title: Coexistence of lymphoblastic and monoblastic populations with identical mixed lineage leukemia gene rearrangements and shared immunoglobulin heavy chain rearrangements in leukemia developed in utero.
abstract: Congenital leukemia often provides insight into mechanisms of in utero leukemogenesis. A 10-day-old boy with clinical features of skin nodules, marked hepatosplenomegaly, and subcutaneous bleeding received a diagnosis of congenital leukemia. This patient initially had a dominant B progenitor lymphoblast population and minor monocyte component. Treatment with prednisolone, vincristine, and doxorubicin resulted in a loss of lymphoblast population and a rapid increase and dominance of the monocyte component within 10 days. Complete remission initially was obtained with additional combination chemotherapy with epipodophyllotoxin (VP-16) and cytosine arabinoside (Ara-C), but relapse characterized by a lymphoblastic population in the bone marrow was subsequently observed. The authors hypothesize that the leukemic cells originated from a common B-monocyte lineage stem cell during fetal hematopoiesis.
authors.affiliation: Department of Pediatrics, Toyama City Hospital, Hokubu-Machi, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Miura
authors.forename: A
authors.initials: A
authors.lastname: Yachie
authors.forename: I
authors.initials: I
authors.lastname: Hashimoto
authors.forename: T
authors.initials: T
authors.lastname: Okabe
authors.forename: N
authors.initials: N
authors.lastname: Murata
authors.forename: A
authors.initials: A
authors.lastname: Fukuda
authors.forename: S
authors.initials: S
authors.lastname: Koizumi
mesh_terms: D015321:Gene Rearrangement; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics*; D007223:Infant; D007231:Infant, Newborn; D015448:Leukemia, B-Cell / Q000151:congenital* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D007948:Leukemia, Monocytic, Acute / Q000151:congenital* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D008297:Male; D013234:Stem Cells / Q000473:pathology*
chemical_list: D007143:Immunoglobulin Heavy Chains