THERAPEUTIC HOTLINE: Primary cutaneous CD4 + small/medium-sized pleomorphic T cell lymphoma coexisting with myelodysplastic syndrome transforming into chronic myelomonocytic leukemia successfully treated with cyclophosphamide
ABSTRACT Cutaneous T cell lymphomas other than mycosis fungoides, Sezary syndrome, and primary cutaneous CD30+ lymphoproliferations constitute less than 10% of all cutaneous T cell lymphomas. Primary cutaneous small/medium CD4+ T cell lymphoma is a member of this third group of cutaneous lymphomas, separated out as provisional entity in the World Health Organization classification – European Organization for Research and Treatment of Cancer (WHO-EORTC) classification. It still awaits development of more precise diagnostic criteria and optimal therapy.
We report a case of primary cutaneous CD4 + small/medium-sized pleomorphic T cell lymphoma accompanied with myelodysplastic syndrome successfully treated with cyclophosphamide.
It seems that cyclophosphamide as a single-agent chemotherapy in patients with disseminated lesions might be safe and quite effective therapeutic option.
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ABSTRACT: CD4+ small/medium-sized pleomorphic T-cell lymphoma (SMPTCL) is a controversial primary cutaneous lymphoma, in which the candidate neoplastic cells express a follicular T-helper phenotype. We describe 16 cases of SMPTCL and compare expression of PD-1, CXCL-13 and ICOS in these tumors with 40 dermatitis cases. Histopathologic examination and immunocytochemistry were performed for 16 tumors and 40 assorted dermatitis cases. All but one patient presented with solitary lesions. Each biopsy revealed a dense nodular non-epitheliotropic infiltrate of atypical T-cells. Neoplastic cells were CD3+/CD4+/CD8(-)/CD30(-). Cutaneous recurrence occurred in one patient over a median follow up of 8 months (range 5-36). All tumors widely expressed PD-1 and ICOS to a lesser extent. CXCL-13 stained much fewer cells. Of the dermatitis cases, PD-1 (most numerous) and ICOS labeled lymphoid cells in all cases, albeit fewer than in the tumors, and CXCL-13 was negative in 32. A rosette pattern of PD-1 expression was identified in all the SMPTCL cases but not in dermatitis. There remains uncertainty about the appropriate nosological status of SMPTCL, which some authors consider to be a pseudolymphoma. However, this study suggests a significant difference in the prevalence and pattern of follicular T-helper cell markers between this tumor and lymphoid proliferations known to be reactive.Journal of Cutaneous Pathology 12/2013; 40(12):1006-13. DOI:10.1111/cup.12234 · 1.58 Impact Factor
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ABSTRACT: Abstract CD4+ primary cutaneous small/medium-sized pleomorphic T-cell lymphoma (CD4+ PCSM-TCL) is a rare T-cell lymphoma associated with a favorable prognosis. A retrospective study of 23 patients with CD4+ PCSM-TCL as defined by WHO-EORTC and WHO classifications was conducted. The median age was 63 years. The head and neck were the most commonly affected locations followed by the trunk. Two patients had evidence of systemic involvement at relapse. All tumors were CD3+ and CD4+; CD5 and CD7 loss occurred in 52% and 84% respectively. The median follow-up was 33.6 months. Eleven patients had excisional biopsy only, six had localized radiotherapy, and two received excision and localized radiation. Cytotoxic chemotherapy and localized radiation were used in one patient with aggressive and invasive features. All patients had a complete remission but one developed systemic involvement. Our case series demonstrates that CD4+ PCSM-TCL is an indolent T-cell lymphoma that can be treated with local modalities and raises the question of its current classification as a lymphoma. Introduction.Leukemia and Lymphoma 07/2014; 56(4):1-12. DOI:10.3109/10428194.2014.938331 · 2.89 Impact Factor
- American Journal of Hematology 10/2014; 89(10). DOI:10.1002/ajh.23783 · 3.80 Impact Factor