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1.
Spine J ; 2024 Jun 06.
Artículo en Inglés | MEDLINE | ID: mdl-38851494

RESUMEN

BACKGROUND CONTEXT: The Neck Disability Index (NDI), a common Patient-Reported Outcome Measure (PROM) for neck pain, lacks sufficient study on its psychometric properties in preoperative patients with cervical spondylotic myelopathy (CSM). PURPOSE: This study aims to address these gaps by conducting an exploratory factor analysis of the Japanese NDI. The objectives are 2-fold: (1) to scrutinize the psychometric properties and internal consistency of the Japanese NDI, and (2) to explore the specific regions of pain and numbness influencing the NDI. STUDY DESIGN/SETTING: A single-center observational study. PATIENT SAMPLE: A total of 100 preoperative patients with CSM. OUTCOME MEASURES: The NDI and Numerical Rating Scale (NRS) were employed to assess preoperative neck disability and pain and numbness in various body regions. METHODS: Demographic and clinical characteristics were collected, encompassing age, sex, body mass index, American Society of Anesthesiologists classification, smoking status, comorbidities, pain and numbness at various sites using the NRS, and NDI. For evaluating the psychometric properties and internal consistency of the NDI, exploratory factor analysis (EFA) and Cronbach's α coefficient were utilized. Furthermore, the impact of pain and numbness on NDI factors was examined through multivariable regression analysis. RESULTS: EFA identified 2 factors-Physical and Mental-highlighting the dual nature of neck-related disability. Physical factors (std.ß=0.724, p<.001) exerted a stronger impact on NDI scores compared to Mental factors (std.ß=0.409, p<.001). Cronbach's α coefficient was 0.831 for physical factors and 0.723 for mental factors, indicating a high level of internal consistency. Numbness in the hand (std.ß=0.338, p<.001) and pain in the neck (std.ß=0.202, p=.035) were significant variables influencing Physical factor, while numbness in the hand (std.ß=0.485, p<.001) and pain in the head (std.ß=0.374, p<.001) impacted Mental factor. CONCLUSION: This study contributes valuable insights into the psychometric properties of the NDI in preoperative patients with CSM. The identified factors emphasize the importance of addressing both physical and sensory symptoms in preoperative care.

2.
J Pathol Transl Med ; 2024 Jun 25.
Artículo en Inglés | MEDLINE | ID: mdl-38910357

RESUMEN

Herein, we report a case of plasmablastic lymphoma (PBL) and diffuse large B-cell lymphoma (DLBCL) that occurred concurrently in the large intestine. An 84-year-old female presented with a palpable rectal tumor and ileocecal tumor observed on imaging analyses. Endoscopic biopsy of both lesions revealed lymphomatous round cells. Hartmann's operation and ileocecal resection were performed for regional control. The ileocecal lesion consisted of a proliferation of CD20/CD79a-positive lymphoid cells, indicative of DLBCL. In contrast, the rectal tumor showed proliferation of atypical cells with pleomorphic nuclei and abundant amphophilic cytoplasm, with immunohistochemical findings of CD38/CD79a/MUM1/MYC (+) and CD20/CD3/CD138/PAX5 (-). Tumor cells were positive for Epstein-Barr virus- encoded RNA based on in situ hybridization and MYC rearrangement in fluorescence in situ hybridization analysis. These findings indicated the rectal tumor was most likely a PBL. Sequencing analysis for immunoglobulin heavy variable genes indicated a common B-cell origin of the two sets of lymphoma cells. This case report and literature review provide new insights into PBL tumorigenesis.

3.
Artículo en Inglés | MEDLINE | ID: mdl-38898226

RESUMEN

Prognosis for patients undergoing hematopoietic cell transplantation (HCT) has been improving. Short-term survival information, such as crude survival rates that consider deaths immediately after the transplantation, may not be sufficiently useful for assessing long-term survival. Using the data of the Japanese HCT registry, the net survival rate of patients who survived for a given period was determined according to age, disease, and type of transplant. We included a total of 41,716 patients who received their first allogeneic hematopoietic cell transplantation between 1991 and 2015. For each disease, age group, graft source subcategory, net survival was calculated using the Pohar-Perme method, and 5-year conditional net survival (CS) was calculated. Ten-year net survivals of total patient cohort were 41.5% and 47.4% for males and females, respectively. Except for myelodysplastic syndrome, multiple myeloma, and adult T-cell leukemia/lymphoma, 5-year CS for 5-year transplant survivors exceeded 90%. CS was especially high for aplastic anemia, of which was over 100% for children and younger adults receiving cord blood, suggesting that these patients have similar longevity to an equivalent group from the general population. These findings provide useful information for long-term survival, and can serve as benchmark for comparisons among registries, including other cancers.

