Siderosis following transfusions of blood

Abstract

D e p a r t m e n t of Pathology, Radcliffe In$rmary, Oxford * (PLATE XXXV) THE first example of the condition of transfusional siderosis recorded in the literature occurs in an account by Kark (1937) of two cases of aplastic anzmia.The first received over 290 transfusions in nine years and developed the classical picture of hzmochromatosis. The second had 61 transfusions in three years, but did not show pigmentation of the skin or glycosuria and died from cachexia secondary to an adenocarcinoma of the stomach. Unfortunately the exact number of transfusions given to the first of these patients is unknown and since post-mortem examination was refused no pathological details are available. However, it is recorded that eight years after the onset of the refractory anzmia, when he had received over 200 pints of blood, the skin showed a bluish pigmentation, and the lips and teeth also became pigmented. The liver became enlarged to the level of the umbilicus. The skin was ichthyotic and almost hairless, and although there was no glycosuria a fasting blood-sugar was found to be 150 mg./100 ml. He died suddenly nine years after the onset of symptoms.Since that time the condition though rare has been described with increasing frequency. Thus Schwartz and Blumenthal (1948) in describing 5 cases of their own could collect only 13 other cases from the literature. Many small series have been described since then, mainly in the American literature, the largest, being those of Muirhead et al. (1949-5 cases) and Wyatt et al. (1950-6 cases).A recent series has been that of Cappell et al. (1957-5 cases). The total number of cases reviewed in the literature is now about 47. Their salient features arc given in table I.It can be seen that the condition of transfusional siderosis has been described in association with a variety of anzmic states: 22 cases with refractory anzemia, 11 with aplastic anzmia, 5 with hzmolytic anzmia, 4 with leuco-erythroblastic anzmia, 1 with multiple myeloma, 1 with chronic lymphatic leukzemia, 1 with pyelonephritis and 2 where the cause of the anzemia was not recorded. The age in these cases ranged from 14 to 55, and their clinical and pathological manifestations from mild siderosis, confined to the liver and reticuloendothelial system, to the complete picture of hzmochromatosis with cirrhosis of the liver, skin pigmentation and glycosuria. These changes did not appear to be consistently related to either the duration of the anzmia or the amount of blood given by transfusion. At one extrpme there were cases such as those described by Wallerstein and Robbins (1953) andChesner (1946), where few or no transfusions were given and yet hzmoc~hromatosis developed, and at the other there were cases such as those of Wyatt and Goldenberg (1949-115 pints), Bothwell (1953-150 pints) and Cappell et al. (1957-610 and 527 pints), where large numbers of transfusions were given without the development of hepatic fibrosis or glycosuria.

Transfusional iron overload: The relationship between tissue iron concentration and hepatic fibrosis in thalassaemia

“…If continued iron absorption contributes significantly to iron-loading in heavily transfused patients the amount of iron found in the cadavers of such subjects should exceed the amount received as blood in life. However, review of published data reveals that with but few exceptions this applies only in cases that have received relatively few or no transfusions; in the majority of heavily transfused subjects (including thalassaemics) cadaver iron has actually fallen short of the amount transfused in life and, as pointed out by Oliver (1959), the observed discrepancies tend to increase with the number of transfusions ( fig. 9).…”

Thyroid hormone profile in beta-thalassemia major children

“…Hemosiderosis of various endocrine glands including the thyroid gland has been documented histologically in chronically transfused patients including thalassemics. Iron deposition in various endocrinal glands is responsible for the hormonal derangements [3][4][5][6] . Other factors like hypoxia due to persistent anemia and perfusion defect, also contribute to the derangement.…”

Iron Overload of the Liver by Trimethylhexanoylferrocene in Rats

“…in primary (heredi tary) haemochromatosis a hepatocellular iron storage is prominent [8]. whereas transfusional siderosis and dietary (Bantu) siderosis is characterized by a conspicuous iron storage in Kupffer cells and portal macrophages [8][9][10]. These characteristics in the storage pattern of iron are strongly marked in the early stages of the iron storage dis eases and are less evident in advanced ones.…”