Diabetes in Patients with ß-thalassemia or other Hemoglobinopathies – Analysis from the DPV Database

Abstract

Diabetes mellitus is a common endocrinopathy in patients with thalassemia major, but the occurrence of hemoglobinopathies is rare in Germany and Western Europe. The longitudinal German-Austrian DPV (Diabetes Patienten Verlaufsdokumentation) registry allows a comprehensive characterization of this group of patients. Patients from the DPV-registry aged<30 years with thalassemia major or other hemoglobinopathies were compared to patients with type 1 diabetes (T1D) and type 2 diabetes (T2D) using the statistical software SAS 9.4. 94 patients (0.13% of patients) with hemoglobinopathies are registered in DPV. 82.4% of 17 patients with thalassemia major, 100% of 12 patients with sickle cell disease (SCD) and>90% of 65 patients with other hemoglobinopathies receive insulin treatment. In the majority of patients with thalassemia major, hemosiderosis is documented. Patients with thalassemia major developed diabetes at a median age of 14.6 [IQR 8.4-18.0] years (9.0 years [5.3-12.5] in T1D; 18.7 years [14.2-25.6] in TD2; both p<0.01). They show high HbA1c/fructosamine levels and frequent hypoglycemia, reflecting poor metabolic control. Diabetes in thalassemia major is probably caused by hemosiderosis due to polytransfusion, while patients with SCD/thalassemia minor are most likely affected by T1D. The high rate of hypoglycemia in patients with ß-thalassemia major may be caused by liver fibrosis and a lack of hepatic glycogen stores.

Ocular findings in patients with transfusion-dependent β-thalassemia in southern Iran

“…Additionally, the two detected patients with IOP > 22 mmHg were diabetic. Since DM is a common endocrinopathy in thalassemia patients [ 22 ] and due to the higher possibility of ocular involvement in diabetic patients, TDT patients with DM need more accurate ophthalmologic examinations and close monitoring. However, the frequency of fundus abnormalities in our study was similar in patients with and without DM.…”

Similar burden of type 2 diabetes among adult patients with sickle cell disease relative to African Americans in the U.S. population: a six‐year population‐based cohort analysis

“…Similarly, an epidemiologic study in Bahrain found relatively high prevalence of diabetes (8·3%), but it was lower than the general population after adjusting for age and sex (Mohamed et al , ). In another longitudinal registry study, only 10 patients with SCD and diabetes were identified from the German‐Austrian Diabetes Patienten Verlaufsdokumentation registry and all received insulin treatment, suggestive of type 1 diabetes mellitus (Warncke et al , ). These studies are limited due to relatively small sample sizes, lack of a nationally representative sample, non‐contemporary study periods and shorter follow‐up.…”

Endocrinopathies in Turkish Children with Thalassemia Major

“…This present study suggested that hypoglycemia is not only seen in thalassemic patients with diabetes, but also in normoglycemic thalassemic patients. Hemosiderosis impairing glucagon secretion, and low liver glycogen storage due to hepatic fibrosis are possible mechanisms (16). Insulin resistance and delayed insulin secretion due to hemosiderosis could be other possible mechanisms of hypoglycemia, as seen in patients with cystic fibrosis (17).…”