4.
Eur Spine J ; 33(6): 2198-2205, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38679673

RESUMEN

PURPOSE: The primary objective was to validate the construct validity of the Japanese Core Outcome Measures Index (COMI) in preoperative patients aged 60 years or older undergoing lumbar spine surgery for lumbar spinal stenosis (LSS) and lumbar disk herniation (LDH). Additionally, as a secondary aim, we explored the impact of these diseases on quality of life (QOL). METHODS: The analysis included 199 preoperative patients aged 60 and above who were scheduled for lumbar spine surgery. To assess QOL, Japanese versions of the COMI, Oswestry Disability Index (ODI), EuroQol-5 Dimension-3 Level (EQ-5D-3L), and SF-12v2 were employed. The study assessed the validity of the COMI and compared demographic and clinical characteristics between the LSS (147 cases) and LDH (52 cases) groups. It used multivariate covariance analysis (MANCOVA) to examine the impact of diseases (LSS and LDH) on each patient-reported outcome measure while considering covariates. RESULTS: Compared to the LSS group, the LDH group showed more difficulty with the COMI summary score (LSS/LDH [mean]: 6.9/8.1, p < 0.001), ODI score (46.8/57.4, p < 0.001), and EQ-5D utility (0.53/0.43, p < 0.001). The LDH group also reported more difficulties in the COMI-function, COMI-symptom-specific well-being, COMI-disability, ODI-personal care, ODI-social life, and SF-12v2-bodily pain subscales. MANCOVA demonstrated that these results were not influenced by covariates such as gender and medical history. CONCLUSIONS: This study highlights the distinct impact of LSS and LDH on preoperative QOL in older patients undergoing lumbar spinal surgery. Tailored interventions are essential to address the specific challenges posed by these conditions and improve patient-centered outcomes and postoperative recovery.


Asunto(s)
Desplazamiento del Disco Intervertebral , Vértebras Lumbares , Medición de Resultados Informados por el Paciente , Calidad de Vida , Estenosis Espinal , Humanos , Estenosis Espinal/cirugía , Desplazamiento del Disco Intervertebral/cirugía , Desplazamiento del Disco Intervertebral/complicaciones , Masculino , Femenino , Anciano , Vértebras Lumbares/cirugía , Persona de Mediana Edad , Japón , Anciano de 80 o más Años , Evaluación de la Discapacidad , Pueblos del Este de Asia
5.
Prog Rehabil Med ; 9: 20240011, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38567018

RESUMEN

Objectives: Osteoporotic vertebral compression fractures (OVCFs) are common in older individuals and lead to pain, spinal deformities, and limited mobility. Paraspinal muscle function correlates with fracture severity, and this association may be more significant in patients with lumbar spinal stenosis (LSS). However, studies on the effects of OVCFs are lacking. This study aimed to investigate the relationship between OVCFs, fat infiltration, and muscle atrophy in patients with LSS. Methods: This study included 177 patients with preoperative LSS, of whom 16 had OVCFs and 161 did not. Lumbar lordosis angle, fat infiltration, and paraspinal muscle atrophy were evaluated in these patients. Information on patient characteristics such as smoking, diabetes, hemodialysis, steroid use, American Society of Anesthesiologists score, and bladder or bowel dysfunction were obtained from medical records. Logistic regression analysis was conducted to identify factors independently associated with OVCF. Results: Patients in the OVCF group were significantly older (P=0.006) than those without fractures, and a higher proportion of the OVCF group showed muscle atrophy (P=0.034). Significant variables and those with moderate effect sizes were included in the logistic regression analysis. Muscle atrophy (P=0.028) was independently associated with OVCF. Conclusions: Muscle atrophy was associated with preoperative OVCFs in patients with LSS. Identifying OVCFs in these patients may underscore the importance of tailored treatment and rehabilitation strategies for the paraspinal muscles.

