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Association of large granular lymphocyte/natural killer cell proliferative disease and second hematologic malignancy
Author(s) -
Bassan Renato,
Rambaldi Alessandro,
Allavena Paola,
Abbate Mauro,
Marini Basilio,
Barbui Tiziano
Publication year - 1988
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/ajh.2830290206
Subject(s) - leukemia , lymphocytosis , immunology , natural killer cell , chronic myelogenous leukemia , cd3 , lymphocyte , k562 cells , biology , medicine , cancer research , immune system , cytotoxic t cell , cd8 , biochemistry , in vitro
We describe the first three patients with a large granular lymphocytosis/lymphocytic leukemia and another blood malignancy. In two, a myeloproliferative disorder developed soon after the diagnosis of abnormal proliferation of large granular lymphocytes‐natural killer (LGL‐NK) cells, a myelodysplastic syndrome evolving to acute leukemia and a Philadelphia‐positive chronic myelogenous leukemia. In these cases, LGLs expressed the phenotype of CD2 + NK and CD3 ‐ NK cells, respectively, and were clonal in the first patient as demonstrated by T‐cell receptor gene rearrangement study. In the third case, a similarly clonal excess of LGLs, phenotypically CD3 + NK cells, was detected following a diagnosis of B‐cell hairy‐cell leukemia. Clinically, the concurrence of LGL proliferation and other leukemia did not seem to confer a worse prognosis on the patients. Although an association by chance remains a possible explanation, a common origin from an altered precursor cell for both myeloid and LGL proliferations in the first two cases is discussed, whereas in the third it might be related to the severe immune derangement frequently observed in hairy‐cell leukemia.