engleski

A PALE GIRL WITH DARK URINE: AN UNCOMMON CASE OF MACROHEMATURIA WITH A FAVOURABLE OUTCOME

Introduction: Autoimmune hemolytic anemia is a rare cause of hemolytic anemia in children, most commonly mediated by „warm“ IgG antibodies. As opposite, „cold“ IgG antibody mediated anemia occurs in only 1% of autoimmune hemolytic anemias in children. Material and methods: We present a 2.5-year-old, otherwise healthy girl who initially came to our attention because of pallor and dark urine. Two weeks earlier she had symptoms suggestive of both upper respiratory and urinary tract infection which was treated with wide- spectrum antibiotics and led to the complete resolution of symptoms. Two weeks later she developed fever with joint pain, headache and macrohaematuria. A wide differential diagnosis was considered, including hemolytic-uremic syndrome. Results: Her laboratory findings showed hemolytic anemia (Hgb 92), mild thrombocytopenia (Trc 145), normal renal function and mild hyperlipidemia with only a few erythrocytes in the urine sediment. As a part of workup, the screening for antibodies against red blood cells was performed, revealing positive direct (DAT) and negative indirect (IAT) antiglobulin test. Finally, more specific test revealed positive „cold“ biphasic IgG anti P1 antibodies which are patognomonic for paroxysmal cold hemoglobinuria. Conclusions: Paroxysmal cold hemoglobinuria (Donath-Landsteiner anemia) is a form of autoimmune hemolytic anemia characterised with abrupt onset of hemolytic anemia and hemoglobinuria after exposure to cold weather. Although rare in children, it should not be disregarded in the differential diagnosis of macrohematuria. Treatment is symptomatic with warm electrolyte infusions and erythrocte transfusions. The outcome is favourable with self-limiting course and has no repercussions on patients future life, although recurrent episodes of hemoglobinuria have been described

autoimmune hemolytic anemia ; Donath-Landsteiner anemia ; children

nije evidentirano