Hereditary Hemochromatosis and Alpha-Thalassemia Presenting with Diabetes Mellitus: A Rare Case Report

  • Pourya Farhangi Medical School, Tehran University of Medical Sciences, Tehran, Iran.
  • Minoo Sadat Hajmiri Department of Endoscrinology, Vali-Asr Hospital, Endocrinology and Metabolism Research Center (EMRC), Imam Khomeini Complex Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Nooshin Shirzad Department of Endoscrinology, Vali-Asr Hospital, Endocrinology and Metabolism Research Center (EMRC), Imam Khomeini Complex Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Mahboobeh Hemmatabadi Department of Endoscrinology, Vali-Asr Hospital, Endocrinology and Metabolism Research Center (EMRC), Imam Khomeini Complex Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Keywords: Hemochromatosis; Alpha-thalassemia; Diabetes mellitus; Hypogonadism

Abstract

Hereditary hemochromatosis (HH) is a rare genetic disorder, causing systemic iron overload. High amounts of iron in the bloodstream gradually oversaturate the trans- ferrin which can cause sedimentation of iron in the pancreas, liver, heart, pituitaryand joints, though it can establish multiorgan involvements.

We present a case of TFR2 (type 3) HH who had minor α-thalassemia and uncontrolled diabetes mellitus, and discuss the clinical presentation and patient management.

A 33-year-old man with type 3 HH and alpha-thalassemia trait, presented with uncon- trolled diabetes mellitus, skin hyperpigmentation and hypogonadism. The patient had high blood glucose ,despite the administration of 80 units of Glargineand 80 units of Aspart insulins per day, but after changing them into human insulins, his diabetes mellitus was surprisingly controlled with only 32 units of NPH and 18 units of Regular insulins. Furthermore, he was treated with testosterone (due to hypogonadism) and Deferasirox (due to iron overload).

Published
2022-11-13
Section
Articles