Paroxysmal nocturnal hemoglobinuria in a girl with hemolysis and "hematuria".
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder of unknown frequency. In its classic form, PNH is characterized by hemolysis accompanied by nocturnal hemoglobinuria. The clinical course is unpredictable and may vary from severe hemolysis and recurrent venous thrombosis to latent periods with milder symptoms. We report a 15-year-old girl with hemolytic episodes, abdominal pain, and passage of dark urine. Hemoglobinuria was demonstrated by a "blood"-positive dipstick test in the absence of red blood cells in the urinary sediment. The diagnosis of PNH was confirmed by flow cytometry.
Dolezel
Zdenek
Z
Department of Pediatrics, University Hospital Brno, Cernopolni 9, 625 00 Brno, Czech Republic. zdolezel@med.muni.cz
Dostalkova
Dana
D
Blatny
Jan
J
Starha
Jiri
J
Gerykova
Hana
H
D000293:Adolescent; D003937:Diagnosis, Differential; D005260:Female; D006417:Hematuria / Q000175:diagnosis; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006461:Hemolysis; D006801:Humans