1.Immune Checkpoint Inhibitor-associated Myositis in a Patient with Metastatic Cholangiocarcinoma
Chung Seok LEE ; Soo-Hyun KIM ; Sung Rok LEE ; Young-Chul CHOI ; Hyung Jun PARK
Korean Journal of Neuromuscular Disorders 2024;16(2):43-46
An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.
2.Immune Checkpoint Inhibitor-associated Myositis in a Patient with Metastatic Cholangiocarcinoma
Chung Seok LEE ; Soo-Hyun KIM ; Sung Rok LEE ; Young-Chul CHOI ; Hyung Jun PARK
Korean Journal of Neuromuscular Disorders 2024;16(2):43-46
An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.
3.Immune Checkpoint Inhibitor-associated Myositis in a Patient with Metastatic Cholangiocarcinoma
Chung Seok LEE ; Soo-Hyun KIM ; Sung Rok LEE ; Young-Chul CHOI ; Hyung Jun PARK
Korean Journal of Neuromuscular Disorders 2024;16(2):43-46
An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.
4.Diagnostic Journey of Korean Patients with Spinal Muscular Atrophy
Soo-Hyun KIM ; Chung Seok LEE ; Sung Rok LEE ; Young-Chul CHOI ; Seung Woo KIM ; Ha Young SHIN ; Hyung Jun PARK
Yonsei Medical Journal 2024;65(10):572-577
Purpose:
Spinal muscular atrophy (SMA) is an autosomal recessive genetic disease characterized by the loss of motor neurons in the spinal cord and brainstem, leading to muscle atrophy and weakness. To understand the diagnostic process of Korean patients with SMA, we analyzed their clinical characteristics and challenges.
Materials and Methods:
We conducted a retrospective study of 38 patients with SMA (9 type II and 29 type III) between January 2000 and September 2023. Clinical, laboratory, and genetic data were reviewed.
Results:
The median ages at symptom onset and diagnosis were 3.0 years [interquartile range (IQR): 1.0–7.3 years] and 25.0 years (IQR: 10.5–37.3 years), respectively. The median diagnostic delay was 19.6 years (IQR: 6.4–31.0 years). A significantly longer delay was observed in SMA type III patients (median: 21.0 years, IQR: 11.0–31.0 years) compared to SMA type II patients (median: 3.0 years, IQR: 0.9–21.0 years) (p=0.021). No significant difference was observed in the number of clinic visits before diagnosis between patients with SMA type II (median: 2.0, IQR: 1.0–4.5) and those with type III (median: 2.0, IQR: 2.0–6.0, p=0.282). The number of clinic visits before diagnosis showed no significant association with the age at symptom onset and diagnosis (p=0.998 and 0.291, respectively).
Conclusion
Our investigation is the first examination of the diagnostic journey of Korean patients with SMA. As treatments for SMA progress, the significance of an accurate diagnosis has increased, highlighting the importance of reviewing the diagnostic advancements made thus far.
5.Immune Checkpoint Inhibitor-associated Myositis in a Patient with Metastatic Cholangiocarcinoma
Chung Seok LEE ; Soo-Hyun KIM ; Sung Rok LEE ; Young-Chul CHOI ; Hyung Jun PARK
Korean Journal of Neuromuscular Disorders 2024;16(2):43-46
An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.
6.Immune Checkpoint Inhibitor-associated Myositis in a Patient with Metastatic Cholangiocarcinoma
Chung Seok LEE ; Soo-Hyun KIM ; Sung Rok LEE ; Young-Chul CHOI ; Hyung Jun PARK
Korean Journal of Neuromuscular Disorders 2024;16(2):43-46
An 83-year-old man with cholangiocarcinoma developed immune checkpoint inhibitor-associated myositis after two cycles of durvalumab, presenting with muscle weakness and myalgia. His serum creatine kinase (CK) level peaked at 26,329 U/L. Needle electromyography confirmed a myogenic process, and MRI revealed extensive muscle edema. Muscle biopsy indicated multifocal necrotic fibers. Following steroid treatment, his CK levels normalized and muscle strength returned. This case represents the first reported instance of durvalumab-associated myositis in Korea.
