Langerhans' cell histiocytosis involving the pituitary, thyroid, lung, and liver

Sujatha Sampathkumar, Cheryl Younger, Harvey Cramer, Naga Chalasani, Paul A. Skierczynski

Research output: Contribution to journalArticle

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Abstract

Objective: To report the development, in an adult patient, of Langerhans' cell histiocytosis (LCH) involving the thyroid and most probably the pituitary gland, lungs, and liver. Methods: We present a case report of a 29-year-old woman who requested a medical consultation because of polyuria and was found to have pituitary dysfunction. We describe the subsequent follow-up, which revealed progressive liver disease that necessitated transplantation and also the presence of a goiter, and discuss the unpredictable pathologic features of LCH. Results: After the diagnosis of central diabetes insipidus and partial hypopituitarism, the patient was diagnosed 2 years later with sclerosing cholangitis. The hepatic involvement was progressive, and she required transplantation and immunosuppression. Three months before liver transplantation, a goiter was discovered. Fine-needle aspiration of the thyroid revealed infiltration by LCH. The goiter decreased in size after chemotherapy with vinblastine and prednisone. Computed tomography of the chest showed bilateral thin-walled cysts, consistent with eosinophilic granulomas. Conclusion: In patients with central diabetes insipidus and pituitary stalk thickening on imaging studies, LCH should be considered in the differential diagnosis. Other hormonal deficiencies may be present initially or may evolve after many years. Thus, continual surveillance is necessary. In addition, an ongoing potential exists for involvement of other organ systems such as the thyroid, liver, and lungs. We suggest consideration of LCH as a possible cause of a goiter in such patients. In our patient, it remains to be seen what effect the immunosuppressive therapy for the liver transplant has on the LCH disease process.

Original languageEnglish
Pages (from-to)217-221
Number of pages5
JournalEndocrine Practice
Volume8
Issue number3
StatePublished - 2002

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Langerhans Cell Histiocytosis
Thyroid Gland
Goiter
Lung
Liver
Neurogenic Diabetes Insipidus
Pituitary Gland
Transplantation
Eosinophilic Granuloma
Polyuria
Sclerosing Cholangitis
Hypopituitarism
Vinblastine
Immunosuppressive Agents
Fine Needle Biopsy
Prednisone
Liver Transplantation
Immunosuppression
Cysts
Liver Diseases

ASJC Scopus subject areas

  • Endocrinology

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Langerhans' cell histiocytosis involving the pituitary, thyroid, lung, and liver. / Sampathkumar, Sujatha; Younger, Cheryl; Cramer, Harvey; Chalasani, Naga; Skierczynski, Paul A.

In: Endocrine Practice, Vol. 8, No. 3, 2002, p. 217-221.

Research output: Contribution to journalArticle

Sampathkumar, S, Younger, C, Cramer, H, Chalasani, N & Skierczynski, PA 2002, 'Langerhans' cell histiocytosis involving the pituitary, thyroid, lung, and liver', Endocrine Practice, vol. 8, no. 3, pp. 217-221.
Sampathkumar, Sujatha ; Younger, Cheryl ; Cramer, Harvey ; Chalasani, Naga ; Skierczynski, Paul A. / Langerhans' cell histiocytosis involving the pituitary, thyroid, lung, and liver. In: Endocrine Practice. 2002 ; Vol. 8, No. 3. pp. 217-221.
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