Burkitt leukemia with precursor B-cell features that developed after                    ruxolitinib treatment in a patient with hydroxyurea-refractory                        JAK2V617F-myeloproliferative                    neoplasm

Katsuhiro Fukutsuka; Futoshi Iioka; Fumiyo Maekawa; Miho Nakagawa; Chiyuki Kishimori; Masahiko Hayashida; Shunsuke Tagawa; Takashi Akasaka; Gen Honjo; Hitoshi Ohno

doi:10.3960/jslrt.21001

Case report

Burkitt leukemia with precursor B-cell features that developed after ruxolitinib treatment in a patient with hydroxyurea-refractory JAK2^V617F-myeloproliferative neoplasm

Katsuhiro Fukutsuka, Futoshi Iioka, Fumiyo Maekawa, Miho Nakagawa, Chiyuki Kishimori, Masahiko Hayashida, Shunsuke Tagawa, Takashi Akasaka, Gen Honjo, Hitoshi Ohno

Author information

Katsuhiro Fukutsuka
Tenri Institute of Medical Research, Nara, Japan,
Futoshi Iioka
Department of Hematology, Tenri Hospital, Nara, Japan,
Fumiyo Maekawa
Tenri Institute of Medical Research, Nara, Japan,
Miho Nakagawa
Tenri Institute of Medical Research, Nara, Japan,
Chiyuki Kishimori
Tenri Institute of Medical Research, Nara, Japan,
Masahiko Hayashida
Tenri Institute of Medical Research, Nara, Japan,
Shunsuke Tagawa
Department of Hematology, Tenri Hospital, Nara, Japan,
Takashi Akasaka
Department of Hematology, Tenri Hospital, Nara, Japan,
Gen Honjo
Department of Diagnostic Surgical Pathology, Tenri Hospital, Nara, Japan
Hitoshi Ohno Corresponding author
Tenri Institute of Medical Research, Nara, Japan,
Department of Hematology, Tenri Hospital, Nara, Japan,

Keywords: myeloproliferative neoplasm, JAK2^V617F mutation, ruxolitinib, precursor B-cell Burkitt leukemia, t(8;14)(q24;q32)/MYC-IGH

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Supplementary material

2021 Volume 61 Issue 2 Pages 114-119

DOI https://doi.org/10.3960/jslrt.21001

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Abstract

A 62-year-old woman, who had a 16-year history of JAK2^V617F-mutated myeloproliferative neoplasm (MPN), developed Burkitt leukemia (BL) 16 months after treatment with ruxolitinib to control hydroxyurea-refractory conditions. BL cells were CD10⁺, CD19⁺, CD20⁻, CD34⁻, cytoplasmic CD79a⁺, and TdT⁺, and lacked surface immunoglobulins but expressed the cytoplasmic μ heavy chain. In the bone marrow, nuclear MYC⁺ BL cells displaced the MPN tissues. t(8;14)(q24;q32) occurred at a CG dinucleotide within MYC exon 1 and at the IGHJ3 segment, and an N-like segment was inserted at the junction. The V-D-J sequence of the non-translocated IGH allele had the unmutated configuration. DNA from peripheral blood at a time of the course of MPN exhibited homozygous JAK2^V617F mutation, while that at BL development included both JAK2^V617F and wild-type DNAs. Although the association between JAK1/2 inhibitor therapy for MPN and secondary development of aggressive B-cell neoplasm remains controversial, this report suggests that, in selected patients, close monitoring of clonal B-cells in the BM is required before and during treatment with JAK1/2 inhibitors.

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