Secondary hemochromatosis. I. Transfusion (exogenous) hemochromatosis.

(" bronszed diabetes ") is a condition of unknown etiology in which the body iron stores are vastly inscreased ansd in which there is portal cirrhosis of the liver. Diabetes, pigmenstation of the skins, and pan-creatic fibrosis are frequently, though not invariably, present. Numerous blood transsfusionss ins certain patienits have resulted ins the productions of a condition clinically somewhat similar to hemochromatosis. These two consditionss have beets separated by terminsg the former, idiopathic type " endogensous " (since the imscreased irons ins the body has beets absorbed through the gastrointestinal tract) while the latter, traissfusion type has beets called " exogenous. " The literature contains abount twensty cases' .9. 19-21 which have beets presented as examples of the exogetsous type. The purpose of this paper is to review these cases critically, point out serious deficienscies ins the presenst conscept of exogensous hemochromat.osis, cotstribute two more cases, ansd postulate a possible etiology. CASE REPORTS Case 1 E. P., a 58 year old white male, enstered the hospital ots July 25, 1949, because of weak-nsess and fatigue, fever, anti weight loss of two months duration. Prior to adnsission he had received four blood tramssfusionss and oral iron therapy. The patiemst hsad had imsguinal hernsiorrhaphies in 1930, pnseumonia in 1940, and chronic idiopathic pneumonsitis in 1942; on each of these occasioiss the hemoglobin was 14 Gm. per 100 nil. and the leukocyte counts and morphology were normal. Ins December 1944, prior to repeat herniormhsaphies, the hensoglobin was 12 Gm. per 100 ml. atsd the leukocyte count 3900 per cu. mm., with 44 per cemst neutrophils, 52 per censt lymphocytes, ansd 3 per censt monocytes. Ins August 1946, when admitted because of a fracture of the os calcis, the heukocyte picture was unchansged but the hemoglobin was 10.5 Gm. per 100 ml. On phsysical examinsations the patient appeared pale, the liver edge was felt 2 cm. below the right costal margin, and the spleen was barely palpable. The oral tensperature was 100.6 F., the pulse rate 100 per minute, atsd the h)lood pressure 100 mm. Hg systolic ansd 60 mm. Hg diastolic. Laboratory studies revealed the hemoglobin to h)e 6.6 Gns. per 100 nsl., the leukocyte count 2200 per cu. nim., amid the differenstial counst as follows: 52 per censt. neutrophils (30 per censt mature, 4 per cent metamyelocytes, 2 per cent myelocytes, 14 per cent pre-nsvelocvtes, and 2 per cenit blast forms), 32 …