Endocrine Abstracts

Introduction: Langerhans cell histiocytosis (LCH) is a very rare haematological disease, with difficult diagnosis for its very variable clinical picture. It is caused by monoclonal proliferation of cutaneous Langerhans cells. It affects most commonly the skin and bones, less often bone marrow, liver, pituitary and CNS.

Case report: A 74-year-old female patient with progressive sclerosing cholangitis was referred to a transplant centre to consider liver transplant treatment options. Langerhans cell histiocytosis (LCH) was diagnosed by a liver biopsy. Other examinations, including trepanobiopsy and PET/CT, revealed no other lesions of LCH. The finding was evaluated by the hematologist as unifocal histiocytosis with isolated liver involvement at MGUS level. After the examination, the patient underwent liver transplantation. Immunosuppressive therapy (tacrolimus, mycophenolate mofetil and corticoids) was initiated. Postoperative course was significantly complicated by bleeding and biliary leak, requiring repeated surgical revisions, protracted abdominal infection and repeated need for percutaneous biloma drainage. Chronic sepsis, malnutrition, sarcopenia, decreased mobility and depressive syndrome dominated in the clinical picture. Two months after transplantation, polyuria (of about 7 – 10 litres/24 hours) appeared. The fluid withdrawal test confirmed suspected diabetes insipidus. Etiopathogenetically, there was a possible association with the administration of linezolid in antibiotic therapy, but also with the underlying histiocytosis. After the application of desmopressin (in doses of 60 – 300 µg/day) the polyuria gradually decreased to diuresis about 3 litres/24 h. CT examination described a small pituitary bearing, possible granuloma in histiocytosis or adenoma. Later, the MR region of the Turkish saddle was performed showing a very small pituitary volume with an empty-sella and slight infundibulum enlargement indicating possible discrete LCH in this region. Pituitary profile revealed hyperprolactinaemia (1912 mIU/l), low levels of LH, FSH and TSH, and normal levels of ACTH as well as cortisol. Cabergoline was added to the therapy. Its administration in a small dose (0.5 mg/week) led to a rapid decrease of prolactinemia. The gradual withdrawal of desmopressin did not cause polyuria to return. Currently, the patient is in a satisfactory clinical condition and except cabergoline treated only with a small dose of corticoids as a part of her maintenance immunosuppressive regiment.

Conclusion: We point to a rare hematological disease complicated with probably related endocrinopathy.