A SMALL-CELL-PREDOMINANT VARIANT OF PRIMARY KI-1 (CD-30)+ T-CELL LYMPHOMA

被引:193
作者
KINNEY, MC
COLLINS, RD
GREER, JP
WHITLOCK, JA
SIOUTOS, N
KADIN, ME
机构
[1] VANDERBILT UNIV, MED CTR, DEPT PEDIAT, DIV HEMATOL & ONCOL, NASHVILLE, TN 37232 USA
[2] BETH ISRAEL HOSP, DEPT PATHOL, BOSTON, MA 02215 USA
[3] HARVARD UNIV, MED CTR, BOSTON, MA 02115 USA
关键词
KI-1; CD30; ANAPLASTIC LARGE-CELL LYMPHOMA; T-CELL LYMPHOMA;
D O I
10.1097/00000478-199309000-00001
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
We describe nine patients with a primary Ki-1 (CD30)+ T-cell lymphoma containing numerous, often CD30-negative, small lymphocytes with irregular nuclei and a minor population of large CD30+ tumor cells. All previously described primary Ki-1+ lymphomas have been large-cell neoplasms. In this small-cell variant, the diagnosis of lymphoma was difficult to make because there was a predominance of small lymphocytes and, in some cases, clinical features suggested an inflammatory process. Patients were young (age range 0.3-40 years, median 14 years), and frequently had B symptoms (56%); sites of involvement were predominantly skin (78%) and lymph node (67%). The actuarial 2-year disease-free survival was 14%, and the overall survival was 51%. Two patients had a rapidly fatal course. In all cases histologic sections showed a predominance of small lymphocytes with marked nuclear irregularity and often a perivascular/intravascular distribution of CD30+ large cells. All cases had a T-cell phenotype. In four cases the large and small cells could be compared and had a similar aberrant T-cell phenotype. Large cells were CD30+, but only rare small cells expressed CD30. Cytogenetic studies revealed a t(2;5)(p23;q35) in four of four cases studied. Four patients had numerous large cells on repeat biopsies; two of these developed sheets of large CD30+ cells typical of anaplastic large-cell lymphoma (ALCL). These cases provide further evidence that primary Ki-1+ lymphoma has a morphologic spectrum that includes a small-cell variant. Although very different morphologically from previously described Ki-1+ ALCL, this small-cell variant is clearly part of the disease spectrum on the basis of clinical features, the presence of the t(2;5)(p23;q35), the aberrant T-cell phenotype in the small and large cells, as well as histologic progression seen in several patients.
引用
收藏
页码:859 / 868
页数:10
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