic test should be obtained in patients with unexplainedhaemolytic anaemia, cytopenias and aplastic anaemia, venous thromboses and recurrent bouts of abdominal pain.
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