PAROXYSMAL NOCTURNAL HIEMOGLOBINURIA : AN ACQUIRED DYSHÆMOPOIESIS

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Abstract
Summary: The clinical and hæmatological features of three cases of paroxysmal nocturnal hæmoglobinuria are reported. Each patient developed a second hæmatological 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 hæmoglobinuria. Folic acid deficiency was attributed to increased demands associated with a chronic hæmolytic process. Iron deficiency was attributed to inadequate replacement of hæmoglobin and hæmosiderin iron lost in the urine.In each case a considerable time elapsed before the correct diagnosis was made. Delay in diagnosis occurred in one case because the result of the acid‐serum test was initially negative, and in the other two cases because the correct diagnosis was not considered until later in the course of the disease.It is suggested that a diagnosis of paroxysmal nocturnal hæmoglobinuria should be considered in every case of refractory anæmia. As shown in our three cases, the diagnosis of paroxysmal nocturnal hæmoglobinuria cannot be excluded by the temporary absence of hæmoglobinuria or hæmosiderinuria, or by negative results in the acid‐serum test even on several occasions. The circumstances in which these diagnostic criteria may be absent are discussed.All three patients required blood transfusions to maintain their hæmoglobin value at a level compatible with a relatively normal existence. One patient (Case II) developed hæmolytic reactions to transfusions despite the use of carefully washed cells. The problem of these reactions was partly overcome by the adoption of a two‐stage transfusion régime. Additional measures were undertaken to treat complications, but these measures did not appear to influence the patients' transfusion requirements.The name “paroxysmal nocturnal hæmoglobinuria” is considered a little inappropriate, as it is descriptive of merely one prominent feature of the disease. Although paroxysmal nocturnal hæmoglobinuria defies accurate definition at present, it is suggested that it should be regarded as an acquired dyshæmatopoiesis of unknown ætiology, which is usually, but not invariably, accompanied by nocturnal hæmoglobinuria.