Treatment pattern of chronic lymphocytic leukemia/small lymphocytic lymphoma in Korea: a multicenter retrospective study (KCSG LY20-06)

Background/Aims@#Little attention is paid to chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) in Korea due to the rarity of the disease. With its rising incidence, we aimed to evaluate recent changes in treatment patterns and survival outcomes of patients with CLL/SLL. @*Methods@#A total of 141 patients diagnosed with CLL/SLL between January 2010 and March 2020 who received systemic therapy were analyzed in this multicenter retrospective study. @*Results@#The median patient age was 66 years at diagnosis, and 68.1% were male. The median interval from diagnosis to initial treatment was 0.9 months (range: 0–77.6 months), and the most common treatment indication was progressive marrow failure (50.4%). Regarding first-line therapy, 46.8% received fludarabine, cyclophosphamide, plus rituximab (FCR), followed by chlorambucil (19.9%), and obinutuzumab plus chlorambucil (GC) (12.1%). The median progression-free survival (PFS) was 49.3 months (95% confidence interval [CI], 32.7–61.4), and median overall survival was not reached (95% CI, 98.4 mo– not reached). Multivariable analysis revealed younger age (≤ 65 yr) (hazard ratio [HR], 0.46; p < 0.001) and first-line therapy with FCR (HR, 0.64; p = 0.019) were independently associated with improved PFS. TP53 aberrations were observed in 7.0% (4/57) of evaluable patients. Following reimbursement, GC became the most common therapy among patients over 65 years and second in the overall population after 2017. @*Conclusions@#Age and reimbursement mainly influenced treatment strategies. Greater effort to apply risk stratifications into practice and clinical trials for novel agents could help improve treatment outcomes in Korean patients.

Coil Embolization for Supra-annular Rupture During Transcatheter Aortic Valve Replacement Under Extracorporeal Membrane Oxygenation Support

no abstract available.

Cardiac Imaging of Acute Myocarditis Following COVID-19 mRNA Vaccination

Increasing rates of coronavirus disease 2019 (COVID-19) vaccination coverage will result in more vaccine-related side effects, including acute myocarditis. In Korea, we present a 24-year-old male with acute myocarditis following COVID-19 vaccination (BNT162b2).His chest pain developed the day after vaccination and cardiac biomarkers were elevated.Echocardiography showed minimal pericardial effusion but normal myocardial contractility.Electrocardiography revealed diffuse ST elevation in lead II, and V2-5. Cardiac magnetic resonance images showed the high signal intensity of T2- short tau inversion recovery image, the high value of T2 mapping sequence, and late gadolinium enhancement in basal inferior and inferolateral wall. It was presumed that COVID-19 mRNA vaccination was probably responsible for acute myocarditis. Clinical course of the patient was favorable and he was discharged without any adverse event.

Personal Listening Device Use Habits, Listening Belief, and Perceived Change in Hearing Among Adolescents

Purpose@#This study aimed to identify personal listening device (PLD) usage habits, listening belief, and perceived change in hearing, and to investigate how the variables related to perceived change in hearing among adolescents. @*Methods@#The participants were 183 middle school students and 233 high school students from Ulsan. Data were collected by self-reported questionnaires from August 1, 2019, to October 22, 2019, and analyzed with descriptive statistics, χ2 test, Mann–Whitney U test, and hierarchical multiple logistic regression. @*Results@#Most students started to use PLDs from elementary school, and mean preferred listening level was 70.13 dB. Mean perceived susceptibility to music-induced hearing loss (MIHL) and perceived barriers to prevent MIHL were 5.18 and 3.40, respectively. Perceived change in hearing was reported on 14.2% of middle school students and 32.2% of high school students, and as the average score of perceived barriers to prevent MIHL increases by one point, the odds of decrease in hearing increase by 2.05 times (p < .001) and 1.35 times (p < .05), respectively. @*Conclusion@#Considering that most adolescents are exposed to PLD in elementary schools and about a quarter experienced a decrease in hearing after PLD use, educational programs on hearing conservation are required to start as early as in schools. Particularly, knowledge and skills to overcome barriers to prevent MIHL should be emphasized in educational programs.

