Acute mixed lineage leukemia in children: the experience of St Jude Children's Research Hospital.
To characterize the biology and optimal therapy of acute mixed-lineage leukemia in children, we reviewed the pathologic and clinical features, including response to therapy, of 35 patients with mixed-lineage leukemia. The majority of cases (91%) had blasts cells that simultaneously expressed either T-lineage plus myeloid markers (T/myeloid, n = 20) or B-lineage plus myeloid markers (B/myeloid, n = 12). Overall survival rates for the B/myeloid and T/myeloid subgroups were not significantly different from each other or from the rate for acute myeloid leukemia (AML) but were inferior to the outcome in children with acute lymphoblastic leukemia (ALL). Patients who failed to achieve complete remission with AML-directed therapy could often be induced with a regimen of prednisone, vincristine, and L-asparaginase. Analysis of gene-expression patterns identified a subset of biphenotypic leukemias that did not cluster with T-cell ALL, B-progenitor ALL, or AML. We propose that treatment for biphenotypic leukemia begin with one course of AML-type induction therapy, with a provision for a switch to lymphoid-type induction therapy with a glucocorticoid, vincristine, and L-asparaginase if the patient responds poorly. We also suggest that hematopoietic stem cell transplantation is often not required for cure of these patients.
Rubnitz
Jeffrey E
JE
Department of Oncology, St Jude Children's Research Hospital, Memphis, TN 38105-2794, USA. jeffrey.rubnitz@stjude.org
Onciu
Mihaela
M
Pounds
Stanley
S
Shurtleff
Sheila
S
Cao
Xueyuan
X
Raimondi
Susana C
SC
Behm
Frederick G
FG
Campana
Dario
D
Razzouk
Bassem I
BI
Ribeiro
Raul C
RC
Downing
James R
JR
Pui
Ching-Hon
CH
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000473:pathology; D002648:Child; D020869:Gene Expression Profiling; D006801:Humans; D016130:Immunophenotyping; D015456:Leukemia, Biphenotypic, Acute / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D015470:Leukemia, Myeloid, Acute; D022423:Myeloid Cells / Q000473:pathology; D017410:Practice Guidelines as Topic; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy; D012189:Retrospective Studies; D015996:Survival Rate; D013601:T-Lymphocytes / Q000473:pathology; D016896:Treatment Outcome