PMON37 Diabetes and Hypopituitarism Secondary to Iron Overload in a Patient with Pure Red Cell Aplasia

Abstract

Abstract Background Pure red cell aplasia is a rare bone marrow disorder that results in decline in red blood cell production by the bone marrow. Treatment involves multiple blood transfusions to keep up the red blood cell count, which can lead to iron overload. Hypopituitarism is a condition, where two or more pituitary hormones are deficient. Here we present a case of diabetes and hypopituitarism secondary to iron overload from blood transfusions. Clinical Case A 19 year male with pure red cell aplasia was admitted for anemia and was noted to have short stature with growth percentile at 0.36% with predicted MPH estimated to be 180.5 cm. He was Tanner stage 2 on exam and work-up showed IGF1 128 (n 281-510 ng/mL), Testosterone of <1 (n 250-1100 ng/dL), LH 1.1 (n 1-10 mIU/mL), FSH 1.5 (n 1-10 mIU/mL), delayed bone age of 15 years consistent with delayed puberty and hypogonadotropic hypogonadism so he was initiated on 50 mg IM Testosterone monthly. After 1 month, he presented to the hospital again for mild DKA secondary to high dose steroids as part of treatment for his underlying medical condition and also pancreatic dysfunction given history of regular transfusions and iron overload. Initial Fructosamine level was 438 equivalent to HgbA1c of ∼10-11%. He was started on SQ insulin regimen with both long- and short-acting insulin and 50 mcg Levothyroxine as thyroid levels checked during admission showed hypothyroid state, thus, meeting diagnostic criteria for hypopituitarism. Brain MRI was obtained which was negative for mass or lesions, however abdominal MRI showed very high level iron overload on liver, spleen and pancreas. Steroids were weaned and insulin regimen was adjusted on a regular interval according to his blood glucose levels, however, he was not able to wean off insulin even after steroids were discontinued and was transitioned to 70/30 regimen for ease of management with normalization of Fructosamine levels. His testosterone was eventually increased up to 200 mg and was transitioned to daily topical Androgel and currently increasing dose gradually. His most recent testosterone level was 38 ng/dL with goal range of 400-600 ng/dL. His thyroid levels continued to be stable on current dose. He had passed the ACTH stimulation test ∼4-6 weeks after discontinuation of steroids and decided not to pursue growth hormone therapy. Conclusion Patients who receive regular blood transfusions are at risk of iron overload, which can affect multiple organs including the pancreas and pituitary gland. This can subsequently lead to insulin-dependent diabetes and hypopituitarism, which should bring awareness of regular assessment of diabetes and pituitary function starting early in life. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.