Subcutaneous panniculitis-like T-cell lymphoma: Definition, classification, and prognostic factors: An EORTC Cutaneous Lymphoma Group Study of 83 cases

Department of Dermatology, Leiden University Medical Center, the Netherlands.
Blood (Impact Factor: 10.43). 01/2008; 111(2):838-45. DOI: 10.1182/blood-2007-04-087288
Source: PubMed

ABSTRACT In the WHO classification, subcutaneous panniculitis-like T-cell lymphoma (SPTL) is defined as a distinct type of T-cell lymphoma with an aggressive clinical behavior. Recent studies suggest that distinction should be made between SPTL with an alpha/beta T-cell phenotype (SPTL-AB) and SPTL with a gammadelta T-cell phenotype (SPTL-GD), but studies are limited. To better define their clinicopathologic features, immunophenotype, treatment, and survival, 63 SPTL-ABs and 20 SPTL-GDs were studied at a workshop of the EORTC Cutaneous Lymphoma Group. SPTL-ABs were generally confined to the subcutis, had a CD4-, CD8+, CD56-, betaF1+ phenotype, were uncommonly associated with a hemophagocytic syndrome (HPS; 17%), and had a favorable prognosis (5-year overall survival [OS]: 82%). SPTL-AB patients without HPS had a significantly better survival than patients with HPS (5-year OS: 91% vs 46%; P<.001). SPTL-GDs often showed (epi)dermal involvement and/or ulceration, a CD4-, CD8-, CD56+/-, betaF1- T-cell phenotype, and poor prognosis (5-year OS: 11%), irrespective of the presence of HPS or type of treatment. These results indicate that SPTL-AB and SPTL-GD are distinct entities, and justify that the term SPTL should further be used only for SPTL-AB. SPTL-ABs without associated HPS have an excellent prognosis, and multiagent chemotherapy as first choice of treatment should be questioned.

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Available from: Cesare Massone, Jul 22, 2015
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    • "Unlike the majority of SPTL (α/β) cases in the literature, complete spontaneous resolution of the lesion occurred in the present case with no further treatment. Spontaneous involution of this disease has been previously observed in only two of the 63 cases recently reviewed in the literature [4] "
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    ABSTRACT: Subcutaneous panniculitis-like T-cell lymphomas (SPTLs) α/β are rare in childhood. The present report refers to a case of a 7-year-old male child presenting an extensive skin lesion that began when he was 5 years of age. Two biopsies were evaluated using the CD3, CD4, CD8, CD56, βF1, and TIA markers. A dense infiltrate of CD3+, CD4-, CD8+, CD56-, βF1+, and TIA+ pleomorphic lymphocytes was found in the subcutis. The previous biopsy showed cytophagic histiocytic panniculitis with a small focus on CD8+ and βF1+ malignant cells. The lesion regressed spontaneously. This case shows that prognosis may be excellent in SPTL (α/β). On the other hand, it also serves as an alert that a biopsy performed in an area of cytophagic panniculitis may lead to misdiagnosis.
    11/2011; 2011:639240. DOI:10.1155/2011/639240
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    • "The presence of HPS, however, significantly decreases survival rates; the five-year survival rates for TCRαβ without HPS are around 90%, whereas if HPS is present the rate falls to less than 50%. On the other hand, there is rapid clinical deterioration in Cγδ-TCL whether HPS is present or not, with a 5-year survival rate of less than 20% in either group [3] [4]. Even without treatment, metastasis of TCRαβ SPTCL is very rare. "
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    ABSTRACT: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a very rare form of skin lymphoma that is localized primarily to the subcutaneous adipose tissue without palpable involvement of the lymph nodes. Diagnosis of SPTCL is a challenge, especially during its early phases when symptoms mimic other, more common conditions, such as benign panniculitis, eczema, dermatitis, psoriasis and cellulitis. Clinical and systemic features are nonspecific and can include fever, chills, and weight loss. Further complicating diagnosis is the high number of false negatives provided by biopsy. Here we present a case of SPTCL that illustrates the full course of the disease, from presentation and multiple misdiagnoses to correct disease recognition and successful treatment. A review of the challenges of diagnosis is provided with recommendations for more accurate and timely recognition of SPTCL.
    03/2011; 2011:824528. DOI:10.1155/2011/824528
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    • "However, the factors determining response to sole high-dose systemic corticosteroid therapy and the requirement of multiagent chemotherapy need to be defined in a larger prospective study. Autoimmune phenomena occur frequently among SPTL patients (Willemze et al., 2008). Three of our cases had an autoimmune disease, and two other cases presented with laboratory abnormalities suggestive of an autoimmune disorder, for example, antinuclear autoantibodies, a positive Coombs test, but the criteria for any specific autoimmune disease were not fulfilled. "
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    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.
    Journal of Investigative Dermatology 04/2008; 128(9):2304-9. DOI:10.1038/jid.2008.6 · 6.37 Impact Factor
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