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[An adolescent with paroxysmal nocturnal hemoglobinuria]
R Rodrigues Pereira1, D J Swank, W Sizoo
1St. Clara Ziekenhuis, Rotterdam.
Tijdschrift Voor Kindergeneeskunde
|August 1, 1988
Summary
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare blood disorder. This case highlights PNH complications like hemolysis, thrombosis, and gastrointestinal issues following mononucleosis.
Area of Science:
- Hematology
- Internal Medicine
- Pediatrics
Background:
- Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired clonal hematopoietic stem cell disorder.
- It is characterized by complement-mediated intravascular hemolysis, thrombosis, and bone marrow failure.
Observation:
- A 17-year-old female presented with anemia, infectious mononucleosis, and abdominal pain.
- Following infectious mononucleosis, she developed significant complications including hemolysis and thrombosis.
- She experienced severe abdominal pain and bowel obstruction due to mesenteric micro-infarction and progressive thrombocytopenia from bone marrow aplasia.
Findings:
- The patient exhibited previously undescribed PNH manifestations: necrotizing enterocolitis with rectovaginal fistula, myocardial infarction, significant weight loss, and hyponatremia during exacerbations.
- These findings expand the known clinical spectrum of PNH, particularly in association with viral infections.
Implications:
- This case underscores the importance of considering PNH in young patients presenting with unexplained hemolysis, thrombosis, and cytopenias, especially after viral infections.
- The unusual combination of complications suggests a complex interplay between infection, PNH pathogenesis, and potential novel therapeutic targets.