Diabetes Mellitus, Extreme Insulin Resistance, and Hypothalamic-Pituitary Langerhans Cells Histiocytosis

Author:

Sollier Mathilde1,Halbron Marine1,Donadieu Jean2,Idbaih Ahmed3ORCID,Cohen Aubart Fleur4,Vigouroux Corinne5,Auclair Martine5,Bourron Olivier1,Bastin Marie1,Béra Géraldine6,Touraine Philippe78,Young Jacques9,Mosbah Héléna1,Hartemann Agnès1,Andreelli Fabrizio1ORCID,Amouyal Chloé1

Affiliation:

1. Diabetology-Metabolism Dpt, Sorbonne Université, APHP, Institut Hospitalo–Universitaire de Cardiometabolisme et Nutrition (ICAN), Pitié-Salpêtrière-Charles Foix Hospital, 75013 Paris, France

2. Hemato-Oncology Dpt, Trousseau Hospital, APHP, 75012 Paris, France

3. Sorbonne Université, Inserm, CNRS, UMRS 1127, Institut du Cerveau et de la Moelle épinière, ICM, AP-HP, Hôpitaux Universitaires La Pitié Salpêtrière -Charles Foix, Service de Neurologie 2, Mazarin, 75013 Paris, France

4. Department of Internal Medicine 2, Sorbonne Université, APHP, Institut Hospitalo–Universitaire de Cardiometabolisme et Nutrition (ICAN), Pitié-Salpêtrière-Charles Foix Hospital, 75013 Paris, France

5. Inserm UMRS 938, Centre de Recherche Saint–Antoine, Sorbonne Université, Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity, Institut Hospitalo–Universitaire de Cardiometabolisme et Nutrition (ICAN), Paris, APHP, Saint Antoine Hospital, Paris, France

6. Nuclear Medicine Dpt, Sorbonne Université, APHP, Pitié-Salpêtrière-Charles Foix Hospital, 75013 Paris, France

7. Department of Endocrinology and Reproductive Medicine, Pitié-Salpêtrière Hospital, APHP, Reference Center for Rare Endocrine Diseases of Growth, Reference Center for Rare Gynecological Pathologies, Institut Hospitalo–Universitaire de Cardiometabolisme et Nutrition (ICAN), Sorbonne University, 75013 Paris, France

8. Pitié-Salpêtrière-Charles Foix Hospital, 75013 Paris, France

9. Reproductive Endocrinology Department, Paris Sud University, APHP, Le Kremlin-Bicêtre, 94275, France

Abstract

Background. Langerhans Cell Histiocytosis (LCH) is a rare inflammatory neoplasm characterized by an infiltration of organs by Langerin + (CD207+) and CD1a+ histiocytes. Diabetes insipidus is a frequent manifestation of the disease, while diabetes mellitus is very rare. We report the first case of a 20-year-old man suffering from hypothalamopituitary histiocytosis and diabetes mellitus with serum anti-insulin receptor antibodies. Case Presentation. A 20-year-old patient was admitted for the evaluation of growth delay and hyperphagia. HbA1c level and fasting blood glucose were in the normal range. The diagnosis of hypothalamopituitary histiocytosis was based on histological features after biopsy of a large suprachiasmatic lesion identified on magnetic resonance imaging (MRI). Association of vinblastine and purinethol was started followed by a second-line therapy by cladribine. During the follow-up, the patient was admitted for recurrence of hyperglycemic states and extreme insulin resistance. The screening for serum anti-insulin receptor antibodies was positive. Each episode of hyperglycemia appeared to be correlated with tumoral activity and increase in serum anti-insulin receptor antibodies and appeared to be improved when the disease was controlled by chemotherapy. Conclusion. We report the first description of a hypothalamopituitary histiocytosis associated with serum anti-insulin receptor antibodies, extreme insulin resistance, and diabetes. Parallel evolution of glucose levels and serum anti-insulin receptor antibodies seemed to be the consequence of immune suppressive properties of cladribine.

Publisher

Hindawi Limited

Subject

Endocrinology, Diabetes and Metabolism

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