Sinus histiocytosis with massive lymphadenopathy: complete response to low-dose interferon-alpha.

Palomera, Luis; Domingo, J. Moreno; Olave, T; Romero, Sandra Adolfina Reyes; Gutiérrez, María Inés

doi:10.1200/jco.1997.15.5.2176

Cited by 12 publications

(5 citation statements)

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“…There is also anecdotal evidence of clinical therapeutic benefits for interferon-alpha in other histiocytic disorders (Langerhans cell histiocytosis [22] and Rosai-Dorfaman disease [23]).…”

Section: Resultsmentioning

confidence: 99%

Successful treatment of Erdheim-Chester disease, a non-langerhans histiocytosis, with Interferon-alpha

Braiteh¹,

Boxrud²,

Esmaeli³

et al. 2005

JCO

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Erdheim-Chester disease is a rare non-Langerhans histiocytosis with multisystem involvement. To date, there is no standard treatment for this disorder, and over half of the patients succumb within three years. Because interferon-alpha promotes the terminal differentiation of histiocytes and dendritic cells, we hypothesized that this molecule would be a useful therapy for Erdheim-Chester disease. We therefore treated three patients with advanced disease with interferon-alpha at a starting dose of 3 to 6 x10 6 units, which was later reduced, during maintenance, to 1 x10 6 units s.c. three times per week. Marked improvement was noted in all patients, with substantial retro-orbital disease regression within one month. Improvement in bone lesions, pain, diabetes insipidus and other manifestations was gradual over many months. Responses were durable (3+ to 4.5+ years). Our observations suggest that this welltolerated therapy has a significant impact on the course and outcome of Erdheim-Chester disease.

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“…There is also anecdotal evidence of clinical therapeutic benefits for interferon-alpha in other histiocytic disorders (Langerhans cell histiocytosis [22] and Rosai-Dorfaman disease [23]).…”

Section: Resultsmentioning

confidence: 99%

Successful treatment of Erdheim-Chester disease, a non-langerhans histiocytosis, with Interferon-alpha

Braiteh¹,

Boxrud²,

Esmaeli³

et al. 2005

JCO

View full text Add to dashboard Cite

show abstract

“…The mechanism(s) underlying the salutary effects of interferon-␣ in Erdheim-Chester are unclear but could be due to several of the diverse biologic effects of this agent: maturation and activation of dendritic cells, 9,10 immune-mediated (eg, via natural killer cells) destruction of histiocytes, or direct antiproliferative effects. 21 There is also anecdotal evidence of clinical therapeutic benefits for interferon-␣ in other histiocytic disorders (Langerhanscell histiocytosis 22 and Rosai-Dorfaman disease 23 ).…”

Section: Resultsmentioning

confidence: 99%

Successful treatment of Erdheim-Chester disease, a non–Langerhans-cell histiocytosis, with interferon-α

Braiteh¹,

Boxrud

Esmaeli

et al. 2005

Blood

175

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Erdheim-Chester disease is a rare nonLangerhans histiocytosis with multisystem involvement. To date, there is no standard treatment for this disorder, and more than half of the patients succumb within 3 years. Because interferon-␣ promotes the terminal differentiation of histiocytes and dendritic cells, we hypothesized that this molecule would be a useful therapy for Erdheim-Chester disease. We therefore treated 3 patients with advanced disease with interferon-␣ at a starting dose of 3 to 6 ؋ 10 6 units, which was later reduced, during maintenance, to 1 ؋ 10 6 units subcutaneous 3 times per week. Marked improvement was noted in all patients, with substantial retro-orbital disease regression within 1 month. Improvement in bone lesions, pain, diabetes insipidus, and other manifestations was gradual over many months. Responses were durable (3؉ to 4.5؉ years). Our observations suggest that this well-tolerated therapy has a significant effect on the course and outcome of Erdheim-Chester disease. (Blood. 2005;106:2992-2994)

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“…Yet, it is difficult to say whether prednisolone cures RDD or merely provides symptomatic relief. There are also reports of interferon treatment with inconsistent results [7,24,25]. A few innovative treatment options need attention in the literature, like vitamin A derivatives, thalidomide and imatinib, that are reported to be effective in patients with cutaneous involvement [26][27][28][29] and rituximab for patients with lymphadenopathy [30].…”

Section: Discussionmentioning

confidence: 97%

Extranodal multifocal Rosai–Dorfman disease: response to 2-chlorodeoxyadenosine treatment

et al. 2008

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Rosai-Dorfman disease (RDD) or "sinus histiocytosis with massive lymphadenopathy" is a rare lymphoproliferative disorder of unknown etiology. The disease usually presents with painless lymphadenopathy with occasional extranodal involvement in various organs. We report a case of a 36-year-old man with a history of non-Hodgkin lymphoma (NHL), who recently presented with inguinal lymphadenopathy. Following the diagnosis of RDD on lymph node biopsy, he developed symptoms of spinal cord compression due to a mass lesion discovered at T6-7 vertebral level. 18F-Fluorodeoxyglucose (18FDG) positron emission tomography (PET-CT) revealed extensive disease with lung, renal and bone involvement. The patient received a short course of steroid therapy for cord compression findings and 2-chlorodeoxyadenosine (2-CdA) treatment was initiated for long-term disease control. He had a dramatic sustained response to treatment with six courses of 2-CdA. These results suggest that 2-CdA can be an effective treatment of choice and positron emission tomography with 18FDG can be used for determining the extent of disease and for follow-up in RDD.

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Sinus histiocytosis with massive lymphadenopathy: complete response to low-dose interferon-alpha.

Cited by 12 publications

References 0 publications

Successful treatment of Erdheim-Chester disease, a non-langerhans histiocytosis, with Interferon-alpha

Successful treatment of Erdheim-Chester disease, a non-langerhans histiocytosis, with Interferon-alpha

Successful treatment of Erdheim-Chester disease, a non–Langerhans-cell histiocytosis, with interferon-α

Extranodal multifocal Rosai–Dorfman disease: response to 2-chlorodeoxyadenosine treatment

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