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

Abstract

Background: 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/methods: 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.

Results: 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.

Conclusion: 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.

Zusammenfassung

Hintergrund: Diabetes mellitus ist eine häufige Endokrinopathie bei Patienten mit Thalassämia major. In Deutschland und im westlichen Europa ist die Prävalenz von Hämoglobinopathien allerdings niedrig. Das deutsch-österreichische DPV (Diabetes Patienten Verlaufsdokumentation) Register ermöglicht eine umfassende Charakterisierung dieser Patientengruppe.

Patienten/Methoden: Patienten aus dem DPV-Register im Alter von <30 Jahren mit Diabetes und Thalassämia major oder anderen Hämoglobinopathien wurden im Vergleich zu Patienten mit Typ 1 Diabetes (T1D) und Typ 2 Diabetes (T2D) unter Verwendung der Statistik-Software SAS 9,4 analysiert.

Ergebnisse: Im DPV Register sind 94 Patienten (0,13% aller Patienten) mit Hämoglobinopathien registriert. 82,4% von insgesamt 17 Patienten mit Thalassaemia major, 100% von 12 Patienten mit Sichelzellanämie (SCD) und >90% von 65 Patienten mit anderen Hämoglobinopathien werden mit Insulin behandelt. Beim Großteil der Patienten mit Thalassämia major ist eine Hämosiderose dokumentiert. Patienten mit Thalassämie major erkranken in einem medianen Alter von 14,6 [IQR 8,4–18,0] Jahren (9,0 Jahre [5,3–12,5] bei T1D; 18,7 Jahre [14,2–25,6] bei TD2, beide p<0,01) an Diabetes. Patienten mit Thalassämie weisen hohe HbA1c-/Fructosaminspiegel und häufige Hypoglykämien auf, vereinbar mit einer schlechten Stoffwechsellage.

Schlussfolgerung: Diabetes bei Thalassämia major ist mit größter Wahrscheinlichkeit durch eine durch häufige Transfusionen verursachte Hämosiderose bedingt, während bei Patienten mit Sichelzellerkrankung/Thalassämia minor am ehesten ein autoimmun vermittelter T1D besteht. Ursache der hohen Rate an Hypoglykämien bei Patienten mit ß-Thalassämia major sind vermutlich eine Leberfibrose und zu geringe Glykogenspeicher in der Leber.

Key words

ß-Thalassemia - sickle cell disease - hemoglobinopathy - diabetes mellitus - metabolic control

Schlüsselwörter

ß-Thalassämie - Sichelzellerkrankung - Hämoglobinopathie - Diabetes mellitus - metabolische Kontrolle

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