Paroxysmal Nocturnal Hiemoglobinuria : An Acquired Dyshæmopoiesis

Abstract

The clinical and h<ematological features of three cases of paroxysmal nocturnal h<emoglobinuria are reported. Each patient developed a second h<ematological disorder requiring treatment in its own right. The first patient (Case I} developed severe bone-marrow hypoplasia, the second (Case II} folic acid deficiency, and the third (Case III) iron deficiency. The severe bone-marrow hypoplasia was accepted as a feature of paroxysmal nocturnal h<emoglobinuria. Folic acid deficiency was attributed to increased demands associated with a chronic h<emolytic process. Iron deficiency was attributed to inadequate replacement of h<emoglobin and h<emosiderin iron lost in the urine.

Washed and Volume-Reduced Blood Components

“…47 As a consequence, some hematologists request washed RBC components for routine transfusions in patients with PNH. 48,49 However, it should be noted that most PNH patients do not experience hemolysis after RBC transfusions, even when whole blood is transfused. 48,49 However, it should be noted that most PNH patients do not experience hemolysis after RBC transfusions, even when whole blood is transfused.…”