Bexarotene is active against subcutaneous panniculitis-like T-cell lymphoma in adult and pediatric populations Journal Article


Authors: Mehta, N.; Wayne, A. S.; Kim, Y. H.; Hale, G. A.; Alvarado, C. S.; Myskowski, P.; Jaffe, E. S.; Busam, K. J.; Pulitzer, M.; Zwerner, J.; Horwitz, S.
Article Title: Bexarotene is active against subcutaneous panniculitis-like T-cell lymphoma in adult and pediatric populations
Abstract: Introduction: Subcutaneous panniculitis-like T-cell lymphoma (SPTL-AB) and cutaneous gamma/delta T-cell lymphoma (CGD-TCL) are rare T-cell lymphomas with varying clinical courses. There is no standard treatment, although chemotherapy and hematopoietic stem cell transplantation are commonly used. We describe results using bexarotene for children and adults with these disorders. Methods: We identified 15 patients (12 adults, 3 children) who were treated with bexarotene between 2000 and 2010 from the Memorial Sloan-Kettering Cancer Center lymphoma database, the Stanford Cancer Center Registry, and the National Cancer Institute (NCI) pediatric lymphoma database. There were 8 females and 7 males, with a median age of 45 years (range, 3 years to 85 years). All patients had stage IV disease. Two of 15 and 4 of 15 patients had documented CGD-TCL and SPTL-AB, respectively; others were presumed to have SPTL-AB. Bexarotene was administered at flat doses corresponding to 91 to 339 mg/m 2/d. Two of 15 patients received concurrent denileukin diftitox. Two children received bexarotene as maintenance therapy and were not evaluable for response. Results: Among those treated with bexarotene alone, the overall response rate (ORR) was 82% (6/11 complete response [CR], 3/11 partial response [PR]). One of the 2 patients treated with concomitant denileukin diftitox responded for an ORR of 10/13 (77%), including 54% CR and 23% PR. Median progression-free survival was 38.4 months; median duration of response was 26.3 months. Six patients developed hypothyroidism and 9 developed hyperlipidemia; one patient developed dose-limiting hypertriglyceridemia. One pediatric patient developed insulin-dependent diabetes mellitus. Conclusions: In this retrospective series, bexarotene showed a high response rate in SPTL-AB and CGD-TCL. It was generally well-tolerated with durable responses; therefore, bexarotene represents a promising therapy for children and adults with these disorders. © 2012 Elsevier Inc. All rights reserved.
Keywords: adolescent; adult; cancer survival; child; clinical article; preschool child; school child; treatment outcome; aged; aged, 80 and over; child, preschool; disease-free survival; middle aged; young adult; prednisone; doxorubicin; cancer patient; cancer staging; neoplasm staging; progression free survival; skin neoplasms; maintenance therapy; cyclophosphamide; vincristine; data base; cancer center; cutaneous t cell lymphoma; anticarcinogenic agents; drug response; panniculitis; cancer registry; hypothyroidism; insulin dependent diabetes mellitus; diabetes mellitus, type 1; hyperlipidemia; hypertriglyceridemia; hyperlipidemias; subcutaneous panniculitis-like t-cell lymphoma; denileukin diftitox; bexarotene; lymphoma, t-cell, cutaneous; tetrahydronaphthalenes; cutaneous gamma/delta t-cell lymphoma
Journal Title: Clinical Lymphoma, Myeloma and Leukemia
Volume: 12
Issue: 1
ISSN: 2152-2650
Publisher: Elsevier Inc.  
Date Published: 2012-02-01
Start Page: 20
End Page: 25
Language: English
DOI: 10.1016/j.clml.2011.06.016
PROVIDER: scopus
PUBMED: 22001256
PMCID: PMC3938280
DOI/URL:
Notes: --- - "Export Date: 2 April 2012" - "Source: Scopus"
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors
  1. Melissa P Pulitzer
    203 Pulitzer
  2. Steven M Horwitz
    645 Horwitz
  3. Klaus J Busam
    688 Busam
  4. Patricia Myskowski
    216 Myskowski