PAROXYSMAL NOCTURNAL HEMOGLOBINURIA: REPORT OF A CASE COMPLICATED BY AN AREGENERATIVE (APLASTIC) CRISIS

Abstract

Besides hemolytic anemia with hemoglobinuria, the patient presented a characteristic leukopenia with susceptibility to infection and had repeated bouts of abdominal pain and headache possibly related to minor thrombotic accidents. He ultimately died of cerebral thrombosis. The tendency to thrombosis in paroxysmal nocturnal hemoglobinuria (PNH) may have been due to a defect of the platelets. During his chronic illness he developed an acute aregenerative reaction of the bone marrow (depressed formation of red cells,white cells and platelets). Multiple severe infections resulted apparently from agranulocytosis. In other forms of chronic hemolytic anemia with the aregenerative reaction, this generalized infection is not encountered. Susceptibility to infection of patients with PNH may be due to a defect of the leukocytes. The autopsy report showed that once thrombosis becomes irreversible it not only spreads in the organ of origin (in this case the brain), but also occurs in multiple organs throughout the body. Therapy of PNH, based on these findings, is discussed.