In:
Blood, American Society of Hematology, Vol. 118, No. 21 ( 2011-11-18), p. 5217-5217
Abstract:
Abstract 5217 Objective: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare and distinct type of T-cell lymphoma. The objective of this study was to explore the clinical presentation, treatment, and prognosis of patients with SPTCL. Methods: Twelve cases of SCPTCL, treated in our hospital between June 2005 and June 2010, were included in this study. Their clinicopathological data were reviewed and analyzed retrospectively. Results: The median age at diagnosis was 41 years (range 25–69 years) and 7 (58%) were women. 9 cases had a CD3+, CD4-, CD8- phenotype; 2 cases, a CD3+, CD4-, CD8+ phenotype; and 1 case, a CD3+, CD4+, CD8- T-cell phenotype. In all cases, strong expression of cytotoxic proteins (granzyme B, TIA-1, perforin) was observed. CD56 was expressed in 8 of 12 cases. 4 patients had presented with solitary or localized skin lesions. Ulceration was observed in 3 patients. B symptoms, such as fever, chills, night sweats, and weight loss, had been recorded in 7 of 12 patients. Laboratory abnormalities, mainly anemia, leucopenia, thrombocytopenia or combined cytopenias, and elevated liver function tests and lactate dehydrogenase, were reported in 4 patients. Bone marrow examination showed histiocytic hyperplasia, hemophagocytosis, or decreased cellularity in 3 cases, but no evidence of lymphoma. A HPS was diagnosed in 4 of 12 patients (33%), and was fatal in 2 of them. Four patients presenting with solitary or localized skin lesions had been treated with radiotherapy (2 cases) or surgery (2 cases). All 4 patients reached complete remission and only 1 of them showed a skin relapse, which was treated successfully with radiotherapy again. Eight patients with diffused lesions were treated with chemotherapy (CHOP or CHOP-like courses). After initial treatment 5 (63%) of 8 patients had developed new skin lesions, and 2 of them had developed extracutaneous localizations. Then the 3 of the relapsed patients were treated with autologous stem cell transplantation, all of whom obtained complete remission. At the time of last follow-up (median follow-up: 34 months; range: 10–60 months), 5 patients are in complete remission, 4 patients have ongoing skin disease, while 3 patients have died, 2 of the complications of HPS or therapy-related side effects and 1 of unrelated disease. The 3-year OS and DSS of the patients were 75% and 42%, respectively. Patients without HPS had a significantly better 3-year OS (88%) than patients with HPS (50%; P 〈 .001). Conclusion: SPTCL seems to be a kind of heterogeneity disease. The factors associated with an unfavorable disease course were: diffuse lesions, a low white blood cell count, elevated lactate dehydrogenase, and combine with HPS. Autologous stem cell transplantation may improve the overall survival of high risk patients. Disclosures: No relevant conflicts of interest to declare.
Type of Medium:
Online Resource
ISSN:
0006-4971
,
1528-0020
DOI:
10.1182/blood.V118.21.5217.5217
Language:
English
Publisher:
American Society of Hematology
Publication Date:
2011
detail.hit.zdb_id:
1468538-3
detail.hit.zdb_id:
80069-7
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