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1.
Article in English | WPRIM (Western Pacific) | ID: wpr-999979

ABSTRACT

Background/Aims@#Chronic hepatitis B (CHB) is a risk factor for non-Hodgkin lymphoma (NHL). Our recent study suggested that antiviral treatment may reduce the incidence of NHL in CHB patients. This study compared the prognoses of hepatitis B virus (HBV)-associated diffuse large B-cell lymphoma (DLBCL) patients receiving antiviral treatment and HBV-unassociated DLBCL patients. @*Methods@#This study comprised 928 DLBCL patients who were treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) at two referral centers in Korea. All patients with CHB received antiviral treatment. Time-to-progression (TTP) and overall survival (OS) were the primary and secondary endpoints, respectively. @*Results@#Among the 928 patients in this study, 82 were hepatitis B surface antigen (HBsAg)-positive (the CHB group) and 846 were HBsAg-negative (the non-CHB group). The median follow-up time was 50.5 months (interquartile range [IQR]=25.6–69.7 months). Multivariable analyses showed longer TTP in the CHB group than the non-CHB group both before inverse probability of treatment weighting (IPTW; adjusted hazard ratio [aHR]=0.49, 95% confidence interval [CI]=0.29–0.82, p=0.007) and after IPTW (aHR=0.42, 95% CI=0.26–0.70, p<0.001). The CHB group also had a longer OS than the non-CHB group both before IPTW (HR=0.55, 95% CI=0.33–0.92, log-rank p=0.02) and after IPTW (HR=0.53, 95% CI=0.32–0.99, log-rank p=0.02). Although liver-related deaths did not occur in the non-CHB group, two deaths occurred in the CHB group due to hepatocellular carcinoma and acute liver failure, respectively. @*Conclusions@#Our findings indicate that HBV-associated DLBCL patients receiving antiviral treatment have significantly longer TTP and OS after R-CHOP treatment than HBV-unassociated DLBCL patients.

2.
Article in English | WPRIM (Western Pacific) | ID: wpr-915501

ABSTRACT

Poor graft function (PGF) is a serious, potentially life-threatening complication of allogeneic hematopoietic stem cell transplantation. Eltrombopag has shown multilineage responses in patients with refractory severe aplastic anemia, supporting the idea that it may improve cytopenia in patients with PGF. This retrospective, single center analysis included 8 Korean patients receiving eltrombopag for PGF. Median interval between transplant and eltrombopag treatment was 73 days, and the median duration treatment was 3.5 weeks.With median maximum daily dose of 50 mg, the time to best response was 93 days. Median hemoglobin increased from 8.2 g/dL to 10.9 g/dL, platelet from 18.5 × 109 /L to 54 × 109 /L, and absolute neutrophil count from 1.25 × 109 /L to 3.32 × 109 /L. In conclusion, eltrombopag is a good option for PGF in Korean patients, even at a lower dose compared to western patients.

3.
Preprint in English | bioRxiv | ID: ppbiorxiv-194498

ABSTRACT

Severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2), which is the cause of a present global pandemic, infects human lung alveolar cells (hACs). Characterising the pathogenesis is crucial for developing vaccines and therapeutics. However, the lack of models mirroring the cellular physiology and pathology of hACs limits the study. Here, we develop a feeder-free, long-term three-dimensional (3D) culture technique for human alveolar type 2 (hAT2) cells, and investigate infection response to SARS-CoV-2. By imaging-based analysis and single-cell transcriptome profiling, we reveal rapid viral replication and the increased expression of interferon-associated genes and pro-inflammatory genes in infected hAT2 cells, indicating robust endogenous innate immune response. Further tracing of viral mutations acquired during transmission identifies full infection of individual cells effectively from a single viral entry. Our study provides deep insights into the pathogenesis of SARS-CoV-2, and the application of long-term 3D hAT2 cultures as models for respiratory diseases.

6.
Article in English | WPRIM (Western Pacific) | ID: wpr-67784

ABSTRACT

BACKGROUND/AIMS: Although multiple myeloma (MM) is typically a disease of the elderly, a certain subset of extremely young patients exists. It is necessary to establish clinicopathological characteristics for this population. METHODS: We reviewed the medical records of MM patients whose age was 40 years or younger at diagnosis. RESULTS: A total of 32 patients were analyzed (male to female ratio 19:13, median age 37 years). According to International Staging System, 29%, 48%, and 16% were in stage I, II, and III, respectively. Light chain myeloma accounted for 30%. Clinically significant anemia, hypercalcemia, azotemia, and hypoalbuminemia were present in 29%, 28%, 13%, and 28%, respectively. Three or more lytic bone lesions were detected in 45% of the patients, whereas 13% had no lytic bone lesions. Regarding treatment, 79% of patients received autologous hematopoietic stem cell transplantation. After a median follow-up duration of 64 months, the 1-, 3-, and 5-year overall survival (OS) rates were 84%, 62%, and 54%, respectively. The median OS was 61 months for the entire cohort. CONCLUSIONS: In our study, MM patients aged 40 years or younger at diagnosis showed no superior survival compared to those of the moderately elderly patients based on historical data.


