CD34選択同種末梢血幹細胞移植を施行後 thrombotic microangiopathy や human herpes virus 6 感染症など多彩な合併症を呈した乳児T細胞性急性リンパ性白血病の1例  [in Japanese] Infantile T Cell Acute Lymphoblastic Leukemia Complicated by Thrombotic Microangiopathy and Human Herpes Virus 6 Infection after Allogeneic Peripheral Blood Stem Cell Transplantation Using CD34-positive Cells  [in Japanese]

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化学療法に対して抵抗性となったT細胞性急性リンパ性白血病の1歳7カ月男児に対しHLA2座不一致の母親をdonorとしてCD34選択同種末梢血幹細胞移植を施行した。移植前処置はBU 140 mg/m<sup>2</sup>/day×2, TBI 12 Gy, L-PAM 210 mg/m<sup>2</sup>でGVHD予防はCyAにて行った。幹細胞の純化はBaxter社のIsolex 50 Immunomagnetic Cell Separation Systemを用いて行ない,処理後4.4×10<sup>6</sup>/kgのCD34陽性細胞を移植した。移植後14日目に骨髄細胞の性染色体FISH法を用いた解析により生着を確認した。GVHDに引き続いてTMAによる難治性の下痢を合併し,移植後71日目にHHV-6による間質性肺炎のため死亡した。CD34陽性細胞移植はHLA不一致血縁者から重篤なGVHDを回避して生着を期待しうる有望な治療であるが,重篤なウイルス感染症の増加など克服すべき問題が多く残されていると考えられた。

We report on a 19-month-old boy with refractory T-cell acute lymphoblastic leukemia who underwent allogeneic peripheral blood stem cell transplantation using positively selected CD34 cells from his HLA two-loci mismatched mother. The conditioning regimen consisted of busulfan (140 mg/m<sup>2</sup>/d for 2 days), total body irradiation (12 Gy) and melphalan (210 mg/m<sup>2</sup>). The patient received cyclosporin A for graft-versus-host disease (GVHD) prophylaxis. The CD34-positive cells were separated using an immunomagnetic cell-separation system (Isolex 50). The number of infused CD34-positive cells was 4.4×10<sup>6</sup>/kg. Successful engraftment was confirmed on day 14 by fluorescent in situ hybridization of X chromosomes. The patient experienced severe diarrhea due to thrombotic microangiopathy (TMA) following acute GVHD, and died on day 71 of human herpes virus type 6 pneumonitis. Stem cell transplantation using CD34 positively selected cells from HLA-mismatched related donors may be a useful treatment with low incidence of severe GVHD, but many problems remain to be overcome, including severe viral infections and TMA.


  • Rinsho Ketsueki

    Rinsho Ketsueki 40(7), 574-580, 1999-07-30

    The Japanese Society of Hematology

References:  12


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