In:
Tumori Journal, SAGE Publications, Vol. 99, No. 5 ( 2013-09), p. e251-e256
Abstract:
In rare disorders, there are often no standard therapy recommendations. Patients with refractory disease may require novel experimental approaches. Applied as second- up to fourth-line treatment, lenalidomide (10–25 mg perorally on days 1–21 in a 28-day cycle) was used in our cohort of four adult patients with aggressive, multisystem and relapsing diseases. Complete and long-lasting remissions (more than 1 year, no maintenance therapy) were achieved in patients with Langerhans cell histiocytosis (11 cycles, combination with dexamethasone and etoposide, consolidated by allogeneic blood stem cell transplant) and plasma-cell Castleman disease (15 cycles, monotherapy). Mixed response with complete disappearance of brain infiltrates was reached in Erdheim-Chester disease (6 cycles, monotherapy) and gastrointestinal bleeding was well controlled in multiple angiomatosis (9 cycles, combination with thalidomide). For disease activity evaluation each patient underwent fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography scan imaging, which was complemented by clinical and laboratory investigations.
Type of Medium:
Online Resource
ISSN:
0300-8916
,
2038-2529
DOI:
10.1177/030089161309900524
Language:
English
Publisher:
SAGE Publications
Publication Date:
2013
detail.hit.zdb_id:
280962-X
detail.hit.zdb_id:
2267832-3
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