Spontaneous Remission of Multi-System Histiocytosis X

Broadbent, Valerie; Davies, E Graham; Heaf, D P; Pincott, J. R.; Pritchard, Jon; Levinsky, R J; Atherton, D.J.; Tucker, Stephen B.

doi:10.1016/s0140-6736(84)90127-2

Cited by 142 publications

(55 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…4,14 Spontaneous remission of EG in other sites has been reported, 1,3,5 but most reports of calvarial EG indicate that the majority of cases are immediately referred for excision. Oliveira et al 11 reported 4 cases of calvarial EG in which the lesion resolved even before surgery was performed.…”

Section: Discussionmentioning

confidence: 99%

Nonoperative management of solitary eosinophilic granulomas of the calvaria

Angulo

Nair

Lee

et al. 2013

PED

View full text Add to dashboard Cite

Object Solitary eosinophilic granuloma (EG) of the calvaria is most commonly treated with surgical excision. The authors hypothesize that many solitary EGs will resolve without intervention, and observation may be a reasonable option. This study was undertaken to investigate that hypothesis. Methods The authors reviewed their institutional records and identified 14 cases of solitary calvarial EG. In 6 cases the patients underwent resection based on family and/or neurosurgeon preferences. A strategy of nonoperative management (purposeful observation) was chosen for the other 8 cases. The authors report the clinical course and imaging results in these 8 cases. Results One of the 8 patients underwent surgery 2 months after presentation because of slight enlargement of the lesion and increasing pain. After a median follow-up period of 1 year (range 6–19 months), none of the other patients had required surgery. Five of these 7 patients had pain at presentation. Pain resolved completely in all 5. The remaining 2 remained asymptomatic. Complete resolution of pain was reported in the 5 patients who had pain at presentation. There was complete clinical resolution of the palpable soft-tissue lesion in all 7 cases. Complete radiographic resolution of the lesion was observed in 5 cases and near-complete resolution in the remaining 2. Conclusions Observation is a safe and reasonable approach in the management of solitary calvarial EG and may prevent unnecessary surgical interventions.

show abstract

Section: Discussionmentioning

confidence: 99%

Nonoperative management of solitary eosinophilic granulomas of the calvaria

Angulo

Nair

Lee

et al. 2013

PED

View full text Add to dashboard Cite

show abstract

“…1,2 It can follow a mild clinical course and even resolve spontaneously, 3,4 but it can also involve multiple organ systems with fatal consequences in 20% of disseminated cases. LCH's etiology is unknown.…”

Section: Introductionmentioning

confidence: 99%

“…LCH's etiology is unknown. The benign morphology of its proliferating cells and its characteristic inflammatory infiltrates suggest that LCH may be an inflammatory disorder, 3 and dysregulated expression of inflammatory cytokines, such as interleukin-17A, has been reported. 5 However, the pathologic Langerhans cells (LCs) in LCH are clonal.…”

Section: Introductionmentioning

confidence: 99%

Recurrent BRAF mutations in Langerhans cell histiocytosis

Badalian-Very

Vergilio

Degar

et al. 2010

Blood

1,040

841

View full text Add to dashboard Cite

show abstract

“…Because LCH has been demonstrated to be a monoclonal condition, it has been considered a neoplastic disorder [2,3]. However, the benign histopathological appearance of the lesions, the occurrence of spontaneous remissions [4], and the ability to respond to immunomodulation [5] suggest at least in some cases a reactive clonal disorder rather than a malignant process.…”

Section: Introductionmentioning

confidence: 99%

Treatment of children with Langerhans cell histiocytosis with 2‐chlorodeoxyadenosine

et al. 2002

View full text Add to dashboard Cite

Langerhans cell histiocytosis (LCH) is a disorder characterized by proliferation of activated Langerhans cells. Immune dysregulation is believed to be part of the pathogenesis. Although current therapies are very effective at inducing remission, multiple recurrences and long-term sequelae are common for patients with low-risk disease, and a signi®cant proportion of young patients die of their disease. More effective therapies based on the pathogenesis of LCH are needed. We investigated the use of 2-chloro-deoxyadenosine (2-CdA), a purine analogue with an antiproliferative effect on histiocytes and lymphocytes, in patients with recurrent or high-risk LCH. Six patients with recurrent LCH received 2-CdA (5±7 mg/m 2 /day for 5 days, repeated every 21±28 days). All patients achieved remission. With a median follow-up of 15 months (range, 3±25 months), 5 patients remain in remission. A patient with multisystem disease who recurred after 13 months, achieved a second remission with 2-CdA. Hematologic toxicity was minimal, and no infectious complications were documented. 2-CdA is among the most effective drugs for the treatment of LCH, and this is probably due to both its anti-proliferative and immunomodulatory effects. 2-CdA needs to be considered for the treatment of recurrent LCH. However, its incorporation into front-line treatment of patients with multi-system LCH needs further study. Am.

show abstract

Spontaneous Remission of Multi-System Histiocytosis X

Cited by 142 publications

References 10 publications

Nonoperative management of solitary eosinophilic granulomas of the calvaria

Nonoperative management of solitary eosinophilic granulomas of the calvaria

Recurrent BRAF mutations in Langerhans cell histiocytosis

Treatment of children with Langerhans cell histiocytosis with 2‐chlorodeoxyadenosine

Contact Info

Product

Resources

About