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Bone marrow monosomy 7: Hematologic and clinical manifestations in childhood and adolescence
Author(s) -
Hutter John J.,
Hecht Frederick,
KaiserMccaw Barbara,
Hays Taru,
Baranko Paul,
Cohen Jesse,
Durie Brian
Publication year - 1984
Publication title -
hematological oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 44
eISSN - 1099-1069
pISSN - 0278-0232
DOI - 10.1002/hon.2900020103
Subject(s) - monosomy , bone marrow , pathology , medicine , myeloproliferative disorders , chromosome 7 (human) , leukemia , aneuploidy , chromosome abnormality , myeloid , hypoplasia , karyotype , immunology , biology , chromosome , genetics , gene
The hematologic manifestations and clinical course are described for six children and adolescents with bone marrow monosomy 7. One child with secondary acute myelogenous leukemia had monosomy 7 plus a marker chromosome; the remaining patients had marrow monosomy 7 as the only karyotypic abnormality. The hematologic abnormalities were diverse, but the majority of patients had a smoldering preleukemic or myeloproliferative phase. Leukemic blasts were either undifferentiated or demonstrated evidence of myeloid differentiation. All patients responded poorly to antileukemic therapy. Bone marrow monosomy 7 was observed in one patient with severe marrow hypoplasia. Antileukemic therapy in another patient with > 30 per cent marrow blasts was associated with the development of a bone marrow myeloproliferative disorder with persistence of the monosomy 7 karyotype. We speculate that monosomy 7 may be a specific marker for a pluripotent hematopoetic stem cell abnormality that is associated with either blastic leukemia or a myeloproliferative disorder.