Subject(s)
Aged , Female , Humans , Young Adult , Anemia , Azotemia , Cohort Studies , Diagnosis , Follow-Up Studies , Hematopoietic Stem Cell Transplantation , Hypercalcemia , Hypoalbuminemia , Medical Records , Multiple Myeloma , Treatment Outcome
7.
Article in Korean | WPRIM (Western Pacific) | ID: wpr-23590

ABSTRACT

An emphysematous pancreatitis is a rare, but fatal subtype of acute pancreatitis. Gas-forming bacteria from the bowel may penetrate the pancreas to cause emphysematous pancreatitis. It is characterized by the presence of gas within pancreas bed or retroperitoneal cavity at computed tomographic image and carries a high mortality rate. It requires fluid resuscitation and anti-bacterial therapy to control infection, and needs to consider percutaneous drainage or surgical management depending on the clinical condition. We report a case of 73-year-old patient presented with an emphysematous pancreatitis which developed fulminant multi-organ failure in spite of intensive medical treatment along with a review of the related literatures.


Subject(s)
Aged , Humans , Bacteria , Drainage , Mortality , Multiple Organ Failure , Pancreas , Pancreatitis , Pancreatitis, Acute Necrotizing , Resuscitation , Retroperitoneal Space
8.
Blood Research ; : 17-22, 2016.
Article in English | WPRIM (Western Pacific) | ID: wpr-23503

ABSTRACT

BACKGROUND: Mast cell leukemia (MCL) is the most aggressive form of systemic mastocytosis disorders. Owing to its rarity, neither pathogenesis nor standard treatment is established for this orphan disease. Hence, we tried to treat a patient with MCL based on the exome and transcriptome sequencing results of the patient's own DNA and RNA. METHODS: First, tumor DNA and RNA were extracted from bone marrow at the time of diagnosis. Germline DNA was extracted from the patient's saliva 45 days after induction chemotherapy and used as a control. Then, we performed whole-exome sequencing (WES) using the DNA and whole transcriptome sequencing (WTS) using the RNA. Single nucleotide variants (SNVs) were called using MuTect and GATK. Samtools, FusionMap, and Gene Set Enrichment Analysis were utilized to analyze WTS results. RESULTS: WES and WTS results revealed mutation in KIT S476I. Fusion analysis was performed using WTS data, which suggested a possible RARα-B2M fusion. When RNA expression analysis was performed using WTS data, upregulation of PIK3/AKT pathway, downstream of KIT and mTOR, was observed. Based on our WES and WTS results, we first administered all-trans retinoic acid, then dasatinib, and finally, an mTOR inhibitor. CONCLUSION: We present a case of orphan disease where we used a targeted approach using WES and WTS data of the patient. Even though our treatment was not successful, use of our approach warrants further validation.


Subject(s)
Humans , Bone Marrow , Diagnosis , DNA , Exome , Precision Medicine , Induction Chemotherapy , Leukemia , Leukemia, Mast-Cell , Mast Cells , Mastocytosis, Systemic , Rare Diseases , RNA , Saliva , Transcriptome , Tretinoin , Up-Regulation , Dasatinib
9.
Article in English | WPRIM (Western Pacific) | ID: wpr-44742

ABSTRACT

Catastrophic antiphospholipid syndrome (APS) is defined as a rare, life-threatening autoimmune disorder leading to multiorgan failure. Probable APS, with clinical manifestations similar to APS without antiphospholipid antibodies, was suggested to be seronegative catastrophic APS. The triggering factors of catastrophic APS are various, including infection, trauma, malignancy, and surgery. In approximately 40% of patients, catastrophic APS develops from an unknown cause. We report a case of seronegative catastrophic APS due to an unknown origin. A 20-year-old man presented with cough, abdominal pain, skin lesions, tunnel vision, and watery diarrhea without fever. His symptoms and laboratory test suggested disseminated intravascular coagulation. Considering seronegative catastrophic APS, we treated with intravenous steroid and intravenous immunoglobulin, but the effects were limited. After weekly treatment with rituximab, an immune-modulating agent, his laboratory findings including thrombocytopenia and coagulation tests, returned to normal. We conclude that rituximab can be an effective treatment for seronegative catastrophic APS.


Subject(s)
Humans , Young Adult , Abdominal Pain , Antibodies, Antiphospholipid , Antiphospholipid Syndrome , Autoimmune Diseases , Cough , Diarrhea , Disseminated Intravascular Coagulation , Fever , Immunoglobulins , Skin , Thrombocytopenia , Rituximab
10.
Article in English | WPRIM (Western Pacific) | ID: wpr-44733

ABSTRACT

Here we report a case of a 72-year-old male patient recurred in bone marrow alone with pulmonary tumor embolism after an excision of extramammary Paget's disease of scrotum 3 years ago. The patient received paclitaxel/carboplatin chemotherapy with respiratory support in intensive care unit. Four days after chemotherapy, the oxygen demand decreased and the patient was transferred to general ward. The platelet count recovered after 2 weeks. Finally, he died of hepatic failure from Paget's disease hepatic involvement confirmed by liver biopsy at 10 months after recurrence. This is a rare case of recurred extramammary Paget's disease in bone marrow alone with pulmonary tumor embolism, which was properly diagnosed with high suspicion and was successfully treated with immediate chemotherapy.


Subject(s)
Aged , Humans , Male , Biopsy , Bone Marrow , Drug Therapy , Hypertension, Pulmonary , Intensive Care Units , Liver , Liver Failure , Neoplastic Cells, Circulating , Oxygen , Paget Disease, Extramammary , Patients' Rooms , Platelet Count , Pulmonary Embolism , Recurrence , Scrotum
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