Article

Clinicopathological characterization and genomic aberrations in subcutaneous panniculitis-like T-cell lymphoma.

Department of Dermatology and Allergology, Helsinki University Central Hospital, University of Helsinki, Helsinki, Finland.
Journal of Investigative Dermatology (Impact Factor: 6.19). 04/2008; 128(9):2304-9. DOI: 10.1038/jid.2008.6
Source: PubMed

ABSTRACT Subcutaneous panniculitis-like T-cell lymphomas (SPTLs) represent a rare, difficult-to-diagnose, and poorly characterized subtype of cutaneous T-cell lymphomas (CTCLs) affecting younger people more than the other CTCL forms. We performed a thorough clinical, immunohistological, and molecular analysis of nine Finnish SPTL patients. Specifically, we performed single-cell comparative genomic hybridization (CGH) from laser microdissected, morphologically malignant SPTL cells, as well as loss of heterozygosity (LOH) and fluorescence in situ hybridization (FISH) analysis for the NAV3 (neuron navigator 3) gene. CGH revealed large numbers of DNA copy number changes, the most common of which were losses of chromosomes 1pter, 2pter, 10qter, 11qter, 12qter, 16, 19, 20, and 22 and gains of chromosomes 2q and 4q. Some of the DNA copy number aberrations in SPTL, such as loss of 10q, 17p, and chromosome 19, overlap with those characteristic of common forms of CTCL (mycosis fungoides (MF) and Sezary syndrome (SS)), whereas 5q and 13q gains characterize SPTL. Allelic NAV3 aberrations (LOH or deletion by FISH), previously found in MF and SS, were identified in 44% of the SPTL samples. This study demonstrates that SPTL is also moleculocytogenetically a uniform entity of CTCL and supports the current World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification defining SPTL as a subgroup of its own.

0 Bookmarks
 · 
99 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: A variety of lymphoma entities can involve the subcutaneous tissue. The term subcutaneous panniculitis-like T cell lymphoma is now solely utilized for primary cutaneous CD8+ lymphomas expressing the alphabeta T cell receptor heterodimer. This condition is generally responsive to treatment; however, the development of the hemophagocytic syndrome is a poor prognostic indicator. Overlapping features with lupus panniculitis has been observed, and cases with ambiguous pathology may be classified as atypical lobular lymphocytic panniculitis. These ambiguous cases often respond to systemic steroids or methotrexate. Overall, this condition follows an indolent course; however, evolution into frank lymphomas in some cases reflects the diagnostic difficulties of these conditions. Gamma-delta lymphomas have a poor prognosis regardless of the presence or absence of a hemophagocytic syndrome. Treatment options are limited because of lack of large studies and the rarity of this condition. Prolonged remission may be achieved with allogeneic stem cell transplantation.
    Dermatologic Therapy 01/2010; 23(4):350-5. · 1.96 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Hematodermic neoplasm (HN) is a clinically aggressive neoplasm with a high incidence of cutaneous involvement and a risk of leukemic dissemination. In the recent WHO-EORTC classification, the term blastic natural killer cell lymphoma has been replaced with CD4+/CD56+ HN because of its derivation from a plasmacytoid dendritic cell precursor. Cases of HN that completely lack CD4 or CD56 expression, therefore represents a diagnostic problem. A 68-year-old Korean male was diagnosed with CD4-/CD56+ HN and treated with hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) at initial treatment, and then switched to high dose methotrexate/cytarabine. His disease relapsed and resulted in death from bone and brain disease 6 months after complete clinical remission, despite diagnostic workups, including a radioisotope liver scan and ultrasound-guided fine needle aspiration biopsy. Further cytogenetic studies such as comparative genomic hybridization could elucidate the genetic mechanisms in the development and progression of lymphomas. We report an unusual case of 'CD4-/CD56+/CD123+ HN' showing early liver metastasis.
    Annals of Dermatology 05/2010; 22(2):186-90. · 0.61 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: This review focuses on the evolution and conceptual aspects of classifications for cutaneous lymphomas. The World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification and the WHO classification (4th edn, 2008) represent the first widely accepted classifications for lymphomas, in which the complete spectrum of primary cutaneous lymphomas is included. These classifications for primary cutaneous lymphomas define disease entities with distinct clinical, histological, immunophenotypic and genetic features. Final diagnosis is based on a synoptic integration of these features and implies clinicopathological correlation as a pivotal element of the diagnostic approach for primary cutaneous lymphomas. The entities, their definitions and diagnostic criteria of cutaneous lymphomas listed in the WHO/EORTC and WHO classifications are presented. Recent changes in the terminology and staging, practical implications and future perspectives are discussed.
    Histopathology 01/2010; 56(1):57-70. · 2.86 Impact Factor

Full-text (2 Sources)

View
2 Downloads
Available from
Jul 9, 2014