ANEMIAS, IRON LOADING ANEMIAS, THALASSEMIA

Learning more about the various types of anemia, the possible causes, 
as well as the process of iron absorption, can help us to better understand iron overload!

• Cardiac dysfunction because of secondary hemochromatosis caused by congenital non-spherocytic hemolytic anemia. Jpn Circ J. 2001 Feb Cardiac failure accounts for approximately one-third of the deaths associated with hemochromatosis. Liver dysfunction or hormonal disorders such as diabetes generally precede cardiac failure

• Discriminating Between Iron Deficiency Anemia and Anemia of Chronic Disease Using Traditional Indices of Iron Status vs Transferrin Receptor Concentration  Frank H. Wians, Jr, PhD, Jill E. Urban, MD, Joseph H. Keffer, MD, and Steven H. Kroft, MD Am J of Clin Pathology 2001 

• Hemochromatosis: A Common, Rarely Diagnosed Disease 
  By Vincent J. Felitti, MD, FACP  Commentary by David Baer, MD, FACP.  Hemochromatosis is the most common, life-threatening genetic disorder in North America, yet most physicians have never personally diagnosed a case: all see an unrecognized case in their offices every two weeks.

• Hemochromatosis and Iron therapy of Restless Legs Syndrome  [Abstract]  
  Click here for full text pdf file.   Sleep Med.. 2001 May Barton JC, Wooten VD, Acton RT.

• Hemochromatosis: genetics helps to define a multifactorial disease  Clin Genet, 54(1):1-9 1998 Jul University of Washington, Seattle 98105 wburke@u.washington.edu  Early detection is desirable, because periodic phlebotomy provides effective treatment for iron overload and may prevent complications of the disorder.

• Hepcidin: A putative iron-regulatory hormone relevant to hereditary hemochromatosis and the anemia of chronic disease.  Proc. Natl. Acad. Sci. USA, Vol. 98, Issue 15, 8160-8162, July 17, 2001  The paper by Nicolas et al. in this issue of PNAS (3) presents the exciting possibility that a central player in the communication of body iron stores to the intestinal absorptive cells may have been identified.

• Hereditary hemochromatosis in a patient with congenital dyserythropoietic anemia  Blood 2000 Nov 15 This report points out the need to consider congenital dyserythropoietic anemia in patients with hemochromatosis and unexplained macrocytosis and, conversely, to check for the presence of hereditary hemochromatosis in patients with congenital dyserythropoietic anemia and severe iron overload.

• Investigating Anaemia and Iron Storage Disease
  Discussion of the different types of anemia, as well as the effects of iron overload.  

• Iron deficiency and overload in relation to nutrition 
  Nutritional iron intake in the Netherlands. IJzertekort en ijzerstapeling met betrekking tot voeding Spanjersberg MQI ; Jansen EHJM 34 p in English, 2000 Iron supplementation or fortification in functional foods should be avoided and discouraged until the risks of iron overload have been more clearly determined, since in the general population iron overload is associated with increased risk of several chronic diseases as well.

• Iron deficiency due to excessive therapeutic phlebotomy in hemochromatosis 
  Am J Hematol 2000 Nov

• Iron Metabolism and Deficiency 
  Bruce M. Small, M.D. State University of New York at Buffalo June 9, 1998

• Iron Status Assessment, stages of depletion, deficiency, anemia Spring 2001 [a pdf file]  This is excellent for its description of the 3 stages of decreasing iron levels.  Even when describing IDA, it can be used by the IO patient in learning more about assessing iron status. 

• PULLING IRON OUT OF THE FIRE by Shelly Morrow.  This article appeared in the Sept/Oct 2000 issue of Arthritis Today, published by the Arthritis Foundation, Inc.
  

THALASSAEMIA

• Click here for a PubMed search on Thalassemia & Chelation therapy 174 results

• Classification and diagnosis of iron overload   Haematologica 1998 May The recent description of new conditions associated with iron overload and the identification of the genetic defect of hereditary hemochromatosis prompted us to review this subject and to redefine the diagnostic criteria of iron overload disorders.

• Diabetic ketoacidosis and hypogonadotropic hypogonadism in association with transfusional hemochromatosis in a man with beta-thalassemia major.   J Formos Med Assoc 2001 Jul;100(7):492-6

• Drug Treatment for Iron Overload
  Deferiprone should be used in patients with thalassaemia and other patients with iron overload, who cannot receive effective or sufficient deferoxamine therapy. Optimal Wellness Health News Issue 166 August 13, 2000

• Effects of iron overload on the immune system.   Ann Clin Lab Sci 2000 Oct Department of Pathology, Marshall University and Huntington DVA Medical Center, West Virginia Increased body stores of iron in various clinical situations may tip the immunoregulatory balance unfavorably to allow increased growth rates of cancer cells and infectious organisms, and complicate the clinical management of preexisting acute and chronic diseases.

• Heterozygosity for the H63D mutation in the hereditary hemochromatosis (HFE) gene may lead into severe iron overload in beta-thalassemia minor: observations in a thalassemic kindred.  
  Rev Invest Clin 2001 Mar-Apr  Heterozygosity for beta-thalassemia (minor) by itself does not lead into iron overload; however, when it is inherited together with a homozygous state for either the H63D or the C282Y mutations of the hereditary hemochromatosis gene (HFE gene), iron overload may ensue.

• Iron absorption in the thalassemia syndromes and its inhibition by tea. 
  N Engl J Med. 1979 Jan 4 Tea produced a 41 to 95 per cent inhibition of iron absorption. Since patients with thalassemia intermedia may absorb a large percentage of dietary iron, inhibitors of iron absorption, such as tea, may be useful in their management.

• Iron overload problems in THALASSAEMIA

• Quantitative Texture Analysis in Two-Dimensional Echocardiography: Application to the Diagnosis of Myocardial Hemochromatosis.Echocardiography. 1996 Jan Myocardial walls affected by hemochromatosis show ultrasound image texture alterations that may be quantified with digital image analysis techniques and appear mostly unrelated to hematologic and conventional, as well as radiofrequency-based, echocardiographic parameters. These changes in quantitatively evaluated echo reflectivity are present even before the development of clinical and echocardiographic signs of cardiac dysfunction.

• Reduced Left Ventricular Contractile Reserve Identified by Low Dose Dobutamine Echocardiography as an Early Marker of Cardiac Involvement in Asymptomatic Patients with Thalassemia Major. Echocardiography 1996 Sep;13

• Right ventricular diastolic function in beta-thalassemia major: echocardiographic and clinical correlates. Am Heart J. 2001 Mar Cardiac hemochromatosis remains the most frequent cause of death in beta-thalassemia major. Previous studies suggest an important right ventricular (RV) contribution to cardiac morbidity and mortality.

• THALASSEMIA basics

Back to the "Munnsters" main Hemochromatosis page