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Rare adult acute lymphocytic leukemia with CD56 expression in the ECOG experience shows unexpected phenotypic and genotypic heterogeneity
Author(s) -
Paietta Elisabeth,
Neuberg Donna,
Richards Susan,
Bennett John M.,
Han Lei,
Racevskis Janis,
Dewald Gordon,
Rowe Jacob M.,
Wiernik Peter H.
Publication year - 2001
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/1096-8652(200103)66:3<189::aid-ajh1043>3.0.co;2-a
Subject(s) - cd5 , gene rearrangement , cd19 , cd20 , biology , immunophenotyping , t cell receptor , acute lymphocytic leukemia , immunology , leukemia , antigen , lineage (genetic) , t cell , gene , genetics , immune system , lymphoblastic leukemia
Abstract Expression of CD56, a marker of natural killer (NK) cells, in acute lymphocytic leukemia (ALL) is rare and, to date, has been described only in non‐B lineage ALL. Among 194 patients with CD56 analysis on the ongoing Eastern Cooperative Oncology Group (ECOG) ALL trial, E2993, 6 cases of CD56 + ALL were found (3.1%) with a median of 95% of blast cells expressing CD56, compared with a median of 1% of blast cells in CD56 − ALL ( P = 0.0001). FAB‐L2 characteristics dominated, without granulation. Blast cells from four CD56 + patients expressed T‐cell antigens at variable levels of maturation. A clonal rearrangement of the T‐cell receptor β (TCRβ) gene was detected only in one patient. TCRβ variable gene usage studies in this and one other CD56 + ALL patient demonstrated a significantly perturbed usage pattern in both patients when compared with control lymphocytes. The two remaining cases typed as early pre‐B ALL (CD19 + , CD10 + ), with one case co‐expressing CD7. Cytogenetically, 4 patients were normal, 1 complex abnormal, and 1 Philadelphia chromosome positive. Epstein‐Barr virus (EBV) sequences were detected in one T‐ and both B‐lymphoid cases. Our data suggest that CD56 is expressed at a precursor stage common to the T‐ and the B‐cell lineage. Am. J. Hematol. 66:189–196, 2001. © 2001 Wiley‐Liss, Inc.