Paroxysmal nocturnal hemoglobinuria: rare cause of acute renal failure
Autopsy And Case Reports
Paroxysmal nocturnal hemoglobinuria: rare cause of acute renal failure
Autor Correspondente: V. Takayasu | [email protected]
Palavras-chave: hemoglobinuria Paroxysmal, anemia hemolytic, acute kidney injury, flow cytometry.
Resumos Cadastrados
Resumo Inglês:
Paroxysmal nocturnal hemoglobinuria is a rare acquired disease,
characterized by hemolytic anemia, recurrent infections, cytopenias, and
vascular thrombosis. It occurs by non-malignant clonal expansion of one or
more hematopoietic stem cells that acquired somatic mutations in PIG-A gene
linked to chromosome X. This mutation results in lower erythrocyte expression
of CD55 and CD59 surface proteins and consequently increased susceptibility
to the complement system. The renal involvement is generally benign, resulting
in mild impairment in urinary concentration. Acute renal failure requiring
hemodialytic support accompanying PNH is rarely observed. The authors
report a case of a 37-year-old male who presented with bicytopenia (hemolytic
anemia and thrombocytopenia) associated with acute renal failure requiring
dialysis. Diagnosis was challenging because of the rarity and unfamiliarity with
this entity, but was confirmed by flow cytometry. In the course of the disease,
acute pyelonephritis with multiple renal abscesses was diagnosed requiring
prolonged antibiotic therapy. Patient outcome was favorable after the control
of hemolysis and the infection treatment.
