HYPOPITUITARISM IN LANGERHANS CELL HISTIOCYTOSIS SEVEN CASES AND LITERATURE REVIEW

Pituitary stalk thickening in a patient with Langerhans cell histiocytosis. Histological examination confirmed the diagnosis of Langerhans cell histiocytosis LCH. The variables analyzed included sex, age at diagnosis, disease duration, first symptom, form of disease localized or systemic , type of endocrine and non-endocrine involvement, treatment, and radiographic findings at symptom start and during the course of the disease. J Endocrinol Invest, 24 , pp. Morbidity, treatment, and course. As regards imaging tests, pituitary MRI after the administration of gadolinium is the procedure of choice for assessing the HPA region. Cancer, 67 , pp.

Pituitary MRI only showed an absence of hypersignal of the neurohypophysis. To our knowledge, few studies have selected only patients with a diagnosis of histiocytosis without endocrine involvement. In this last case, moreover, the deficiencies were reversed after radiation therapy.. DI was present at diagnosis in five patients, and developed subsequently in the other three patients two at one year and one at 10 years of diagnosis Table Median follow-up time was 12 years range, 4— Imaging repeated just before the end of the continuation phase showed that all of the initial lesions had disappeared, but a new lesion was present on the medial aspect of the left ilium. Sellar and suprasellar mass 0 and 1.

A patient with LCH with skin involvement received topical corticosteroid treatment. Material and methods A retrospective, observational study was conducted to analyze the clinical data from nine patients 5 women and 4 men with a mean age 52 years range, 21—74 with a histological diagnosis of histiocytosis and HPA involvement 7 with LCH and 2 with ECD. Pulmonary involvement in Erdheim—Chester disease: Chemotherapy treatment using a scheme similar to that used in LCH should also be considered as second line treatment.

  DANSK SKRIFTLIG EKSAMEN STX ESSAY

Course and clinical impact of magnetic resonance imaging findings in diabetes insipidus associated with Langerhans cell histiocytosis. Hypothalamic involvement was defined as the presence of any of the following signs or symptoms: Imaging repeated just before the end of the continuation phase showed that all of the initial lesions had disappeared, but a new lesion was present on the medial aspect of the left ilium.

During follow-up, the sellar and parasellar masses increased in size, requiring repeat surgery and the subsequent administration of fractionated stereotactic RT. English pdf Article in xml format Article references How to cite this article Automatic translation.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Langerhans cell histiocytosis LCH: Replacement therapy was therefore started. Instructions for authors Submit an article Ethics in publishing.

Hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.

There is also an increased incidence of malignancy in long-term survivors. Treatment of Langerhans cell histiocytosis. Blood,pp. You can change the settings or obtain more information by clicking here.

Multisystem Langerhans cell histiocytosis in children: Hematol Oncol Clin North Am, 12pp. DI is the most common involvement at diagnosis.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Drugs that inhibit BRAF serine—threonine kinase, such as vemurafenib 27,28 and imatinib, have shown promising results in small patient series and clinical trials in terms of clinical and radiographic disease improvement, as occurred in one patient from our series.

Treatment was started with glucocorticoids and azathioprine, with no response. This study analyzed the frequency and progression of pituitary hormone deficiencies and the radiographic findings in 9 patients 7 with LCH and 2 with ECD with hypothalamic—pituitary HP axis. Regular monitoring of these patients is recommended.

  MS67 COMMON ESSAY

Hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.

Three years later, his skin lesions worsened and required oral prednisolone for three years. Langerhans cell histiocytosis—clinical and epidemiological aspects. A control pituitary MRI showed an enlargement of the sellar and parasellar masses and the occurrence of tentorial lesions, which required the administration of RT again and the histtiocytosis of steroid treatment.

Seven patients had wnd involvement in bone 5 patientslung 3 patientsorbital soft tissue 2 patientsand skin 1 patient. DI is the most common endocrine deficiency.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Are you a health professional able to prescribe or dispense drugs? Two of the seven patients with LCH had localized involvement, and five systemic diseases Table A final diagnosis of LCH with multifocal skeletal involvement only was made. Inthe patient attended our hospital complaining of bone pain in the lower limbs over the previous three years and clinical symptoms of polyuria and polydipsia for 10 years. A second initiation phase was therefore commenced.

In conclusion, HPA involvement is relatively common in patients with histiocytosis. Today most investigators consider LCH to result from an aberrant immune response, but the stimulus for clonal Langerhans cell proliferation remains unknown.

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Regular monitoring of these patients is recommended.