Letterer-siwe disease in adults

Novice, Fred M.; Collison, Daniel W.; Kleinsmith, D'Anne M.; Osband, Michael E.; Burdakin, John H.; Coskey, Ralph J.

doi:10.1002/1097-0142(19890101)63:1<166::aid-cncr2820630127>3.0.co;2-a

Cited by 30 publications

(18 citation statements)

References 67 publications

(2 reference statements)

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“…Evidence, however, suggests that at least some examples of disseminated disease with features of Letterer-Siwe syndrome are examples of LCH [6] . It was once thought that LCH most likely represented a reactive disorder rather than a neoplastic process.…”

Section: Discussionmentioning

confidence: 99%

Langerhans cell histiocytosis

2013

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Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which Langerhans's cells accumulate in a variety of organs. LCH affects mostly children and adolescents, involves one or many body systems or tissues such as bone, lung, hypothalamus, posterior pituitary gland, skin, lymph node, liver, and various soft tissues. The clinical features of LCH depend on the extent of involvement. Here in, we report a case of 2-year-old girl presented with fever, swelling in front and behind of right ear and ear discharge. She had anaemia and lytic lesions on skull x-ray and high resolution computed tomography. Biopsy was performed and histopathology and immunohistochemistry fi ndings suggested diagnosis of LCH.

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Section: Discussionmentioning

confidence: 99%

Langerhans cell histiocytosis

2013

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“…Good evidence, however, suggests that at least some examples of disseminated disease with features of Letterer-Siwe disease are examples of LCH. 14,15 In 1985 physicians from many parts of the world formed the International Histiocyte Society, which aimed at a better understanding of LCH -and also to improve its therapy. 3 In Textbook of Dermatology, Chu, in 1998, 16 stated: 'The histiocytoses have been separated into three classes according to our current understanding of the biology of these diseases.…”

Section: Discussionmentioning

confidence: 99%

Lessons to be learned — Langerhans' cell histiocytosis

Dewan

Al-Ghamdi

Zahrani

2008

Journal of the Royal Society for the Promotion of Health

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Langerhans' cell histiocytosis (LCH) is a neoplastic disease due to uncontrolled proliferation of Langerhans' cells (LCs); damage to organs involved appears to be due to the various cytokines secreted. The true cause of LCH remains a mystery. LCH can present at any age; there is female predominance and the skin is often the first site to be involved. Scaly papules, vesicles, purpuric nodules, plaques and ulcers are the main diverse presentations typically found on the scalp, flexures (including the perineum), crease below the breast, palms, soles and nails. The patient described here is a 35-year-old Saudi Arabian woman with a vulval ulcer that had been misdiagnosed as an infective lesion 11 months before the correct diagnosis of LCH was made by means of biopsy examination. Diffuse infiltration of LCs with large pale reniform/convoluted nuclei and abundant amphophilic cytoplasm in the company of neutrophils, eosinophils, lymphocytes and plasma cells were observed on light microscopy. Immunohistochemical staining, indicating S100 protein positivity in LCs and the demonstration of Birbeck granules by means of electron microscopy, confirmed the diagnosis. The 'skin-only' LCH may later progress to involve other organs; alternatively skin lesions may be part of the multisystem, disseminated disease. There is need for careful follow-up of all LCH patients, as the course of the disease cannot be reliably predicted from either the clinical presentation or histology of the lesion. In many cases the prognosis is good, there being limited involvement that does not progress. Surgery or local steroids might suffice as therapy. However, for severely symptomatic disease, in which there are complications associated with dysfunction (or failure) of vital organs such as the liver, lungs, and central nervous system, the use of a variety of immunosuppressive treatments--and the recently described 2-chloro-2-deoxyadenosine (2CdA) (cladribine)--have been suggested; however, the type and extent of disease that will respond to this therapy has not yet been clarified. Solid laboratory research and rigorous clinical trials are required for more effective and less toxic therapies for patients with progressive LCH.

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“…LCs belong to the mononuclear phagocyte system and can infiltrate virtually any site of the body, and LCH may occur as a localized lesion or widespread systemic disease [1][2][3]. The clinical manifestation of LCH is extremely variable, and the involvement of skeletal, cutaneous, hematolymphoid, pulmonary, and pituitary systems is often seen [4][5][6][7][8][9][10][11][12][13][14][15][16]. The clinical course of the disease is unpredictable, varying from spontaneous regression to rapid progression and death.…”

Section: Introductionmentioning

confidence: 99%

A case of adult Langerhans cell histiocytosis showing successfully regenerated osseous tissue of the skull after chemotherapy

et al. 2008

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Langerhans cell histiocytosis (LCH) is a proliferative disorder of Langerhans cells and extremely rare in adults. Adult LCH is often associated with osteolytic bone lesions, but large bone-defective lesions have been rarely reported. We report an adult case of LCH accompanied by large osteolytic lesions in the skull that successfully responded to chemotherapy. A 47-year-old woman with LCH who had multiple, large osteolytic areas of more than 3 cm in diameter in the skull was admitted to our hospital. She was treated with systemic chemotherapy consisting of prednisolone, vinblastine, and 6-mercaptopurine. Twelve months later, when she completed the treatment, osteolytic areas were covered with hard osseous tissue, and X-ray examination confirmed regeneration of the bone. This case indicates that chemotherapy can be effective even for the treatment of large osteolytic lesions in adult LCH patients.

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Letterer-siwe disease in adults

Cited by 30 publications

References 67 publications

Langerhans cell histiocytosis

Langerhans cell histiocytosis

Lessons to be learned — Langerhans' cell histiocytosis

A case of adult Langerhans cell histiocytosis showing successfully regenerated osseous tissue of the skull after chemotherapy

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