Skip to
  1. Homepage
  2. Rare diseases
  3. Search
Simple search

Simple search

*
(*) mandatory field





 

Other search option(s)

Subcutaneous panniculitis-like T-cell lymphoma

Orpha number ORPHA86884
Synonym(s) SPTCL
Subcutaneous panniculitic T-cell lymphoma
Prevalence Unknown
Inheritance
  • Sporadic
Age of onset Variable
ICD-10
  • C84.4
OMIM -
UMLS -
MeSH
  • C537503
MedDRA -
SNOMED CT
  • 103682005
  • 404133000

Summary

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic cutaneous lymphoma that has been recognized as a distinct subset of peripheral T-cell lymphomas originating and presenting primarily in the subcutaneous fat tissue.

The exact prevalence is unknown. A slight female preponderance has been reported

SPTCL is characterized by solitary or multiple erythematous subcutaneous nodules and plaques (proliferation of T lymphocytes and macrophages in the subcutaneous fat lobule), which appear in crops and are localized to the lower extremities or may be more generalized. Systemic manifestations include fever, chills, malaise, weight loss, hepatosplenomegaly, mucosal ulcers, and serosal effusions. SPTCL may be accompanied by hemophagocytic syndrome (characterized by the proliferation of histiocytes and phagocytosis of blood elements, hepatosplenomegaly, and coagulopathy and reported in approximately 30-40% of cases ; see these term), which may be fatal.

The etiology is not completely understood. A relationship between subcutaneous T-cell lymphoma and cytophagic histiocytic panniculitis has been raised and still under discussion.

Repeated biopsies may be required to establish the diagnosis.

The differential diagnosis includes other lymphomas as well as atypical lobular panniculitis and cytophagic histiocytic panniculitis (see these term).

SPTCL may be rapidly fatal or indolent. Most patients respond to systemic chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) or local radiation therapy.


(*) Required fields.

Attention: Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.


Captcha image

Detailed information

Review article
  • EN (2012,pdf)
Get Acrobat Reader
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.