Paroxysmal nocturnal hemoglobinuria: a single Spanish center's experience over the last 40 yr.
Bottom Line: The average clonal size upon diagnosis was 48%, presenting a variable evolution.Thrombotic episodes and cancer were five each, and the main causes of death among our patients were equal at 8.9%.Sixteen patients have been treated with eculizumab so far in our series, and being a safe drug, it provides improvement in the patients' quality of life, and the disappearance of clinical symptoms, and avoids the emergence of new thrombosis.
Affiliation: Hematology Service, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain.Show MeSH
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Mentions: Among the 56 patients, there was a loss of three follow-up patients and are not considered for survival assessments. Twelve patients have died, primarily due to cancer, and the remainder of deaths are directly attributable to causes of the underlying disease (thrombosis, mainly); see Table1. Only one of the deaths occurred of complications due to pneumonia. One patient died from cerebral hemorrhage during serious thrombocytopenia by severe aplasia. According to the Kaplan–Meier method, the survival curve is registered in Fig.2 with a median survival of 11 yr.
Affiliation: Hematology Service, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain.