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1.
Cureus ; 16(7): e64830, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39156469

ABSTRACT

We report on the rehabilitation of a patient with amyloid light chain (AL) amyloidosis complicated by nephrotic syndrome. Various symptoms produced by AL amyloidosis, including nephrotic syndrome, complicate rehabilitation therapy. In this case report, long-term physical therapy was initiated prior to autologous peripheral blood stem cell transplantation owing to the risk of further decline in physical function due to decreased mobility and physical activity. Patients were instructed on how to perform home exercise therapy. Furthermore, compliance was monitored using a checklist and regular face-to-face feedback. There was no increase in skeletal muscle mass, but improvements in grip strength, lower extremity muscle strength, and phase angle were observed after 24 weeks of physical therapy. Despite the absence of partial remission (urinary protein level of 3.5 g/gCre or higher), nephrotic syndrome demonstrated a trend toward improvement. Since the effectiveness of physical therapy in such patients has not yet been fully established, this report suggests that long-term rehabilitation therapy for physical function in patients with nephrotic syndrome complicated by persistent proteinuria may be effective.

2.
EJHaem ; 4(1): 273-275, 2023 Feb.
Article in English | MEDLINE | ID: mdl-36819153

ABSTRACT

Translocation t(6;11) occurs in approximately 5% of patients with acute myeloid leukemia (AML) corresponding to 11q23/mixed lineage leukemia (MLL) rearrangement. The AF6 gene on chromosome 6q27 is the fusion partner of the MLL gene on 11q23 in t(6;11), which results in a poor prognosis. The case of a patient with 11q23/MLL-rearranged AML who successfully underwent a third allogeneic stem cell transplantation after treatment with azacitidine (AZA) and venetoclax (VEN) is presented in this article. This report suggests that a combination of AZA and VEN is an effective therapeutic approach for relapsed and refractory MLL-rearranged AML.

3.
Intern Med ; 62(15): 2243-2247, 2023 Aug 01.
Article in English | MEDLINE | ID: mdl-36450475

ABSTRACT

Patients with acute myeloid leukemia (AML) harboring FMS-like tyrosine kinase 3 (FLT3)-internal tandem duplication mutation are associated with a poor survival outcome, even those receiving allogeneic stem cell transplantation (Allo-SCT). An additional treatment strategy with allo-SCT is therefore required to reduce relapse in these patients. Gilteritinib is a specific FLT3 inhibitor that has shown clinical benefit for patients with relapsed and refractory (R/R) AML harboring FLT3 mutation. We herein report a 49-year-old woman with R/R AML who was successfully treated with pre- and post-transplant gilteritinib. Post-transplant gilteritnib yielded a durable response with possible exacerbation of graft-versus-host disease.


Subject(s)
Hematopoietic Stem Cell Transplantation , Leukemia, Myeloid, Acute , Peripheral Blood Stem Cell Transplantation , Female , Humans , Middle Aged , Mutation , Leukemia, Myeloid, Acute/drug therapy , Leukemia, Myeloid, Acute/genetics , fms-Like Tyrosine Kinase 3/genetics , fms-Like Tyrosine Kinase 3/therapeutic use
5.
Acta Haematol ; 145(6): 655-661, 2022.
Article in English | MEDLINE | ID: mdl-35896078

ABSTRACT

Progressive multifocal leukoencephalopathy (PML) is a rare neurological disease caused by the reactivation of latent John Cunningham polyomavirus. Hematological disorders associated with immunomodulatory monoclonal antibodies and hematopoietic stem cell transplantation (HSCT) are risk factors for PML. Blinatumomab is a novel antileukemic immunomodulatory agent and more effective for relapsed and refractory acute lymphoblastic leukemia (ALL) than conventional chemotherapy. But, blinatumomab suppresses humoral immunity due to long-lasting B-cell depletion during and after the treatment. The development of PML involves cellular immunity and impairment of humoral immunity. Although few cases of blinatumomab-related PML have been reported, the use of blinatumomab after allogeneic HSCT may increase the risk of developing PML. The current case report presents a patient of Philadelphia chromosome-positive ALL wherein PML developed after cord blood stem cell transplantation and administrating blinatumomab.


