PMC4197197_01
Clinical course from conventional chemotherapy (mLSG-15) to allogeneic peripheral blood stem cell transplantation. The patient received four sessions of mLSG-15 therapy and achieved complete remission (CR) before receiving allo-PBSCT.
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Fig1
She received following allogeneic peripheral blood stem cell transplantation (allo-PBSCT) in the National Cancer Center Hospital (Tokyo, Japan) (Figure  1).
MRI findings. A) At the onset of neurogenic disorder in July 2012. Multiple high-intensity lesions in T2-weighted images (T2WI) of the medulla oblongata, cervical spinal cord, and thoracic spinal cord were revealed. B) Residual high-intensity lesion in Th3-7. C) Following treatment, the residual lesion in the thoracic spinal cord improved.
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Fig2
Magnetic resonance imaging (MRI) revealed multiple high-intensity lesions in T2-weighted images of the medulla oblongata, cervical spinal cord, and thoracic spinal cord (Figure  2A), and a CSF specimen showed increased cell counts (Figure  3). Th3-7 (Figure  2B). The cell numbers in CSF did not increase, but myelin basic protein (MBP) level was elevated (Figure  4B). Furthermore, the MRI findings improved over time (Figure  2C), although her neurological findings worsened and HTLV-I proviral DNA could not be detected repeatedly in peripheral mononuclear cells (PBMCs) after allo-PBSCT.
Clinical course after onset of the neurogenic disorder. The patient developed paraplegia 14 months after allo-PBSCT. Neurological findings were partially relieved following treatment with a high dose of mPSL accompanied by intrathecal injection of MTX + Ara-C + PSL and irradiation of the whole brain and spine. Three months later, her neurological deficit worsened again. Ultimately, her neurological disorder improved after treatment with a high dose of steroid.
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Fig3
Magnetic resonance imaging (MRI) revealed multiple high-intensity lesions in T2-weighted images of the medulla oblongata, cervical spinal cord, and thoracic spinal cord (Figure  2A), and a CSF specimen showed increased cell counts (Figure  3).
CSF findings. A) Flow cytometric analysis of CSF. Before treatment, the CD4 + CXCR3 + CCR4+ cell population was predominantly elevated. Following treatment, it decreased and the CD4 + CXCR3 + CCR4- cell population increased. B) Neopterin and CXCL10 (IP-10) concentrations in CSF. Before treatment, both neopterin and CXCL10 (IP-10) concentrations were significantly elevated. Following treatment, both biomarkers decreased to within the range of the therapeutic goal for HAM patients.
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Fig4
Th3-7 (Figure  2B). The cell numbers in CSF did not increase, but myelin basic protein (MBP) level was elevated (Figure  4B). Flow cytometric analysis to determine the specific immunophenotype of CD4+ lymphocytes in CSF revealed an expansion of the CD4+CXCR3+CCR4+ cell population (Figure  4A), which conflicted with CNS relapse of ATL but was consistent with HAM (Natsumi et al. ). Furthermore, both the neopterin and CXCL10 (IP-10) concentrations in the CSF were significantly elevated (Figure  4B), although lower than those associated with aggressive HAM (14). HAM patients (Figure  4A,B). Her paralysis improved gradually and steadily only by rehabilitation, to such a degree that she could walk when holding onto parallel bars.