Endocrine Abstracts

Introduction: Type 1a Diabetes (DM1) is a challenging disease with a rising incidence (up to 40 per 100 000 in Finland and Sardinia) being less common in the African population. It is believed to have an autoimmune basis and has been associated with other autoimmune diseases namely autoimmune thyroiditis and celiac disease. Acquired hemophilia is a rare clinical entity characterized by bleeding diathesis induced by circulating inhibitors to coagulation factors (VIII ou IX) that compromise hemostasis. The authors describe a case of a patient with DM1 with concomitant acquired hemophilia.

Case report: A 23 year old female patient with previous hospitalization for severe urinary tract infection and no regular medication, allergies or previous pregnancy or transfusions was admitted to the Hospital for Diabetic Ketoacidosis. Autoantibodies (Anti-insulin, islet cell and Glutamate decarboxylase) were positive and a favorable glycemic response was obtained with an intensive insulin regimen. On the 3^rd day of hospital admission the patient presented with severe hematuria. There was no significant hemoglobin fall but activated plasma thromboplastin time was prolonged (120 seconds). No changes in prothrombin time or platelets were found. Mixing clotting studies showed a failure to correct aPTT and Factor VIII activity was reduced (<0.5% – Normal Range 50–150%). Inhibitors to FVIII were detected (2.68 Bethesda Units). Full autoimmune panel and Rheumatology consult were inconclusive. Due to treatment failure of Cyclophosphamide and corticosteroids the patient was treated with Rituximab with improvement of both the hemophilia and DM1.

Conclusion: In this case no secondary cause of the hemophilia was found, being DM1 the only autoimmune comorbidity possibly contributing to the onset of hemorrhage. Only a few cases of acquired hemophilia have so far been described to affect DM1 patients. Interestingly Rituximab improved both conditions being the patient currently hemorrhage free despite still requiring insulin.