Refractory double-hit lymphoma/leukemia in childhood mimicking B-precursor acute lymphoblastic leukemia at initial presentation.
A 10-year-old girl was referred to our hospital with left preauricular adenopathy and gingival swelling. She was diagnosed with B-cell precursor acute lymphoblastic leukemia (BCP-ALL) based on being positive for expressions of CD10, CD19, TdT and HLA-DR. She showed no CD20 expression at the time of diagnosis. Based on the initial diagnosis of BCP-ALL, induction chemotherapy for BCP-ALL was initiated. However, the blasts did not disappear from her peripheral blood. Bone marrow examination on day 33 identified 81.3% residual blasts with positive expressions of CD19, 20 and HLA-DR and negative CD10 and TdT expressions; these cells were morphologically and phenotypically different from those at the initial diagnosis. Based on cytogenetic studies, the final diagnosis was double-hit lymphoma/leukemia (DHL) with IgH-BCL2 and Igλ-MYC. Although dose intensive chemotherapy, including rituximab, led to complete remission, bone marrow and central nervous system relapse occurred. At relapse, blasts expressed CD10, CD19 and HLA-DR, but not CD20, findings the same as those at the onset. The patient died of the disease 44 days after cord blood transplantation with non-remission status. DHL in childhood is extremely rare and its prognosis is poor. The establishment of an effective treatment for DHL is highly anticipated.
Department of Hematology and Oncology, Children's Cancer Center, Kobe Children's Hospital.
Suguru
S
Uemura
Daiichiro
D
Hasegawa
Takehito
T
Yokoi
Nanako
N
Nino
Teppei
T
Tahara
Akihiro
A
Tamura
Atsuro
A
Saito
Aiko
A
Kozaki
Kenji
K
Kishimoto
Toshiaki
T
Ishida
Keiichiro
K
Kawasaki
Nobuyuki
N
Yamamoto
Takeshi
T
Mori
Noriyuki
N
Nishimura
Yoshiyuki
Y
Kosaka
D001853:Bone Marrow / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D016393:Lymphoma, B-Cell / Q000276:immunology / Q000473:pathology*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*; D000069283:Rituximab / Q000627:therapeutic use*; D016896:Treatment Outcome
D000069283:Rituximab