Adrenal crisis in a 14-year-old boy 12 years after hematopoietic stem cell transplantation

Penger T, Albrecht A, Marx M, Stachel D, Metzler M, Dörr HG (2018)

Publication Type: Journal article

Publication year: 2018

Journal

Endocrinology, Diabetes and Metabolism Case Reports BioScientifica

Book Volume: 2018

Abstract

We report on a boy of Albanian descent with the history of juvenile myelomonocytic leukemia (JMML). JMML was diagnosed at the age of 17 months and treated by hematopoietic stem cell transplantation (HSCT). At the age of 14.3 years, about 12 years after HSCT, he was hospitalized with an adrenal crisis. Hormone findings were consistent with primary adrenal insufficiency. Autoimmune adrenalitis was confirmed by positive autoantibodies against 21-hydroxylase and adrenal tissue. Since autoimmune Hashimoto thyroiditis was already known from the age of 9 years, we assume that both diseases are part of the spectrum of autoimmune polyglandular syndrome (APS) type 2. APS type 2 is a rare endocrine disease characterized by Addison's disease along with autoimmune thyroid disease and/or type 1 diabetes. Learning points: Endocrine sequelae after hematopoietic stem cell transplantation (HSCT) are common and can develop over a long period.Primary adrenal insufficiency after HSCT is absolutely rare.The combination of adrenal autoimmune disease and Hashimoto thyroiditis is consistent with autoimmune polyglandular syndrome type 2.

Authors with CRIS profile

Theresa Penger Professur für Kinderheilkunde mit dem Schwerpunkt Kinder-Endokrinologie und Diabetologie (Stiftungsprofessur) Andrea Albrecht Lehrstuhl für Kinder- und Jugendmedizin Markus Metzler Professur für Kinder- und Jugendmedizin mit dem Schwerpunkt Pädiatrische Onkologie und Hämatologie Helmuth-Günther Dörr Professur für Kinderheilkunde mit dem Schwerpunkt Kinder-Endokrinologie und Diabetologie (Stiftungsprofessur)

How to cite

APA:

Penger, T., Albrecht, A., Marx, M., Stachel, D., Metzler, M., & Dörr, H.-G. (2018). Adrenal crisis in a 14-year-old boy 12 years after hematopoietic stem cell transplantation. Endocrinology, Diabetes and Metabolism Case Reports, 2018. https://dx.doi.org/10.1530/EDM-18-0034

MLA:

Penger, Theresa, et al. "Adrenal crisis in a 14-year-old boy 12 years after hematopoietic stem cell transplantation." Endocrinology, Diabetes and Metabolism Case Reports 2018 (2018).

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