HYPOPITUITARISM IN LANGERHANS CELL HISTIOCYTOSIS SEVEN CASES AND LITERATURE REVIEW

Two patients had anterior panhypopituitarism at diagnosis, three had one or more anterior deficiencies, and four patients had an intact anterior pituitary gland. LCH is a disease of unknown aetiology that is characterised by extreme clinical heterogeneity. Services on Demand Article. Blood, , pp. Systemic therapy is indicated to prevent irreversible damage to normal tissue and long-term consequences. Pediatric Drugs ;13 2:

Mayo Clin Proc, 89 , pp. The treatment administered and the course of the disease varied widely depending on organ involvement. Sella-suprasellar mass in a patient with Erdheim—Chester disease.. Tibia, distal fibula, metatarsus and tarsus bilateral 0. Se continuar a navegar, consideramos que aceita o seu uso. Eur J Cancer, 39 , pp.

Diagnosis of LCH was based on verification of the presence of Birbeck granules by electron microscopy and antigen CD1a or langerin CD on the cell surface. Br J Cancer, Suppl.

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

Regular monitoring of these patients is recommended. Discussion LCH is a disease of unknown aetiology that is characterised by extreme clinical heterogeneity. Features of LCH that indicate a poor prognosis include age of less than 2 years at hypopititarism, any risk organ involvement bone marrow, blood, liver and spleenorgan dysfunction, multi-system disease and unsatisfactory response to initial treatment.

Biol Histiocygosis Marrow Transplant ;16 1: Two of the seven patients with LCH had localized involvement, and five systemic diseases Table 1.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Pituitary deficiencies in our study were not exactly comparable to those reported in the literature, which includes patients diagnosed with histiocytosis both with and without HPA involvement.

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The treatment administered and the course of the disease varied widely depending on organ involvement.

Hormone measurements were performed at the start of clinical signs and every 1—2 years for axis re-evaluation. Localized forms of LCH usually have a slow course and a good response to treatment, 4 while systemic forms involving liver, spleen, bone or lung, young adults, or poor response to chemotherapy are poor prognostic factors.

Cited by Google Similars in Google. After confirmation of the BRAFVE mutation, the patient started treatment with vemurafenib, which has resulted in marked clinical and laboratory improvement. Trends Endocrinol Metab, 18pp.

SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. When baseline hormone measurements clel not conclusive for diagnosis, pituitary reserve was studied using dynamic tests: Subscribe to our newsletter.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Four patients initially had elevated serum prolactin levels, which were less than four times the upper limit of normal in three, and seven times the upper limit hisiocytosis normal in the remaining patient. Three years later, his skin lesions worsened and required oral prednisolone for three years.

Radiology,pp. In adults, mean age at diagnosis is 33 years. Langerhans cell Histiocytosis in adults. Seven patients had non-endocrine involvement in bone 5 patientslung 3 patientsorbital soft tissue 2 patientsand skin 1 patient.

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

Histiocytosis follows an unpredictable course, sometimes consisting of bouts of the disease. LCH is primarily a paediatric disease: Hematol Litedature Clin North Am, 12pp. Report from the International Registry of the Histiocyte Society. A final reviwe of LCH with multifocal skeletal involvement only was made. One of them, as already discussed, recovered from all hormone deficiencies five years after treatment. The second-line treatment consisted of fortnightly methotrexate infusions, weekly vinblastine and daily oral prednisone, for a total of 6 weeks.

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Reevaluation imaging skeletal survey, skeletal isotopes and CT of the facial bones after the initial intensive phase showed that the patient still had active disease in some of the initially affected areas.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Pituitary TD 10 Gy 0. As regards RT, it is used for localized lesions with compression symptoms, but response is usually less marked than in LCH. Any bone can be affected, but most commonly the skull Fig.

In adults, ln noted above, the most common non-endocrine signs occur in bone, lung, and skin.

Irreversible long-term deficits that develop in areas of active LCH ij small stature, growth hormone deficiency, hypothyroidism, hearing loss, cerebellar ataxia, orthopaedic deficits, biliary cirrhosis and portal hypertension.

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