Peripheral T-cell lymphoma in childhood and adolescence a clinicopathologic study of 22 patients

Abstract

Background. Peripheral T‐cell lymphoma (PTCL), although the most common T‐cell lymphoma in adults, is relatively rare in childhood, and only small series have been reported. Methods/Results. Twenty‐two cases of PTCL were studied that occurred in patients 18 months to 20 years of age. Nine were seen when the condition was diagnosed, and the other 13 were referred after they had relapses. The stage at diagnosis was I or II (45%), III (41%), and IV (14%). Patients with Stage IV disease were younger than those with Stage I or II disease (2.5 versus 14.8 years, P = 0.04). Twelve patients had extranodal disease when the diagnosis was made; the skin was the most common site. Ten tumors were classified as diffuse large cell type; five, as diffuse anaplastic large cell type; and seven, as diffuse mixed cell type. Twenty of the 21 tumors tested were CD30 (Ki‐1 or Ber‐H2) antigen positive. Of the nine patients seen when the diagnosis was made and treated by the authors, three had a relapse (median, 12 months), a 2‐year relapse‐free survival (RFS) rate of 61%. For the total group, the RFS was longer for patients older than 12 years of age compared with those who were younger (20 versus 12 months, P = 0.05). Overall, six patients remained in their first complete remission. Sixteen patients had a relapse, and 13 of these underwent bone marrow transplantation (BMT). Six of these remained in complete remission (median, 18 months after BMT). Overall, only 6 of 22 patients died (median survival, > 60 months). Conclusion. It was concluded that aggressive therapy, including BMT for relapses, can provide prolonged disease control in most children with PTCL. Cancer 1993; 71:257‐63.

Morphological and Phenotypic Features in Pediatric Large Cell Lymphoma and Their Correlation with ALK Expression and the t(2;5)(p23;q35) Translocation

“…How the resulting NPM-ALK chimeric protein is related to tumorigenesis has yet to be determined. Although early reports suggested that the t(2;5) translocation might be unique to a morphologic entity of ALCL [27][28][29], more recently it has been shown that the translocation is not limited to cases with typical anaplastic morphology, and is in fact present in lympho-mas with a wide morphologic spectrum, including monomorphic, lymphohistiocytic, small cell-predominant, neutrophil-rich, sarcomatoid, and Hodgkins-like subtypes [8,13,15,16,24,25]. In our study the majority of tumors (two-thirds) were of T-cell or null-cell immunophenotype.…”

“…Our results concur with recent suggestions that primary pediatric LCL with ALK protein expression and/or the t(2;5) translocation appears to represent a distinct pathologic entity occurring in young patients with co-expression of T-cell markers, EMA, and CD30. Recent investigators have emphasized the difficulty of using subjective morphological criteria such as anaplasia to define lymphoma subtypes [24], and it has become apparent that tumors in the ALCL category can have a varied morphologic appearance [8,13,15,16,24,25,38]. Furthermore, some authors have chosen to exclude B-cell tumors from the ALCL category [18,23,26], whereas others have not [22].…”

“…Few studies of large cell lymphomas in an exclusively pediatric population have correlated CD30 expression with morphology or the t(2;5) translocation [12,13,16]. Furthermore, few studies have attempted to correlate these parameters with expression of the NPM-ALK fusion gene created by the t(2;5) translocation.…”

Leukaemic presentation of small cell variant anaplastic large cell lymphoma: report of four cases

“…Hyperleucocytosis with circulating abnormal small lymphoid cells has occasionally been reported in peripheral T‐cell lymphoma (Weisenburger et al , 1985). However, we suspect that in the study by Gordon et al (1993) on peripheral T‐cell lymphoma in childhood and adolescence, the three patients with leucocytosis (two of whom showed circulating lymphoma cells) were in fact affected by the small cell variant of ALCL. Indeed, until recently, some cases of small cell variant of ALCL were misdiagnosed as peripheral T‐cell lymphoma.…”

Peripheral T‐cell lymphoma in a five year old

“…Skin involvement in T/NK‐cell lymphoma is frequent. Peripheral T/NK‐cell lymphomas (PTCL) are characterised by a mixture of small and large atypical lymphoid cells and express CD3 and either CD4 or CD8 13 but do not express CD30 or ALK‐1 that characterise anaplastic large cell lymphoma. A wide variety of histological subtypes have been recognised, the most common being PTCL not otherwise specified (NOS) 14.…”