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Article
May 1960

Hemochromatosis and Hemosiderosis: Study of 211 Autopsied Cases

Author Affiliations
Boston
From the Mallory Institute of Pathology, Boston City Hospital, and the Departments of Pathology of Harvard Medical School and Boston University School of Medicine.
AMA Arch Intern Med. 1960;105(5):686-700. doi:10.1001/archinte.1960.00270170024004
Abstract

Both cirrhosis of the liver and hemochromatosis have been observed to be unusally frequent in this hospital1,2; an incidence of hemochromatosis of 1 in approximately 800 hospital deaths contrasts with a reported incidence of 1 in 7,000 hospital deaths.3 Because of this high incidence, and because of the frequent occurrence of hepatic carcinoma in patients with hemochromatosis, the present study was undertaken. Hemosiderosis as well as hemochromatosis was included in order to examine all diseases characterized morphologically by abnormal iron storage.

Review  Excellent reviews of the literature of hemochromatosis are available in the work of Sheldon,4 and of Finch and Finch,3 so that no attempt is made here to repeat their surveys. Sheldon reviewed 311 cases that he considered acceptable, up to 1935, and Finch and Finch found an additional 707 histologically proven cases between the years 1935 to 1954, to which they added 80 cases studied by them, so

References
1.
Mallory, F. B.:  Cirrhosis of Liver ,  New England J. Med. 206:1231-1239, 1932.Crossref
2.
MacDonald, R. A.:  Cirrhosis and Primary Carcinoma of the Liver: Changes in Their Occurrence at the Boston City Hospital, 1897-1954 ,  New England J. Med. 255:1179-1183, 1956.Crossref
3.
Finch, S. C., and Finch, C. A.:  Idiopathic Hemochromatosis: Iron Storage Disease ,  Medicine 34:381-430, 1955.Crossref
4.
Sheldon, J. H.:  Haemochromatosis , London, Oxford University Press, 1935.
5.
Demulder, R.:  Iron: Metabolism, Biochemistry, and Clinical Pathological Physiology; Review of Recent Literature ,  A.M.A. Arch. Int. Med. 102:254-301, 1958.Crossref
6.
Sheldon, J. H.:  The Iron Content of the Liver in Haemochromatosis, with Special Reference to the Brain ,  Quart. J. Med. 21:123-137, 1927.Crossref
7.
Dubin, I. N.:  Idiopathic Hemochromatosis and Transfusion Siderosis: A Review ,  Am. J. Clin. Path. 25:514-542, 1955.
8.
Dubach, R.; Callender, S. T. E., and Moore, C. V.:  Studies in Iron Transportation and Metabolism: VI. Absorption of Radioactive Iron in Patients with Fever and with Anemias of Varied Etiology ,  Blood 3:526-540, 1948.
9.
Brown, E. B.; Moore, C. V.; Reynafarje, C., and Smith, D. E.:  Intravenously Administered Saccharated Iron Oxide in the Treatment of Hypochromic Anemia: Therapeutic Results, Potential Dangers and Indications ,  J.A.M.A. 144: 1084-1089, 1950.Crossref
10.
Wallerstein, R. O., and Robbins, S. L.:  Hemochromatosis After Prolonged Oral Iron Therapy in a Patient with Chronic Hemolytic Anemia ,  Am. J. Med. 14:256-260, 1953.Crossref
11.
Koszewski, B. J.:  Occurrence of Megaloblastic Erythropoiesis in Patients with Hemochromatosis ,  Blood 7:1182-1195, 1952.
12.
Howell, J., and Wyatt, J. P.:  Development of Pigmentary Cirrhosis in Cooley's Anemia ,  A.M.A. Arch. Path. 55:423-431, 1953.
13.
Zeltmacher, K., and Bevans, M.:  Aplastic Anemia and Its Association with Hemochromatosis ,  Arch. Int. Med. 75:395-403, 1945.Crossref
14.
Cappell, D. F.; Hutchison, H. E., and Jowett, M.:  Transfusional Siderosis: The Effects of Excessive Iron Deposits on the Tissues ,  J. Path. & Bact. 74:245-264, 1957.
15.
MacDonald, R. A.:  Experimental Pigment Cirrhosis; Its Production in Rats by Feeding a Choline Deficient Diet with Added Iron ,  Am. J. Path. , to be published.
16.
Brown, E. B., Jr.; Dubach, R.; Smith, D. E.; Reynafarje, C., and Moore, C. V.:  Studies in Iron Transportation and Metabolism: X. Long-Term Iron Overload in Dogs ,  J. Lab. & Clin. Med. 50:862-893, 1957.
17.
Kinney, T. D.; Hegsted, D. M., and Finch, C. A.:  The Influence of Diet on Iron Absorption: I. The Pathology of Iron Excess ,  J. Exper. Med. 90:137-146, 1949.
