Diabetes Insipedes in a child disclosing localized Langerhans’ Cell Histiocytosis

  • Kamel El-Reshaid Department of Medicine, Faculty of Medicine, Kuwait University
  • Shaikha Al-Bader Department of Medicine, Nephrology Unit, Amiri Hospital, Ministry of health, Kuwait
  • Zaneta Markova Radiology Unit, IMC, Kuwait

Abstract

Langerhans cell histiocytosis (LCH) is monoclonal neoplastic condition of aberrant bone marrow histiocytes.  The latter are part of the innate immune system and certain exogenous/endogenous stimuli may trigger its expansion.  Hence LCH can present with limited or multiple organ involvement that may include; bones, lung, endocrine, skin, lymph nodes, spleen and bone marrow.  In this case report, we describe a 3-year-old boy who presented with severe polyuria and polydipsia.  Laboratory investigations were consistent with diabetes insipidus (DI).  MRI of the brain; confirmed absence of the bright spot in his pituitary gland did not show evidence of tumor or enlargement by inflammation.  Moreover, MRI revealed 2 skull lesions and their subsequent biopsy confirmed LCH.  Systemic examination and tests including PET scan did not show additional lesions.  Since his disease was localized, he received only Desmopressin acetate 120 ug twice daily for his DI without surgery, radiotherapy or chemotherapy.  One year later, his disease remained limited to DI and the 2 bonny lesions. 


Keywords: bone, diabetes insipidus, pituitary, desmopressin, Langerhans cell histiocytosis.

Keywords: bone, diabetes insipidus, pituitary, desmopressin, Langerhans cell histiocytosis

Downloads

Download data is not yet available.

Author Biographies

Kamel El-Reshaid, Department of Medicine, Faculty of Medicine, Kuwait University

Department of Medicine, Faculty of Medicine, Kuwait University

Shaikha Al-Bader, Department of Medicine, Nephrology Unit, Amiri Hospital, Ministry of health, Kuwait

Department of Medicine, Nephrology Unit, Amiri Hospital, Ministry of health, Kuwait

Zaneta Markova, Radiology Unit, IMC, Kuwait

Radiology Unit, IMC, Kuwait

References

1. Maghnie M, Cosi G, Genovese E, et al. Central diabetes insipidus in children and young adults. N Engl J Med 2000; 343:998-1007.
2. Bourque CW. Central mechanisms of osmosensation and systemic osmoregulation. Nature Reviews. Neuroscience 2008; 9: 519–531.
3. Pablo S, Tipton GA, Chan JC. Diabetes insipidus. Pediatr Rev 2000; 21:122-129.
4. Jaffe R, Weiss LM, Facchetti F. Tumours derived from Langerhans cells. In: Swerdlow SH, Campo E, Harris NL, et al, eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC Press; 2008:358–360. World Health Organization Classification of Tumours; vol 2.
5. Charles M. Harmon and Noah Brown. Langerhans Cell Histiocytosis: A Clinicopathologic Review and Molecular Pathogenetic Update. Arch Pathol Lab Med 2015; 139:1211-1214.
6. Psychogenic polydipsia-Management-Step by step. British Medical Journal. 5 May 2016.
7. Ghirardello S, Garre ML, Rossi A, Maghnie M: The diagnosis of children with central diabetes insipidus. J Pediatr Endocrinol Metab 2007; 20: 359–375.
8. Lucas JW, Zada G. Imaging of the pituitary and parasellar region. Semin Neurol 2012; 32:320–331.
9. Yu RC, Chu C, Buluwela L, Chu AC. Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis. Lancet 1994; 343:767–768.
10. Badalian-Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood 2010; 116:1919–1923.
11. Roden AC, Hu X, Kip S, et al. BRAF V600E expression in Langerhans cell histiocytosis: clinical and immunohistochemical study on 25 pulmonary and 54 extrapulmonary cases. Am J Surg Pathol 2014; 38:548–551.
12. Degar BA, Fleming MD, Rollins BJ, Rodriguez-Galindo C. Histiocytoses. In: Orkin SH, Fisher DE, Ginsburg D, Look AT, Lux SE, Nathan DG, eds. Nathan and Oski's Hematology and Oncology of Infancy and Childhood. 8th ed. Philadelphia, PA: Elsevier Saunders; 2015; 2100–2122.
13. Minkov M. Multisystem Langerhans cell histiocytosis in children: current treatment and future directions. Paediatr Drugs 2011; 13: 75-86.
14. Grois N, Potschger U, Prosch H, Minkov M, Arico M, Braier J, Henter JI, Janka-Schaub G, Ladisch S, Ritter J, Steiner M, Unger E, Gadner H: Risk factors for diabetes insipidus in Langerhans cell histiocytosis. Pediatr Blood Cancer 2006; 46:228–233.
15. Grois N, Prayer D, Prosch H, et al. Course and clinical impact of magnetic resonance imaging findings in diabetes insipidus associated with Langerhans cell histiocytosis. Pediatr Blood Cancer 2004; 43:59-65.
16. Imashuku S, Shioda Y, Kobayashi R, et al. Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group. Haematologica 2008; 93:615-618.
Statistics
7 Views | 8 Downloads
How to Cite
1.
El-Reshaid K, Al-Bader S, Markova Z. Diabetes Insipedes in a child disclosing localized Langerhans’ Cell Histiocytosis. JDDT [Internet]. 15Jul.2020 [cited 30Sep.2020];10(4):213-5. Available from: http://www.jddtonline.info/index.php/jddt/article/view/4181