Primary cutaneous T‐cell lymphoma: Clinicopathological features and prognostic parameters of 35 cases other than mycosis fungoides and cd30‐positive large cell lymphoma

Abstract

Within the group of primary cutaneous T-cell lymphomas (CTCLs), mycosis fungoides (MF), Sézary's syndrome (SS), and CD30-positive lymphomas have been delineated as clinicopathological entities. Primary CTCLs that do not belong to one of these entities represent a heterogeneous and ill-defined group of neoplasms. This paper describes the clinical and histological features of 35 of such cases. The object of this study was to define prognostic parameters for this group of primary CTCLs. Using a slightly modified version of the updated Kiel classification, a subdivision was made into CTCL, pleomorphic, small cell type (n = 3); pleomorphic, medium-sized cell type (n = 6); pleomorphic, large cell type (n = 18); and immunoblastic lymphomas (n = 8). Altogether, these lymphomas had a poor prognosis with estimated 2- and 4-year survival rates of 53 and 22 percent, respectively. Patients with pleomorphic, small and medium-sized cell lymphomas (n = 9) proved to have a significantly better survival than those with pleomorphic, large cell lymphomas (P = 0.032) and immunoblastic lymphomas (P = 0.008). Primary cutaneous immunoblastic lymphomas had the worst prognosis with an estimated 2-year survival rate of 14 percent. Other parameters including age (P = 0.345), sex (P = 0.662), extent of skin lesions at presentation (P = 0.0854), and mode of initial treatment (P = 0.609) had no significant effect on the survival time. The results of this study suggest that primary CTCLs other than classical MF, SS, and CD30-positive lymphomas have a poor prognosis in most cases, and that the current classification may be a useful means of predicting the clinical behaviour in these lymphomas.

Primary cutaneous large-cell lymphoma: analysis of 49 patients included in the LNH87 prospective trial of polychemotherapy for high-grade lymphomas

“…B-large cell lymphomas are mostly of follicle center cell origin and T-large cell lymphomas are classified according to the CD30 antigen expression which identify anaplastic large-cell lymphomas having a good prognosis. [3][4][5] Furthermore, these PCLL may have clinical behaviors and prognoses different from those of primary node-based malignant lymphomas of the same histologic subtype. 3,6 Treatment has not yet been well-defined, comprising radiotherapy, polychemotherapy or both, 7,8 and no prospective studies have been performed in this rare and heterogeneous group of patients.…”

“…After treatment, survival rates ranged from 30 to 80% and the worst prognosis is observed in CD30 − Tlarge cell lymphomas. 5,7 For these reasons, PCLL patients included in the multicentric prospective trial of polychemotherapy (LNH87) for the treatment of aggressive lymphomas were selected and their therapeutic results, histologic features and prognostic factors for survival were analyzed. These patients were then compared to those presenting with noncutaneous lymphomas included in the same protocol and fully matched for the main clinical characteristics (age, LDH level, ECOG scale, non cutaneous extranodal site).…”

Recurrence with histological transformation 40 days after autologous peripheral blood stem cell transplantation (APBSCT) for cutaneous CD30-negative large T cell lymphoma

“…Keywords: CD30-negative cutaneous large T cell lymphoma; autologous peripheral blood stem cell transplantation (APBSCT); histological transformation Cutaneous T cell lymphoma (CTCL) other than mycosis fungoides (MF) represents a rare and heterogeneous group of lymphomas; the clinical behavior has seldom been described. [1][2][3][4] In 1997, the European Organization for Research and Treatment of Cancer (EORTC) reported a new classification for primary cutaneous lymphoma. 5 In their report, CTCL were subdivided into two categories: indolent and aggressive forms according to clinical behavior.…”

“…[1][2][3][4] In 1997, the European Organization for Research and Treatment of Cancer (EORTC) reported a new classification for primary cutaneous lymphoma. 5 In their report, CTCL were subdivided into two categories: indolent and aggressive forms according to clinical behavior.…”

Cutaneous T cell lymphomas: mycosis fungoides, Sezary syndrome and HTLV-I-associated adult T cell leukemia (ATL) in Mali, West Africa: a clinical, pathological and immunovirological study of 14 cases and a review of the African ATL cases

“…The Dutch Cutaneous Lymphoma Working Group recently published a specific classification for cutaneous T cell lymphomas (CTCL), based on both clinical and histopathological criteria. [4][5][6][7][8][9] Such classification has been recently proposed by the European Organization for Research and Treatment of Cancer (EORTC). 10 CTCL have been frequently suspected to be of viral origin.…”