[An adolescent with paroxysmal nocturnal hemoglobinuria].
Prompted by the case history of a 17 year old girl with anaemia, mononucleosis infectiosa and abdominal pain, paroxysmal nocturnal haemoglobinuria (PNH) is described. After a mononucleosis infectiosa infection she developed many complications of which the most prominent were hemolysis and thrombosis. Severe abdominal pain and episodic bowel obstruction occurred as a result of micro-infarction of the mesentery; bone marrow aplasia and lysis of platelets resulted in progressive thrombopenia. Pathogenesis and therapeutical possibilities are discussed. Coexistence of a necrotising enterocolitis with rectovaginal fistula, a heart infarction and the striking weight loss and hyponatremia during exacerbations, as seen in our patient, have not previously been described in PNH.
Rodrigues Pereira
R
R
St. Clara Ziekenhuis, Rotterdam.
Swank
D J
DJ
Sizoo
W
W
D000293:Adolescent; D004761:Enterocolitis, Pseudomembranous / Q000209:etiology; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006801:Humans; D007010:Hyponatremia / Q000209:etiology; D007244:Infectious Mononucleosis / Q000150:complications*; D009203:Myocardial Infarction / Q000209:etiology; D013921:Thrombocytopenia / Q000209:etiology; D013927:Thrombosis / Q000209:etiology
Een adolescent met paroxismale nachtelijke hemoglobinurie.