For localized forms in skin or bone structures, watchful waiting is recommended. Langerhans cell histiocytosis infiltration into pancreas and kidney. Its clinical heterogeneity is illustrated by the fact that it was historically described as three different disease entities, based on disease extent and severity. LCH may be localized, when a single organ is affected unifocal or multifocal , or systemic. Introduction Histiocytoses are a heterogeneous group of diseases characterized by the proliferation of cells from the mononuclear phagocyte system. To our knowledge, few studies have selected only patients with a diagnosis of histiocytosis without endocrine involvement. Pituitary TD 10 Gy 0.
One patient recovered all axes five years after the administration of fractionated stereotactic radiation therapy RT to the sellar mass. Base of skull TD English pdf Article in xml format Article references How to cite this article Automatic translation. Cancer Invest ;27 5: Bilateral exophthalmos occurred in , and computed tomography CT of the orbit showed a retro-orbital mass. Radiology, , pp.
The only remaining signs are a slight coxa vara deformity secondary to the right femoral neck scar Fig. 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. Report from the International Registry of the Histiocyte Society. Marked clinical and laboratory improvement with vemurafenib.
Hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.
N Engl J Med,pp. Change over time in hormone deficiencies and pituitary MRI at diagnosis. Pediatr Blood Cancer, 60pp. The purpose of this article was to analyze the frequency and progression of pituitary hormone deficiencies in a series of nine patients with LCH and ECD and HPA involvement.
A high index of suspicion is key to early diagnosis. Extra-osseous involvement of Langerhans cell histiocytosis in children. Pituitary adenoma associated with No patient died during follow-up. Cutaneous Langerhans cell histiocytosis in children under one year.
Current treatment and future directions. Histological examination of a bone biopsy from the left lower limb was consistent with the same condition. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal’s impact. Morbidity, treatment, and course. The initial intensive phase of chemotherapy consisted of daily prednisone and weekly vinblastine for a total of 6 weeks.
Langerhans cell histiocytosis: A case presentation and literature review
On the other hand, ECD mainly affects adults between the fifth and seventh decades of histikcytosis. SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. Base of skull TD Mayo Clin Proc, 89pp.
Subtotal resection was hypopithitarism, and the weven examination was consistent with ECD. Median age at diagnosis was 38 years range, 3— Any child with a suspected solitary LCH lesion must have a full diagnostic work-up to exclude multiple lesions.
The radiographic appearance depends on the phase of the disease. Main clinical characteristics of the nine patients of the series. This item has received. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two receding years.
One of them, as already discussed, recovered from all hormone deficiencies five years after treatment.
Consensus guidelines for the diagnosis and clinical management of Erdheim—Chester disease. DI is the most common endocrine deficiency. Clin Endocrinol Oxfhisstiocytosispp. In adults, as noted above, the most common non-endocrine signs occur in bone, lung, and skin.
Langerhans cell histiocytosis involving the thyroid and parathyroid glands.
The second-line treatment consisted of fortnightly methotrexate infusions, weekly vinblastine and daily oral prednisone, for a total of 6 weeks.