Subject(s)
Antineoplastic Agents , Cord Blood Stem Cell Transplantation , Hematopoietic Stem Cell Transplantation , Leukoencephalopathy, Progressive Multifocal , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Humans , Leukoencephalopathy, Progressive Multifocal/etiology , Leukoencephalopathy, Progressive Multifocal/chemically induced , Cord Blood Stem Cell Transplantation/adverse effects , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Hematopoietic Stem Cell Transplantation/adverse effects , Antineoplastic Agents/adverse effects
6.
Int J Hematol ; 112(2): 243-248, 2020 Aug.
Article in English | MEDLINE | ID: mdl-32170661

ABSTRACT

Acute myeloid leukemia (AML) harboring Fms-like tyrosine kinase 3 (FLT3) internal tandem duplication (ITD) mutation is associated with shorter remission and higher relapse risk. Several FLT3 inhibitors have been used in clinical trials, but their efficacy in extramedullary disease remains unclear. In the present case, a 56-year-old man was diagnosed with FLT3-ITD mutated AML. Due to bone marrow relapse during consolidation therapy, he underwent salvage therapy and a myeloablative conditioning regimen, followed by peripheral blood stem cell transplantation (PBSCT) from a HLA-matched related donor. Acute graft-versus-host disease (GVHD) did not develop, and complete donor chimerism was confirmed on days 27 and 96 after PBSCT. On day 180, he experienced extensive chronic GVHD and had several subcutaneous tumors in his body, which were diagnosed as myeloid sarcoma by pathological examination. We considered this to be a case of isolated extramedullary relapse, as his bone marrow had maintained complete donor chimerism. Treatment with etoposide and ranimustine produced no effect, and tumor progression continued. We started administration of gilteritinib, a FLT3/AXL inhibitor, after identifying the FLT3-ITD mutation in the tumor. Subsequently, there has been a remarkable regression of the tumors. Gilteritinib can be effective in isolated extramedullary relapse after allogeneic stem cell transplantation.


Subject(s)
Aniline Compounds/administration & dosage , Hematopoietic Stem Cell Transplantation , Leukemia, Myeloid, Acute/genetics , Leukemia, Myeloid, Acute/therapy , Neoplasm Recurrence, Local , Pyrazines/administration & dosage , fms-Like Tyrosine Kinase 3/genetics , Humans , Male , Middle Aged , Mutation , Tandem Repeat Sequences/genetics , Transplantation, Homologous , Treatment Outcome , fms-Like Tyrosine Kinase 3/antagonists & inhibitors
7.
Sci Rep ; 7(1): 3663, 2017 06 16.
Article in English | MEDLINE | ID: mdl-28623251

ABSTRACT

To expand our knowledge of the ontogeny of the T-cell receptor (TCR) repertoire of antigen-specific T-cell subsets, we combined next-generation deep sequencing and single-cell multiplex clonotype analysis to evaluate the diversity and frequency of paired TCRs, their functions and whether clonotypic TCRs are shared among different individuals. Using an HLA-A*02-restricted cytomegalovirus (CMV) pp65-derived immunogenic peptide, we found that the more dominant pp65-specific TCR clonotypes in the blood of healthy donors have higher binding affinities for the CMV peptide and arise from clonotypes that are highly shared among individuals. Interestingly, these highly shared HLA-A*02-restricted CMV-specific TCRs were detected in a CMV-seronegative individual as well as in HLA-A*02-negative donors albeit at lower frequency. More intriguingly, these shared TCR clonotypes were abundant in the stem memory T-cell subset, and TCR diversity of the stem memory T-cell repertoire was significantly lower than in the central memory and effector memory T-cell repertoires. These results suggest that the stem memory T-cell subset may serve as a reservoir of highly shared and highly functional memory T-cells.


Subject(s)
Immunologic Memory , Precursor Cells, T-Lymphoid/immunology , Precursor Cells, T-Lymphoid/metabolism , Receptors, Antigen, T-Cell/metabolism , Biomarkers , Cell Line , Gene Expression , Genetic Variation , HLA Antigens/genetics , HLA Antigens/immunology , High-Throughput Nucleotide Sequencing , Humans , Immunity, Cellular , Immunophenotyping , Lymphocyte Activation/immunology , Phosphoproteins/immunology , Receptors, Antigen, T-Cell/genetics , T-Cell Antigen Receptor Specificity , Transduction, Genetic , Viral Matrix Proteins/immunology
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