7.Predictive role of absolute lymphocyte count in daratumumab-treated patients with relapsed/ refractory multiple myeloma
Hee Jeong CHO ; Jae-Cheol JO ; Yoo Jin LEE ; Myung Won LEE ; Do Young KIM ; Ho Jin SHIN ; Sung Nam IM ; Ji Hyun LEE ; Sung Hwa BAE ; Young Rok DO ; Won Sik LEE ; Min Kyung KIM ; Jina JUNG ; Jung Min LEE ; Ju-Hyung KIM ; Dong Won BAEK ; Sang-Kyun SOHN ; Joon Ho MOON
The Korean Journal of Internal Medicine 2023;38(4):578-578
8.Predictive role of absolute lymphocyte count in daratumumab-treated patients with relapsed/ refractory multiple myeloma
Hee Jeong CHO ; Jae-Cheol JO ; Yoo Jin LEE ; Myung Won LEE ; Do Young KIM ; Ho Jin SHIN ; Sung Nam IM ; Ji Hyun LEE ; Sung Hwa BAE ; Young Rok DO ; Won Sik LEE ; Min Kyung KIM ; Jina JUNG ; Jung Min LEE ; Ju-Hyung KIM ; Dong Won BAEK ; Sang-Kyun SOHN ; Joon Ho MOON
The Korean Journal of Internal Medicine 2023;38(2):238-247
Background/Aims:
Daratumumab has shown an encouraging antitumor effect in patients with multiple myeloma (MM), and was known to alter the immune properties by off-targeting immunosuppressive cells. Here, we aimed to evaluate the change in absolute lymphocyte count (ALC) as a surrogate marker for predicting survival outcomes of patients treated with daratumumab.
Methods:
Between 2018 and 2021, the medical records of patients with relapsed/refractory MM (RRMM) treated with daratumumab monotherapy at 10 centers in South Korea were reviewed. We collected the ALC data at pre-infusion (D0), day 2 after the first infusion (D2), and prior to the third cycle of daratumumab therapy (D56).
Results:
Fifty patients who were administered at least two cycles of daratumumab were included. Overall response rate was 54.0% after two cycles of daratumumab treatment. On D2, almost all patients experienced a marked reduction in ALC. However, an increase in ALC on D56 (ALCD56) was observed in patients with non-progressive disease, whereas failure of ALC recovery was noted in those with progressive disease. Patients with ALCD56 > 700/μL (n = 39, 78.0%) had prolonged progression- free survival (PFS) and overall survival (OS) than those with ALCD56 ≤ 700/μL (median PFS: 5.8 months vs. 2.6 months, p = 0.025; median OS: 24.1 months vs. 6.1 months, p = 0.004). In addition, ALCD56 >700/μL was a significant favorable prognostic factor for PFS (hazard ratio [HR], 0.22; p = 0.003) and OS (HR, 0.23; p = 0.012).
Conclusions
Increase in ALC during daratumumab treatment was significantly associated with prolonged survival outcomes in patients with RRMM. The ALC value can predict clinical outcomes in patients treated with daratumumab.
9.Utility of the APACHE II score as a neurological prognostic factor for glufosinate-intoxicated patients with alert mental status
Rok LEE ; Tae Yong SHIN ; Hyung Jun MOON ; Hyun Jung LEE ; Dongkil JEONG ; Dongwook LEE ; Sun In HONG ; Hyun Joon KIM
Journal of The Korean Society of Clinical Toxicology 2023;21(2):135-142
Purpose:
In patients with glufosinate poisoning, severe neurological symptoms may be closely related to a poor prognosis, but their appearance may be delayed. Therefore, this study aimed to determine whether the Acute Physiology and Chronic Health Evaluation II (APACHE II) score could predict the neurological prognosis in patients with glufosinate poisoning who present to the emergency room with alert mental status.
Methods:
This study was conducted retrospectively through a chart review for patients over 18 years who presented to a single emergency medical center from January 2018 to December 2022 due to glufosinate poisoning. Patients were divided into groups with a good neurological prognosis (Cerebral Performance Category [CPC] Scale 1 or 2) and a poor prognosis (CPC Scale 3, 4, or 5) to identify whether any variables showed significant differences between the two groups.