6.
Transplant Proc ; 56(2): 416-421, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38336483

RESUMEN

BACKGROUND: As the Japanese population may have less genetic diversity than other ethnic groups, treatment outcomes may be affected when allogeneic hematopoietic cell transplantation is performed in other races. However, evidence explaining the effect of racial differences is limited. METHODS: We used the Japanese National Database to examine the outcomes of first allogeneic bone marrow transplantations (BMTs) performed between Japanese and non-Japanese patients from 1996 to 2021. We performed propensity score matching using sex, age group, underlying disease group, HLA mismatch, conditioning regimen intensity, and BMT implementation age to select Japanese-to-Japanese BMT patients as the controls. RESULTS: The numbers of non-Japanese-to-Japanese and Japanese-to-non-Japanese BMT cases included in the analysis were 48 and 75, respectively, and the following outcomes were compared: overall survival, non-relapse mortality, acute graft-vs-host disease (GVHD) ≥ grade II, chronic GVHD, and engraftment of neutrophils and platelets. Most parameters did not differ when comparing BMTs according to ethnicity; only platelet engraftment was delayed in Japanese-to-non-Japanese BMT but not in non-Japanese-to-Japanese BMT. CONCLUSIONS: The results of this study suggested that BMT performed in Japanese and non-Japanese patients has little effect on treatment outcomes. The results of this study may be useful for donor selection in Japan, where internationalization has progressed in recent years.


Asunto(s)
Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Humanos , Trasplante de Médula Ósea/efectos adversos , Japón , Resultado del Tratamiento , Enfermedad Injerto contra Huésped/epidemiología
7.
Eur Spine J ; 33(1): 77-83, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37889328

RESUMEN

PURPOSE: This cross-sectional study serves two main purposes. Firstly, it aims to validate the preoperative Japanese Core Outcome Measures Index for the Neck (COMI-Neck) in patients with cervical spondylotic myelopathy (CSM) and ossification of the posterior longitudinal ligament (OPLL). Secondly, it seeks to elucidate differences in preoperative quality of life (QOL) between these two cervical pathologies using patient-reported outcome measures (PROMs). METHODS: A total of 103 preoperative patients (86 with CSM and 17 with OPLL) scheduled for cervical spine surgery were included in the study. Validated PROMs, including the Japanese COMI-Neck, Neck Disability Index (NDI), EuroQol-5 Dimension-3 level (EQ-5D-3L), and SF-12v2, were used to assess QOL. Baseline demographic and clinical data were collected, and statistical analyses were performed to compare the PROMs between CSM and OPLL groups. RESULTS: The Japanese COMI-Neck demonstrated good construct validity, with positive correlations with NDI and negative correlations with EQ-5D-3L and SF-12v2. Comparison of preoperative PROMs between CSM and OPLL groups revealed differences in age, body mass index, and EQ-5D-3L scores. The CSM group had higher NDI scores for concentration and lower EQ-5D-3L scores for self-care compared to the OPLL group. CONCLUSIONS: This study validated the preoperative Japanese COMI-Neck in CSM and OPLL patients and identified specific QOL issues associated with each condition. The findings highlight the importance of considering disease-specific QOL and tailoring treatment plans accordingly. Further research should include postoperative assessments and a more diverse population to enhance generalizability.


Asunto(s)
Osificación del Ligamento Longitudinal Posterior , Enfermedades de la Médula Espinal , Espondilosis , Humanos , Vértebras Cervicales/cirugía , Estudios Transversales , Ligamentos Longitudinales , Osificación del Ligamento Longitudinal Posterior/cirugía , Osificación del Ligamento Longitudinal Posterior/complicaciones , Osteogénesis , Evaluación de Resultado en la Atención de Salud , Medición de Resultados Informados por el Paciente , Calidad de Vida , Enfermedades de la Médula Espinal/cirugía , Enfermedades de la Médula Espinal/complicaciones , Espondilosis/cirugía , Espondilosis/complicaciones , Resultado del Tratamiento
8.
Blood Adv ; 8(3): 640-652, 2024 02 13.
Artículo en Inglés | MEDLINE | ID: mdl-38100431

RESUMEN

ABSTRACT: To clarify the different characteristics and prognostic factors of cord blood transplantation (CBT) in adult patients with lymphoid neoplasms in Europe and Japan, we conducted a collaborative study. Patients aged 18-75 years receiving their first CBT (Europe: single CBT, n = 192; double CBT, n = 304; Japan: single CBT, n = 1150) in 2000-2017 were analyzed. Fewer patients with Hodgkin lymphoma (Europe vs Japan, 26% vs 5%), and older patients (≥50 years) (39% vs 59%) with a higher refined disease risk index (rDRI) (high-very high: 49% vs 14%) were included in the Japanese registry. High-very high rDRI was associated with inferior overall survival (OS) (vs low rDRI, Europe: hazard ratio [HR], 1.87; P = .001; Japan: HR, 2.34; P < .001) with higher progression/relapse risks. Total body irradiation (TBI)-containing conditioning contributed to superior OS both in Europe (vs TBI-reduced-intensity conditioning [RIC], non-TBI-RIC: HR, 1.93; P < .001; non-TBI-Myeloablative conditioning [MAC]: HR, 1.90; P = .003) and Japan (non-TBI-RIC: HR, 1.71; P < .001; non-TBI-MAC: HR 1.50, P = .007). The impact of HLA mismatches (≥2) on OS differed (Europe: HR, 1.52; P = .007; Japan: HR, 1.18; P = .107). CBT for lymphoid neoplasms, especially in those with high rDRI showed poor outcomes despite all the different characteristics in both registries. TBI should be considered in conditioning regimens to improve these outcomes. The different impacts of HLA mismatches call attention to the fundamental differences among these populations.


Asunto(s)
Trasplante de Células Madre de Sangre del Cordón Umbilical , Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Linfoma , Adulto , Humanos , Japón/epidemiología , Trasplante de Células Madre de Sangre del Cordón Umbilical/efectos adversos , Recurrencia Local de Neoplasia , Linfoma/terapia , Acondicionamiento Pretrasplante
9.
Sci Adv ; 9(50): eadj4407, 2023 Dec 15.
Artículo en Inglés | MEDLINE | ID: mdl-38091391

RESUMEN

Myeloid/natural killer (NK) cell precursor acute leukemia (MNKPL) has been described on the basis of its unique immunophenotype and clinical phenotype. However, there is no consensus on the characteristics for identifying this disease type because of its rarity and lack of defined distinctive molecular characteristics. In this study, multiomics analysis revealed that MNKPL is distinct from acute myeloid leukemia, T cell acute lymphoblastic leukemia, and mixed-phenotype acute leukemia (MPAL), and NOTCH1 and RUNX3 activation and BCL11B down-regulation are hallmarks of MNKPL. Although NK cells have been classically considered to be lymphoid lineage-derived, the results of our single-cell analysis using MNKPL cells suggest that NK cells and myeloid cells share common progenitor cells. Treatment outcomes for MNKPL are unsatisfactory, even when hematopoietic cell transplantation is performed. Multiomics analysis and in vitro drug sensitivity assays revealed increased sensitivity to l-asparaginase and reduced levels of asparagine synthetase (ASNS), supporting the clinically observed effectiveness of l-asparaginase.


Asunto(s)
Asparaginasa , Leucemia Mieloide Aguda , Humanos , Leucemia Mieloide Aguda/terapia , Enfermedad Aguda , Células Asesinas Naturales , Resultado del Tratamiento , Proteínas Represoras , Proteínas Supresoras de Tumor
10.
Ann Med Surg (Lond) ; 85(10): 4683-4688, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37811055

RESUMEN

Background: Patients with bilateral lower limb deep venous thrombosis (DVT) have a higher risk of pulmonary thromboembolism (PTE) and mortality than patients with unilateral lower limb DVT. Preoperative dilatation of the soleal vein (SV) diameter is a predictor of postoperative DVT. The purpose of this study is to investigate the cutoff value for SV diameter as a risk factor for VTE development. Materials and methods: The authors examined 274 patients with unilateral THA who met the inclusion criteria in a retrospective study. The mean age of the patients was 65.7±11.2 years, with 70 males and 204 females. Bilateral lower limb vein ultrasonography was performed preoperatively and ~1 week after THA. The frequency and localization of DVT were investigated in postoperative ultrasonography. The patients were divided into three groups: no DVT (non-DVT), unilateral lower limb DVT (Uni-DVT), and bilateral lower limb DVT (Bi-DVT). The three groups were compared in terms of preoperative venous vessel maximum diameter. Results: There were 62 patients (22.6%) who had postoperative DVT. There are no symptomatic PTE patients. DVT was found in 44 patients (16.0%) of the Uni-DVT group and 18 patients (6.6%) of the Bi-DVT group. The SV maximum diameter was 6.41±1.79 mm in the non-DVT group, 7.06±2.13 mm in the Uni-DVT group, and 8.06±2.26 mm in the Bi-DVT group, with a significant difference (P=0.001) between the non-DVT and Bi-DVT groups. In the Bi-DVT group, the cutoff value for preoperative SV maximum diameter was 6.75 mm (95% CI: 0.625-0.831; P=0.001; sensitivity, 77.8%; specificity, 60.4%; area under the curve, 0.728). Conclusions: In THA, preoperative ultrasonography with a maximum SV diameter of 6.75 mm or greater was the risk of bilateral DVT leading to fatal PTE is increased.

11.
Cytotherapy ; 25(11): 1212-1219, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37354150

RESUMEN

BACKGROUND AIMS: The prognostic impact of platelet recovery after autologous hematopoietic cell transplantation (AHCT) on clinical outcomes remains to be elucidated. We aimed to clarify the impact of platelet recovery on clinical outcomes, risk factors of delayed platelet recovery and the necessary dose of CD34+ cells for prompt platelet recovery in each patient. METHODS: Using a nationwide Japanese registry database, we retrospectively analyzed clinical outcomes of 5222 patients with aggressive non-Hodgkin lymphoma (NHL) or multiple myeloma (MM). RESULTS: At a landmark of 28 days after AHCT, a delay of platelet recovery was observed in 1102 patients (21.1%). Prompt platelet recovery was significantly associated with superior overall survival (hazard ratio [HR] 0.32, P < 0.001), progression-free survival (HR 0.48, P < 0.001) and decreased risks of disease progression (HR 0.66, P < 0.001) and non-relapse/non-progression mortality (HR 0.19, P < 0.001). The adverse impacts of a delay of platelet recovery seemed to be more apparent in NHL. In addition to the dose of CD34+ cells/kg, disease status, performance status and the hematopoietic cell transplant-specific comorbidity index in both diseases were associated with platelet recovery. We then stratified the patients into three risk groups according to these factors. For the purpose of achieving 70% platelet recovery by 28 days in NHL, the low-, intermediate- and high-risk groups needed more than 2.0, 3.0 and 4.0 × 106 CD34+ cells/kg, respectively. In MM, the low-risk group needed approximately 1.5 × 106 CD34+ cells/kg, whereas the intermediate- and high-risk groups required 2.0 and 2.5 × 106 CD34+ cells/kg to achieve about 80% platelet recovery by 28 days. CONCLUSIONS: A delay of platelet recovery after AHCT was associated with inferior survival outcomes.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Linfoma no Hodgkin , Mieloma Múltiple , Humanos , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Mieloma Múltiple/terapia , Estudios Retrospectivos , Linfoma no Hodgkin/terapia , Linfoma no Hodgkin/etiología , Plaquetas , Antígenos CD34 , Trasplante Autólogo
12.
Healthcare (Basel) ; 10(12)2022 Nov 30.
Artículo en Inglés | MEDLINE | ID: mdl-36553933

RESUMEN

This study of a randomized controlled trial aimed to clarify the effect of functional movement screen (FMS) training on the FMS score and the number of injuries in high-school baseball players. Accordingly, 71 high-school baseball players (age: 15-17 years) were randomized into an intervention group (n = 37; FMS training 4 times per week for 12 weeks on the ground of each team) or a control group (n = 34; team practice without limits). No significant differences were observed in terms of the participant characteristics of the two groups. The FMS score of the intervention group significantly increased after 12 weeks of training compared with the control group. However, there was no difference between the groups in terms of the FMS score after 24 weeks. Injuries in the intervention group were significantly reduced after 24 weeks. The time lost due to noncontact injuries (12 weeks/follow-up) was 56.5 h/113 h in the intervention group and 33 h/325.5 h in the control group. Injuries were found all over the body. Based on these results, FMS training was proven to reduce injury in high-school baseball players. Although continued training is required to improve FMS scores, the number of injuries decreased after training.

13.
Healthcare (Basel) ; 10(11)2022 Nov 11.
Artículo en Inglés | MEDLINE | ID: mdl-36421587

RESUMEN

Shoulder range of motion (ROM) adaptation is common observed among volleyball players, but studies on the shoulder joint function of adolescent athletes are lacking. This study aimed to clarify the prevalence of glenohumeral internal rotation deficit (GIRD) among adolescent players and differences in ROM based on sex. A questionnaire survey and ROM measurements of the shoulder joint and trunk using a plastic goniometer were conducted on 123 volleyball players (63 males and 60 females; mean age, 15.8 years). The prevalence of GIRD was investigated for internal rotation differences of >10° and total rotation motion of <5°. Questionnaire items and ROM were compared between GIRD and non-GIRD patients, and sex differences in ROM were also presented. Of the participants, 38.2% (n = 47/123) had GIRD. The GIRD group showed a decrease in external rotation on the dominant side (p = 0.003, 1 − beta = 0.84), but this was not associated with a history of shoulder injury. Sex differences in shoulder ROM showed hypomobility in males and hypermobility in females. However, there was no association between shoulder injury and GIRD among adolescent players. There are sex differences in ROM, which should be considered in future studies.

14.
Int J Hematol ; 116(5): 696-711, 2022 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-35809214

RESUMEN

The presence of a JAK2 V617F or JAK2 exon 12 mutation is one of the three major criteria listed for the diagnosis of polycythemia vera (PV) in the 2017 World Health Organization Classification. However, a nationwide study has not yet been conducted in Japan since the discovery of JAK2 mutations. Therefore, the Japanese Society of Hematology (JSH) retrospectively analyzed the clinical characteristics of 596 Japanese patients with PV diagnosed between April 2005 and March 2018. Among the 473 patients with complete data on JAK2 mutations available, 446 (94.3%) and 10 (2.1%) were positive for the JAK2 V617F and JAK2 exon 12 mutations, respectively. During a median follow-up of 46 months (range: 0-179 months), 47 (7.9%) deaths occurred. The major causes of death were secondary malignancies (23.4%), acute leukemia (12.8%), non-leukemic progressive disease (10.6%) and thrombotic (6.4%) and hemorrhagic complications (6.4%). Thrombotic and hemorrhagic events occurred during the clinical course in 4.0% (n = 24) and 3.5% (n = 21) of patients, respectively. These results show that the international PV prognostic score (age, venous thrombosis and leukocytosis) is applicable to Japanese patients with PV.


Asunto(s)
Hematología , Policitemia Vera , Trombosis , Humanos , Policitemia Vera/complicaciones , Japón/epidemiología , Janus Quinasa 2/genética , Estudios Retrospectivos , Trombosis/etiología , Mutación
15.
Medicine (Baltimore) ; 101(28): e29354, 2022 Jul 15.
Artículo en Inglés | MEDLINE | ID: mdl-35839054

