{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T10:44:26Z","timestamp":1790419466878,"version":"4.1.0"},"reference-count":38,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,9,14]],"date-time":"2017-09-14T00:00:00Z","timestamp":1505347200000},"content-version":"vor","delay-in-days":19584,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Australasian Annals of Medicine"],"published-print":{"date-parts":[[1964,2]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The clinical and h\u00e6matological features of three cases of paroxysmal nocturnal h\u00e6moglobinuria are reported. Each patient developed a second h\u00e6matological disorder requiring treatment in its own right. The first patient (Case I) developed severe bone\u2010marrow hypoplasia, the second (Case II) folic acid deficiency, and the third (Case III) iron deficiency. The severe bone\u2010marrow hypoplasia was accepted as a feature of paroxysmal nocturnal h\u00e6moglobinuria. Folic acid deficiency was attributed to increased demands associated with a chronic h\u00e6molytic process. Iron deficiency was attributed to inadequate replacement of h\u00e6moglobin and h\u00e6mosiderin iron lost in the urine.<\/jats:p><jats:p>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\u2010serum 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.<\/jats:p><jats:p>It is suggested that a diagnosis of paroxysmal nocturnal h\u00e6moglobinuria should be considered in every case of refractory an\u00e6mia. As shown in our three cases, the diagnosis of paroxysmal nocturnal h\u00e6moglobinuria cannot be excluded by the temporary absence of h\u00e6moglobinuria or h\u00e6mosiderinuria, or by negative results in the acid\u2010serum test even on several occasions. The circumstances in which these diagnostic criteria may be absent are discussed.<\/jats:p><jats:p>All three patients required blood transfusions to maintain their h\u00e6moglobin value at a level compatible with a relatively normal existence. One patient (Case II) developed h\u00e6molytic reactions to transfusions despite the use of carefully washed cells. The problem of these reactions was partly overcome by the adoption of a two\u2010stage transfusion r\u00e9gime. Additional measures were undertaken to treat complications, but these measures did not appear to influence the patients' transfusion requirements.<\/jats:p><jats:p>The name \u201cparoxysmal nocturnal h\u00e6moglobinuria\u201d is considered a little inappropriate, as it is descriptive of merely one prominent feature of the disease. Although paroxysmal nocturnal h\u00e6moglobinuria defies accurate definition at present, it is suggested that it should be regarded as an acquired dysh\u00e6matopoiesis of unknown \u00e6tiology, which is usually, but not invariably, accompanied by nocturnal h\u00e6moglobinuria.<\/jats:p>","DOI":"10.1111\/imj.1964.13.1.24","type":"journal-article","created":{"date-parts":[[2017,9,14]],"date-time":"2017-09-14T14:35:12Z","timestamp":1505399712000},"page":"24-31","source":"Crossref","is-referenced-by-count":5,"title":["PAROXYSMAL NOCTURNAL HIEMOGLOBINURIA : AN ACQUIRED DYSH\u00c6MOPOIESIS"],"prefix":"10.1111","volume":"13","author":[{"given":"J.","family":"HIRSH","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"UNGAR","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. S.","family":"ROBINSON","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,9,14]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1955.tb05511.x"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(59)90005-1"},{"key":"e_1_2_1_4_1","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1093\/clinchem\/5.4.275","article-title":"A Microbiologic Method for Detecting Folic Acid Deficiency in Man","volume":"5","author":"BAKER H.","year":"1959","journal-title":"Clin. Chem."},{"key":"e_1_2_1_5_1","first-page":"245","article-title":"Biochemical Studies on Leucocytes. II. Phosphatase Activity in Chronic Lymphatic Leucemia, Acute Leucemia, and Miscellaneous Hematologic Conditions","volume":"38","author":"BECK S. W.","year":"1951","journal-title":"J. Lab. clin. Med."},{"key":"e_1_2_1_6_1","unstructured":"BUENAVENTURA A. R.(1962) \u201cParoxysmal Nocturnal H\u00e6moglobinuria : A Report of Three Cases\u201d \u201cProceedings of the Second Congress of Asian and Pacific Society of Hematology\u201d Nagoya Japan."},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.2.5151.559"},{"key":"e_1_2_1_8_1","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1182\/blood.V5.9.843.843","article-title":"Paroxysmal Nocturnal Hemoglobinuria : A Specific Test for the Disease Based on the Ability of Thrombin to Activate the Hemolytic Factor","volume":"5","author":"CROSBY W. H.","year":"1950","journal-title":"Blood"},{"key":"e_1_2_1_9_1","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1182\/blood.V8.9.769.769","article-title":"Paroxysmal Nocturnal Hemoglobinuria : Relation of the Clinical Manifestations to Underlying Pathogenic Mechanisms","volume":"8","author":"CROSBY W. 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