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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two infants suffering from histiocytosis X were treated successfully with a topically applied nitrogen mustard. All cutaneous symptoms disappeared completely within 2 weeks in one case while the skin improved significantly in the other. No irritation or dermatitis was experienced from the treatment. The only side-effect noted was hyperpigmentation.
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PMID:Histiocytosis X in two infants--treated with topical nitrogen mustard. 31 16

In 16 children with multisystem Langerhans cell histiocytosis (mean age 22 months, range 5 to 36 months) severe symptomatic skin involvement was treated with topical nitrogen mustard (mechlorethamine hydrochloride). In each case, rapid clinical improvement occurred within 10 days; subsequent complete healing was observed in 14 children, and partial healing in 2 others in whom treatment was a component of palliative care. Mean duration of treatment was 3.5 months (range 2 to 6 months). Systemic treatment was averted in 11 patients because response to topical therapy was so favorable, but bone marrow or respiratory failure led to a fatal outcome in 5 other patients. Adverse effects were minimal. One patient developed contact allergy to topical nitrogen mustard after 2 years of intermittent therapy, but was successfully desensitized and was then able to continue treatment. We conclude that the topical application of nitrogen mustard is an effective treatment for cutaneous Langerhans cell histiocytosis. Although adverse effects were minimal in the short term, there remains concern about the possibility of long-term cutaneous carcinogenicity.
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PMID:Topical nitrogen mustard: an effective treatment for cutaneous Langerhans cell histiocytosis. 186 Dec 22

Prompted by evidence that Langerhans cell histiocytosis (LCH) is a nonmalignant disorder of immune regulation, we used cyclosporine (12 mg/kg/d orally) to treat three young children with advanced multisystem LCH. All three patients had partial responses to cyclosporine within 2 months of therapy, as evidenced by complete resolution of organ dysfunction and regression of the majority of lesions. Complete responses were attained by adding relatively nontoxic chemotherapy (ie, prednisone and vinblastine). Toxicity from cyclosporine comprised mild and reversible elevations of the serum creatinine and blood urea nitrogen. These results indicate that further evaluation of cyclosporine for the treatment of patients with advanced LCH is warranted.
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PMID:Cyclosporine therapy for advanced Langerhans cell histiocytosis. 179 85

Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Four new cases are reported, and there is a review of the 38 cases in the literature. This disease may involve the vulva, vagina, cervix, endometrium, and ovary. Four distinct patient groups, segregated on the basis of initial presentation and subsequent anatomic extent of disease, were categorized as follows: (1) "pure" genital LCH, (2) genital LCH with subsequent multi-organ involvement, (3) oral or cutaneous LCH with subsequent genital and multi-organ involvement, and (4) diabetes insipidus with subsequent genital and multi-organ disease. Although involvement of the genital tract can occur at any age, it is most common in young adulthood. Clinically, LCH may mimic either primary neoplasia or various inflammatory lesions; the major pathologic differential diagnosis is venereal and other inflammatory diseases. The pure genital form may have a distinct nosologic position in the spectrum of LCH similar to the "pure," self-limited cutaneous histiocytosis seen in infants. There is no correlation between histologic findings and the outcome of the genital lesions. There is also no correlation between clinical presentation and/or the extent of involvement and outcome of genital lesions; complete regression, partial improvement, persistent lesions, and recurrences were seen in all four groups of patients. The treatment of genital LCH is not well defined and is highly individualized. Therapy has included surgery, radiation, topical corticosteroids, topical nitrogen mustard, systemic chemotherapy, and combination therapy; mixed results were obtained with all treatment modalities. Although no modality has been shown to yield a superior outcome, complete surgical excision is advocated as initial therapy.
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PMID:Langerhans cell histiocytosis of the female genital tract. 200 54

Histiocytosis X (HX) is a rare disorder of histiocytic proliferation characterized by a broad spectrum of clinicopathologic disease. An unusual case of Letterer-Siwe disease (LSD) or subacute disseminated HX in a 71-year-old woman is presented. The patient had a 3-year history of splenomegaly before skin lesions developed. She presented to our clinic at 1.5 years later and the diagnosis of HX was made by skin biopsy. Topical nitrogen mustard (NM) therapy resulted in complete clearing of cutaneous lesions. Her condition was stable over the next 10 months. However, she subsequently suffered a rapid and fatal dissemination of her disease. Systemic treatment with prednisone, vinblastine sulphate, and suppressin A (SA) (a calf thymus derived hormone preparation that specifically induces suppressor T-cells) was ineffective. Characteristic histopathologic, immunohistochemical, and electron microscopic findings of HX are illustrated. A review of the adult cases of LSD and treatment options for HX are presented and discussed.
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PMID:Letterer-Siwe disease in adults. 264 29

The case histories of two elderly patients with cutaneous histiocytosis X treated topically with nitrogen mustard are presented. The cutaneous lesions cleared within 2 to 3 weeks, and remission was maintained with daily topical administration of nitrogen mustard. The clinical impression of improvement was substantiated by light and electron microscopic studies prior to and after therapy.
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PMID:Histiocytosis X: treatment with topical nitrogen mustard. 696 94

Otitis externa is a common symptom of Langerhans' cell histiocytosis, and occurs in half of all children with the disease. Previous treatment regima have been either unsuccessful, or associated with undesirable side-effects. This study describes the successful use of topical 20% nitrogen mustard ear drops in the management of five children who had Langerhans' cell histiocytosis with otitis externa. All of them had previously been resistant to other forms of treatment. The children were aged between 6 months and 6 years. The otitis externa fee period to date is up to 5 years. Topical nitrogen mustard ear drops are rapidly effective, can be administered at home, have not caused any adverse effects, and are of long-term benefit.
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PMID:Otitis externa in Langerhans' cell histiocytosis--the successful use of topical nitrogen mustard. 806

We present the case of a 27-year-old Caucasian woman who suffered from Langerhans cell histiocytosis with axillary and scalp involvement. She also developed diabetes insipidus after 2 years of skin lesions. Topical nitrogen mustard therapy was performed for the skin lesions, but we had to stop this therapy because of severe local irritation and systemic urticaria. Afterwards, we administered etoposide systemically, but no improvement appeared in 6 weeks. Finally we used methotrexate for 3 months and the result was very good at the end of the first month.
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PMID:Langerhans cell histiocytosis in an adult. 807 73

The authors carried out a retrospective analysis of 131 cases of Langerhans' cell histiocytosis treated at two centers over a 30-year period. All those with head and neck manifestations have been reviewed with particular reference to management and outcome. The role of chemotherapy, radiotherapy, and surgery is discussed, and we present our results of the successful introduction of topical nitrogen mustard (Mustine) ear drops for meatal skin involvement. During the period of the study our modus operandi has evolved from an aggressive medical and surgical approach, favored early in the series, to our present more conservative approach, with an attendant reduction in morbidity.
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PMID:Langerhans' cell histiocytosis in childhood: management of head and neck manifestations. 829 58

Langerhans cell histiocytosis (LCH) is an enigmatic disease usually occurring in children. Tumor lysis syndrome (TLS) is a clinical syndrome associated with severe metabolic derangement and oliguric acute renal failure. In this report, we present the clinical course of an infant with advanced LCH who had TLS develop after chemotherapy. Treatment with continuous arteriovenous hemofiltration resulted in effective control of serum uric acid, potassium, creatinine, phosphorus, and blood urea nitrogen levels in the blood.
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PMID:Tumor lysis syndrome in an infant with Langerhans cell histiocytosis successfully treated using continuous arteriovenous hemofiltration. 1121 9


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