RESUMEN

RATIONALE: Adult-onset Still disease (AOSD) is a rare inflammatory disease of unknown etiology. AOSD is common in young or middle-aged adults; however, in recent years, there have been increasing reports of elderly AOSD. Differentiating AOSD from diseases such as infections and malignancies is difficult. Moreover, rare fulminant AOSD cases with resistance to corticosteroids and immunosuppressive drugs have been reported. PATIENT CONCERNS: An 80-year-old woman presented with flaccid fever, generalized arthralgia, and erythema of the anterior chest for 2 weeks. On day 5 of hospitalization, the patient developed pleural effusion with hypoxemia and her vital signs indicated rapid progression to shock. During the clinical course, the levels of inflammatory markers, including maximum level of ferritin and white blood cells (WBCs) were elevated (252,796 ng/mL and 86,500/µV, respectively) with disseminated intravascular coagulation syndrome (DIC) and macrophage activation syndrome (MAS). DIAGNOSIS: The patient was diagnosed with elderly AOSD as per the Yamaguchi criteria for AOSD. The state of disease was extremely severe with rapid progression and was, thus, categorized as a fulminant form of elderly AOSD. INTERVENTIONS: The patient was treated with prednisolone (PSL) pulse therapy (1000 mg/d) twice and plasma exchange in the intensive care unit for the primary disease and shock. Although she recovered from shock, she developed DIC and MAS. Methotrexate (MTX; 10 mg/d) improved the DIC and MAS. However, severe pleuritis recurred and the patient developed pericarditis; her primary disease was poorly controlled. Finally, tocilizumab (TCZ) was introduced using interleukin-18 (IL-18) as a surrogate marker. The IL-18 level was measured repeatedly following admission, with the peak level (170,000 pg/mL) recorded on the 75th day of hospitalization, immediately prior to introducing TCZ. OUTCOMES: The combined use of MTX, TCZ, and PSL was effective in suppressing elderly AOSD, which was unsuccessfully controlled with MTX and PSL. Frequent monitoring of IL-18 levels proved useful for differentiating elderly AOSD from other diseases. LESSONS: A fulminant form of elderly AOSD was treated with a combination of MTX, TCZ, and PSL. Repeated monitoring of IL-18 levels can be useful for decision-making in treating elderly AOSD.


Asunto(s)
Coagulación Intravascular Diseminada , Síndrome de Activación Macrofágica , Enfermedad de Still del Adulto , Adulto , Anciano , Anciano de 80 o más Años , Anticuerpos Monoclonales Humanizados , Femenino , Humanos , Interleucina-18 , Metotrexato/uso terapéutico , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Prednisolona/uso terapéutico , Enfermedad de Still del Adulto/complicaciones , Enfermedad de Still del Adulto/diagnóstico , Enfermedad de Still del Adulto/tratamiento farmacológico , Resultado del Tratamiento
17.
Transplant Cell Ther ; 28(8): 504.e1-504.e7, 2022 08.
Artículo en Inglés | MEDLINE | ID: mdl-35577325

RESUMEN

Because cord blood (CB) units are usually selected based on the cell dose per kilogram, overweight (body mass index [BMI] ≥25 kg/m2to < 30 kg/m2) and obese (30 kg/m2 ≤ BMI) recipients tend to have difficulty in getting appropriate CB units. In general, actual body weight (ABW) is used for CB unit selection. However, ideal body weight (IBW) has been reported to be more closely correlated with successful engraftment after autologous, allogeneic bone marrow, and peripheral blood stem cell transplantation than ABW. We conducted this analysis to clarify the threshold of CD34+ cell doses based on ideal body weight (CD34IBW) and to compare the outcomes among the groups stratified by the threshold according to actual body weight (CD34ABW) and CD34IBW for overweight and obese recipients in cord blood transplantation (CBT). We retrospectively analyzed 650 overweight and obese recipients who received single-unit CBT. To focus on the recipients who received a low CD34+ cell dose/kg, those who received 1.5×105 CD34+ cells/ABW or more were excluded. Using a cut-off of 0.8×105 CD34+ cells/kg, we compared the outcomes in 3 groups with low CD34ABW and low CD34IBW (CD34Low/Low), low CD34ABW but high CD34IBW (CD34Low/High), and high CD34ABW and high CD34IBW (CD34High/High). Hematopoietic recoveries were significantly delayed in the CD34Low/Low group compared with those in the CD34Low/High group (hazard ratio [HR] 0.67 for neutrophil, P < .001; HR 0.72 for platelet, P = .014), whereas those were comparable in the CD34Low/High and CD34High/High groups (HR 1.22 for neutrophil, P = .16; HR 1.29 for platelet, P = .088). Moreover, the CD34Low/High group demonstrated longer overall survival than the CD34Low/Low group (HR 1.48, P = .011) and comparable survival to the CD34High/High group (HR 0.93, P = .68). This finding may address the lack of availability of CB units for some overweight and obese recipients for whom suitable donors are unavailable. Further investigations are warranted to evaluate the appropriateness of ABW and IBW.


