Hemochromatosis medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]; Associate Editor(s)-in-Chief: Sunny Kumar MD [3]
Overview
The treatment of hemochromatosis depends on levels of iron deposition in body tissues, symptoms and complications due to damaged organs secondary to inflammatory response towards deposition.
Medical Therapy
- Treatment is initiated when ferritin levels reach 300 micrograms per litre (or 200 in nonpregnant premenopausal women).
- Treatment of organ damage (heart failure with diuretics and ACE inhibitor therapy).
- Limiting intake of alcoholic beverages, vitamin C (increases iron absorption in the gut), red meat (high in iron) and potential causes of food poisoning (shellfish, seafood).
- Increasing intake of substances that inhibit iron absorption, such as high-tannin tea, calcium, and foods containing oxalic and phytic acids (these must be consumed at the same time as the iron-containing foods in order to be effective.
Following are the recommendations for treating hemochromatosis according to American Association for the Study of Liver Diseases:
Phlebotomy is recommended option for the patients with iron over load weather they are symptomatic or not.[1][2][3][4][5][6][7][8][9][10][11][12]
1 Starting therapeutic (induction) phlebotomy: Staring blood removal till iron level gets normal.
- 1.1 Asymptomatic hemochromatosis:
- Preferred regime: 1 unit of blood (about 473 mL or 1 pint) is removed per week
- Alternative regime: 0.5 to 2 units of blood can often be removed from men, whereas it may be possible to remove only 0.5 units of blood from women or frail or elderly patients with other medical problems
- 1.2 Symptomatic patients with end-organ damage hemochromatosis:
- 1.3 Preferred regime: 1 unit of blood (about 473 mL or 1 pint) is removed per week
- 1.4 Stop frequent phlebotomy when serum ferritin reaches 50-100 μg/L
2 Maintenance phlebotomy: When serum ferritin levels in the range of 50 to 100 ng/mL
- 2.1 Preferred regime: Removing 1 unit of blood (about 473 mL or 1 pint) is removed per 2 months
- 2.2 Alternative regime: Removing 1 unit of blood (about 473 mL or 1 pint) is removed per 4 months
- 2.3 Check serum ferritin level every 10-12 phlebotomies
3 Dietary restrictions:
- 3.1 Preferred regime: No restrictions.
- 3.2 Alternative regime: avoid iron supplements, avoid vitamin C supplements, avoid red meat, avoid alcohol completely
4 Non-HEF hemochromaosis:
- 4.1 Preferred regime: 1 unit of blood (about 473 mL or 1 pint) is removed per week
- 4.2 Alternative regime: In HIC is normal then follow dietary restrictions.
5 Secondary Iron Overload:
- 5.1 Dyserythropoietic syndromes:
- 5.1.1 Preferred regimen: Deferoxamine (Desferal) at a dose of 20-40 mg/kg body weight per day
- 5.1.2 Alternative regime: Deferasirox (Exjade) given orally
- 5.2 chronic hemolytic anemia:
- 5.2.1 Preferred regimen: Deferoxamine at a dose of 20-40 mg/kg body weight per day
- 5.2.2 Alternative regime: Deferasirox given orally
- 5.3 Liver Biopsy
- 5.3.1: Liver biopsy for evaluation not required.
- 5.3.2: Consider follow-up liver biopsy to ascertain adequacy of iron removal.
References
- ↑ Adams PC, Speechley M, Kertesz AE (1991). "Long-term survival analysis in hereditary hemochromatosis". Gastroenterology. 101 (2): 368–72. PMID 2065912.
- ↑ Niederau C, Fischer R, Pürschel A, Stremmel W, Häussinger D, Strohmeyer G (1996). "Long-term survival in patients with hereditary hemochromatosis". Gastroenterology. 110 (4): 1107–19. PMID 8613000.
- ↑ Falize L, Guillygomarc'h A, Perrin M, Lainé F, Guyader D, Brissot P; et al. (2006). "Reversibility of hepatic fibrosis in treated genetic hemochromatosis: a study of 36 cases". Hepatology. 44 (2): 472–7. doi:10.1002/hep.21260. PMID 16871557.
- ↑ Adams PC, Deugnier Y, Moirand R, Brissot P (1997). "The relationship between iron overload, clinical symptoms, and age in 410 patients with genetic hemochromatosis". Hepatology. 25 (1): 162–6. doi:10.1002/hep.510250130. PMID 8985284.
- ↑ Kowdley KV (2004). "Iron, hemochromatosis, and hepatocellular carcinoma". Gastroenterology. 127 (5 Suppl 1): S79–86. PMID 15508107.
- ↑ Brittenham GM, Klein HG, Kushner JP, Ajioka RS (2001). "Preserving the national blood supply". Hematology Am Soc Hematol Educ Program: 422–32. PMID 11722996.
- ↑ Lynch SR, Cook JD (1980). "Interaction of vitamin C and iron". Ann N Y Acad Sci. 355: 32–44. PMID 6940487.
- ↑ Bonkovsky HL, Lambrecht RW, Shan Y (2003). "Iron as a co-morbid factor in nonhemochromatotic liver disease". Alcohol. 30 (2): 137–44. PMID 12957298.
- ↑ Facchini FS, Hua NW, Stoohs RA (2002). "Effect of iron depletion in carbohydrate-intolerant patients with clinical evidence of nonalcoholic fatty liver disease". Gastroenterology. 122 (4): 931–9. PMID 11910345.
- ↑ Brittenham GM, Griffith PM, Nienhuis AW, McLaren CE, Young NS, Tucker EE; et al. (1994). "Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major". N Engl J Med. 331 (9): 567–73. doi:10.1056/NEJM199409013310902. PMID 8047080.
- ↑ Brittenham GM, Badman DG, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)Workshop (2003). "Noninvasive measurement of iron: report of an NIDDK workshop". Blood. 101 (1): 15–9. doi:10.1182/blood-2002-06-1723. PMID 12393526.
- ↑ Hankins JS, McCarville MB, Loeffler RB, Smeltzer MP, Onciu M, Hoffer FA; et al. (2009). "R2* magnetic resonance imaging of the liver in patients with iron overload". Blood. 113 (20): 4853–5. doi:10.1182/blood-2008-12-191643. PMC 2686136. PMID 19264677.