18.
Gillman, T.; Hathorn, M., and Canham, P. A. S.:  Experimental Dietary Siderosis ,  Am. J. Path. 35:349-368, 1959.
19.
Mallory, F. B.; Parker, F., and Nye, R. N.:  Experimental Pigment Cirrhosis Due to Copper and Its Relation to Hemochromatosis ,  J.M. Res. 42:461-490, 1920.
20.
Cappell, D. F.:  Late Results of Intravenous Injection of Colloidal Iron ,  J. Path. & Bact. 33: 175-196, 1930.
21.
Kaufman, N.; Cartaya, J. A.; White, P. L.; Hegsted, D. M., and Kinney, T. D.:  An Attempt to Produce Hemochromatosis Experimentally and the Effect of High Levels of Copper and Choline in the Diet ,  J. Nutrition 46:433-444, 1952.
22.
Seki, M., and Chalmers, T. C.:  The Influence of Diet on the Distribution of Iron in the Rat Liver ,  Clin. Res. 6:188-189, 1958 (Abstract).
23.
Kleckner, M. S., Jr.; Baggenstoss, A. H., and Weir, J. F.:  Hemochromatosis and Transfusional Hemosiderosis: A Clinical and Pathologic Study ,  Am. J. Med. 16:382-394, 1954.Crossref
24.
Peterson, R. E.:  The Serum Iron in Acute Hepatitis ,  J. Lab. & Clin. Med. 39:225-236, 1952.
25.
Rather, L. J.:  Hemochromatosis and Hemosiderosis: Does Iron Overload Cause Diffuse Fibrosis of the Liver?  Am. J. Med. 21:857-866, 1956.
26.
Lillie, R. D.:  Histopathologic Technic and Practical Histochemistry , New York, Blakiston Company, Division of McGraw-Hill Book Company, Inc., 1954, p. 243.
27.
Mallory, F. B.:  Pathological Technique , Philadelphia, W. B. Saunders Company, 1938, p. 136.
28.
MacDonald, R. A., and Mallory, G. K.:  The Natural History of Postnecrotic Cirrhosis: A Study of 221 Autopsy Cases ,  Am. J. Med. 24: 334-357, 1958.
29.
Mallory, F. B.:  Cirrhosis of the Liver: Five Different Types of Lesions from Which It May Arise ,  Bull. Johns Hopkins Hosp. 22:1-17, 1911.
30.
Hartroft, W. S.; Lucas, C. C., and Best, C. H.:  Effects of Choline Deficiency in Man , in  The Vitamins: Chemistry, Physiology, Pathology , Vol. 2, edited by Sebrell, W. H., Jr., and Harris, R. S., New York, Academic Press, 1954, p. 104.
31.
Berk, J. E., and Lieber, M. M.:  Primary Carcinoma of the Liver in Hemochromatosis ,  Am. J.M. Sc. 202:708-714, 1941.
32.
Rath, C. E., and Finch, C. A.:  Sternal Marrow Hemosiderin ,  J. Lab. & Clin. Med. 33: 81-86, 1948.
33.
MacDonald, R. A.:  A Study of 356 Carcinoids of the Gastrointestinal Tract: Report of 4 New Cases of the Carcinoid Syndrome ,  Am. J. Med. 21:867-878, 1956.
34.
MacDonald, R. A.:  Primary Carcinoma of the Liver: A Clinicopathologic Study of 108 Cases ,  A.M.A. Arch. Int. Med. 99:266-279, 1957.
35.
Bell, E. T.:  The Relation of Portal Cirrhosis to Hemochromatosis and to Diabetes Mellitus ,  Diabetes 4:435-446, 1955.
36.
Pechet, G., and MacDonald, R. A.: Study of Hepatic Pigments with Special Reference to Hemofuscin and Hemosiderin, unpublished data.
37.
Moore, C. V.; Arrowsmith, W. R.; Welch, J., and Minnich, V.:  Studies in Iron Transportation and Metabolism: IV. Observations on the Absorption of Iron from the Gastrointestinal Tract ,  J. Clin. Invest. 18:553-580, 1939.
38.
Gubler, C. J.; Cartwright, G. E., and Wintrobe, M. M.:  The Effect of Pyridoxine Deficiency on the Absorption of Iron by the Rat ,  J. Biol. Chem. 178:989-996, 1949.
39.
Chodos, R. B.; Ross, J. F.; Apt, L.; Pollycove, M., and Halkett, J. A. E.:  The Absorption of Radioiron Labeled Foods and Iron Salts in Normal and Iron-Deficient Subjects and in Idiopathic Hemochromatosis ,  J. Clin. Invest. 36:314-326, 1957.
40.
Althausen, T. L.; Doig, R. K.; Weiden, S.; Motteram, R.; Turner, C. N., and Moore, A.:  Hemochromatosis: Investigation of 23 Cases, with Special References to Etiology, Nutrition, Iron Metabolism, and Studies of Hepatic and Pancreatic Function ,  A.M.A. Arch. Int. Med. 88:553-570, 1951.
41.
Gillman, J., and Gillman, T.:  Structure of the Liver in Pellagra ,  A.M.A. Arch. Path. 40: 239-263, 1945.
42.
Gillman, T.; Hathorn, M., and Lamont, N. McE.:  The Liver in Hemochromatosis ,  Lancet 2: 146-147, 1957.
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