Results:
There were 66 patients (67.3%) with good neurological prognoses and 32 (32.8%) with poor prognoses. In the multivariate logistic analysis, the APACHE II score, serum amylase, and co-ingestion of alcohol showed significant results, with odds ratios of 1.387 (95% confidence interval [CI], 1.027–1.844), 1.017 (95% CI, 1.002–1.032), and 0.196 (95% CI, 0.040–0.948), respectively. With an APACHE II score cutoff of 6.5, the AUC was 0.826 (95% CI, 0.746–0.912). The cutoff of serum amylase was 75.5 U/L, with an AUC was 0.761 (95% CI, 0.652–0.844), and the AUC of no co-ingestion with alcohol was 0.629 (95% CI, 0.527–0.722).
Conclusion
The APACHE II score could be a useful indicator for predicting the neurological prognosis of patients with glufosinate poisoning who have alert mental status.
10.Pegfilgrastim Prophylaxis Is Effective in the Prevention of Febrile Neutropenia and Reduces Mortality in Patients Aged ≥ 75 Years with Diffuse Large B-Cell Lymphoma Treated with R-CHOP: A Prospective Cohort Study
Seong Hyun JEONG ; Seok Jin KIM ; Dok Hyun YOON ; Yong PARK ; Hye Jin KANG ; Youngil KOH ; Gyeong-Won LEE ; Won-Sik LEE ; Deok-Hwan YANG ; Young Rok DO ; Min Kyoung KIM ; Kwai Han YOO ; Yoon Seok CHOI ; Hwan Jung YUN ; Jun Ho YI ; Jae-Cheol JO ; Hyeon-Seok EOM ; Jae-Yong KWAK ; Ho-Jin SHIN ; Byeong Bae PARK ; Shin Young HYUN ; Seong Yoon YI ; Ji-Hyun KWON ; Sung Yong OH ; Hyo Jung KIM ; Byeong Seok SOHN ; Jong Ho WON ; Se-Hyung KIM ; Ho-Sup LEE ; Cheolwon SUH ; Won Seog KIM
Cancer Research and Treatment 2022;54(4):1268-1277
Purpose:
Febrile neutropenia (FN) can cause suboptimal treatment and treatment-related mortality (TRM) in diffuse large B-cell lymphoma (DLBCL) patients treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP).
Materials and methods:
We conducted a prospective cohort study to evaluate the effectiveness of pegfilgrastim prophylaxis in DLBCL patients receiving R-CHOP, and we compared them with the PROCESS cohort (n=485).
Results:
Since January 2015, 986 patients with DLBCL were enrolled. Pegfilgrastim was administered at least once in 930 patients (94.3%), covering 90.3% of all cycles. FN developed in 137 patients (13.9%) in this cohort (23.7% in the PROCESS cohort, p<0.001), and 4.2% of all cycles (10.2% in the PROCESS cohort, p<0.001). Dose delay was less common (≥3 days: 18.1% vs. 23.7%, p=0.015; ≥5 days: 12.0% vs. 18.3%, p=0.023) in this cohort than in the PROCESS cohort. The incidence of TRM (3.2% vs. 5.6%, p=0.047) and infection-related death (1.8% vs. 4.5%, p=0.004) was lower in this cohort than in the PROCESS cohort. The 4-year overall survival (OS) and progression-free survival (PFS) rates of the two cohorts were not different (OS: 73.0% vs. 71.9%, p=0.545; PFS: 69.5% vs. 68.8%, p=0.616). However, in patients aged ≥75 years, the 4-year OS and PFS rates were higher in this cohort than in the PROCESS cohort (OS: 49.6% vs. 33.7%, p=0.032; PFS: 44.2% vs. 30.3% p=0.047).
Conclusion
Pegfilgrastim prophylaxis is effective in the prevention of FN and infection-related death in DLBCL patients receiving R-CHOP, and it also improves OS in patients aged ≥75 years.

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