Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors are dealing with the therapy problems of disseminated Langerhans cell histiocytosis on the basis of seven patients. In the treatment the combination of Vinblastine and Prednisolone was usually applied. The therapy was successful in four cases. The most significant prognostic factors were found to be the presence of organ dysfunction (mainly hepatic and pulmonary), the bone involvement and immunological parameters, respectively. Real therapy goals are stressed.
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PMID:[Therapeutic problems in disseminated histiocytosis X]. 833 43

GVHD is the most common and well-known cause of morbidity and mortality following allogeneic BM transplantation. The GVHD following OLT is an uncommon complication but has a high mortality and poses a major diagnostic and therapeutic challenge. We herein discussed a 12-month-old girl with multi-system LCH, who developed end-stage liver disease despite intensive chemotherapy. She underwent ABO-compatible liver transplantation at 28 months while in remission from LCH. The donor was her 26-yr-old father. Post-operative course was uneventful. The GVHD manifested with skin rash and BM suppression on post-transplant day 94 and confirmed by both microchimerism and skin biopsy. Prednisolone, basiliximab, and ATG were administered immediately but the bone marrow suppression was not improved and the patient died because of Candida sepsis at six-month post-transplant. GVHD after OLT should be keep in mind in patients with rash and BM suppression after liver transplantation. In LDLT, a patient who carries risk factors should investigated for optimal HLA matching.
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PMID:Late-onset graft-versus-host disease after pediatric living-related liver transplantation for Langerhans cell histiocytosis. 2188 42

A 16-year-old girl was referred for the evaluation of headache exacerbation and progressive loss of visual field from one month ago. She also suffered from intermittent diarrhea since 12 months ago and secondary amenorrhea, headache, weight loss (4 - 5 kg) and weakness from six months ago. She had a history of transient polydipsia and excessive urine output during this period. Brain Magnetic Resonance Imaging (MRI) reported a 15 x 15 x 9 millimeters mass lesion in the sellar region. It was extended to the suprasellar cistern with mild compression of the optic chiasm and mild thickening of the pituitary stalk with posterior displacement were reported. In an exisional biopsy of pituitary stalk lesion, the pathology result was indicative of Langerhans cell histiocytosis (LCH). The patient underwent four periods of chemotherapy with prednisolone and vinblastin in 28-day intervals followed by one cycle of radiation therapy. In three months follow up after treatment the tumor size was reduced, Levothyroxin and Prednisolone were tapered, and pituitary hormones were improved.
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PMID:Pituitary stalk thickening in a case of langerhans cell histiocytosis. 2577 95