Asunto(s)
Trasplante de Células Madre de Sangre del Cordón Umbilical , Trasplante de Células Madre Hematopoyéticas , Antígenos CD34 , Peso Corporal , Humanos , Peso Corporal Ideal , Neutrófilos , Obesidad/terapia , Sobrepeso/terapia , Estudios Retrospectivos
18.
Rinsho Ketsueki ; 63(3): 177-181, 2022.
Artículo en Japonés | MEDLINE | ID: mdl-35387929

RESUMEN

Herein we report a case of successful treatment of secondary graft failure due to poor graft function (PGF) using eltrombopag. A 25-year-old woman with aplastic anemia (stage 3) underwent allogeneic bone marrow transplantation (BMT) from her HLA-matched brother. Neutrophil engraftment was achieved on day 17, but she remained dependent on platelet transfusion. Chimerism analysis showed complete donor type, but she also became dependent on red blood cell transfusion later. Eltrombopag was administered on day 253 after BMT, after which she exhibited hematopoietic recovery, resulting in the withdrawal of transfusion dependency. Blood counts continued to be stable after eltrombopag was discontinued. The use of eltrombopag enabled outpatient treatment and induced hematopoietic recovery without significant side effects. Eltrombopag may be an effective and safe option for PGF after BMT.


Asunto(s)
Anemia Aplásica , Trasplante de Células Madre Hematopoyéticas , Adulto , Femenino , Humanos , Masculino , Anemia Aplásica/etiología , Benzoatos , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Hidrazinas/uso terapéutico , Pirazoles
19.
Intern Med ; 61(10): 1581-1585, 2022 May 15.
Artículo en Inglés | MEDLINE | ID: mdl-35314552

RESUMEN

We herein report two cases of coronavirus disease 2019 (COVID-19) vaccine-induced immune thrombocytopenia (ITP). A 69-year-old Japanese man developed severe thrombocytopenia after COVID-19 vaccination. He had oral bleeding and hemoptysis but no thrombotic symptoms. He improved rapidly with oral prednisolone therapy. A 34-year-old Japanese woman had generalized purpura after COVID-19 vaccination. Her platelet count improved rapidly after treatment with prednisolone and eltrombopag. The occurrence of two cases of ITP after COVID-19 vaccination at a single institution suggests that there could be more such undiagnosed cases, especially cases of mild secondary ITP.


Asunto(s)
COVID-19 , Púrpura Trombocitopénica Idiopática , Trombocitopenia , Adulto , Anciano , Vacunas contra la COVID-19/efectos adversos , Femenino , Humanos , Masculino , Prednisolona/uso terapéutico , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Púrpura Trombocitopénica Idiopática/etiología , Trombocitopenia/inducido químicamente , Vacunación
20.
Bone Marrow Transplant ; 57(5): 810-816, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35314792

RESUMEN

Chromosome analysis is a powerful prognostic tool in myeloid malignancies. Recipients who experience relapse after allogeneic hematopoietic cell transplantation (allo-HCT) often show chromosomal changes between diagnosis and relapse. However, the clinical impact of chromosomal changes and the efficacy of post-relapse treatment according to chromosomal changes have not been fully investigated. We retrospectively analyzed 72 recipients who had experienced relapse after allo-HCT for acute myeloid leukemia or myelodysplastic syndrome. We categorized them into two groups: with or without clonal chromosomal changes at relapse after allo-HCT. Post-relapse survival was shorter in the clonal chromosomal change group (median 117 days vs 275 days, P = 0.019). Moreover, acquisition of chromosome 7 abnormality or complex changes tended to be associated with inferior survival in a univariate analysis (median 92 days vs median 173 days, P = 0.043), and this adverse impact was confirmed in a multivariate analysis (hazard ratio 2.07, P = 0.024). The patterns of chromosomal changes from diagnosis to relapse after allo-HCT were heterogenous, and further investigations are required to clarify the effect of individual chromosomal changes.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Leucemia Mieloide Aguda , Síndromes Mielodisplásicos , Cromosomas , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Leucemia Mieloide Aguda/genética , Leucemia Mieloide Aguda/terapia , Síndromes Mielodisplásicos/genética , Síndromes Mielodisplásicos/terapia , Pronóstico , Recurrencia , Estudios Retrospectivos
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