Cardiac Imaging of Acute Myocarditis Following COVID-19 mRNA Vaccination

Increasing rates of coronavirus disease 2019 (COVID-19) vaccination coverage will result in more vaccine-related side effects, including acute myocarditis. In Korea, we present a 24-year-old male with acute myocarditis following COVID-19 vaccination (BNT162b2).His chest pain developed the day after vaccination and cardiac biomarkers were elevated.Echocardiography showed minimal pericardial effusion but normal myocardial contractility.Electrocardiography revealed diffuse ST elevation in lead II, and V2-5. Cardiac magnetic resonance images showed the high signal intensity of T2- short tau inversion recovery image, the high value of T2 mapping sequence, and late gadolinium enhancement in basal inferior and inferolateral wall. It was presumed that COVID-19 mRNA vaccination was probably responsible for acute myocarditis. Clinical course of the patient was favorable and he was discharged without any adverse event.

Personal Listening Device Use Habits, Listening Belief, and Perceived Change in Hearing Among Adolescents

Purpose@#This study aimed to identify personal listening device (PLD) usage habits, listening belief, and perceived change in hearing, and to investigate how the variables related to perceived change in hearing among adolescents. @*Methods@#The participants were 183 middle school students and 233 high school students from Ulsan. Data were collected by self-reported questionnaires from August 1, 2019, to October 22, 2019, and analyzed with descriptive statistics, χ2 test, Mann–Whitney U test, and hierarchical multiple logistic regression. @*Results@#Most students started to use PLDs from elementary school, and mean preferred listening level was 70.13 dB. Mean perceived susceptibility to music-induced hearing loss (MIHL) and perceived barriers to prevent MIHL were 5.18 and 3.40, respectively. Perceived change in hearing was reported on 14.2% of middle school students and 32.2% of high school students, and as the average score of perceived barriers to prevent MIHL increases by one point, the odds of decrease in hearing increase by 2.05 times (p < .001) and 1.35 times (p < .05), respectively. @*Conclusion@#Considering that most adolescents are exposed to PLD in elementary schools and about a quarter experienced a decrease in hearing after PLD use, educational programs on hearing conservation are required to start as early as in schools. Particularly, knowledge and skills to overcome barriers to prevent MIHL should be emphasized in educational programs.

Effects of anxiety and smartphone dependency on sleep quality among pregnant women with preterm labor

Purpose@#The purpose of this study was to investigate the effects of anxiety and smartphone dependency on sleep quality in pregnant women with preterm labor. @*Methods@#The participants of this study were 111 pregnant women who were between 20 and 37 weeks of gestation and experienced preterm labor. The data were collected from October 1, 2018 to October 25, 2019. The collected data were analyzed using descriptive statistics (frequency, percentage, and standard deviation), as well as the t-test, Pearson correlation coefficients, and hierarchical multiple regression. @*Results@#Significant negative correlations were found between anxiety and sleep quality and between smartphone dependency and sleep quality. Participants’ history of preterm birth, pregnancy method, bowel movements, anxiety, and smartphone dependency significantly affected sleep quality, with an explanatory power of 18%. @*Conclusion@#In order to improve the quality of sleep, which is an important health-related factor for pregnant women experiencing preterm labor, it will be necessary to identify a history of premature birth, pregnancies achieved using artificial reproductive technology, bowel problems, and smartphone dependency in advance and to provide nursing interventions accordingly.

Clinical implications of APEX1 and Jagged1 as chemoresistance factors in biliary tract cancer

PURPOSE: Biliary cancer is a highly malignant neoplasm with poor prognosis and most patients need to undergo palliative chemotherapy, however major clinical problem associated with the use of chemotherapy is chemoresistance. So far, we aimed at investigating clinical implications of apurinic/apyrimidinic endodeoxyribonuclease 1 (APEX1) and Jagged1 as chemoresistance factors in biliary tract cancer. METHODS: We used 5 human biliary tract cancer cell lines (SNU-245, SNU-308, SNU-478, SNU-1079, and SNU-1196), and investigated the chemosensitivity of APEX1 and Jagged1 through 3-(4, 5-dimethylthiazol-2-yl)-2, 5-diphenyltetrazolium bromide (MTT) assay and Western blot. Alternately, the 10 patients of advanced biliary cancer consist of 2 group according to the chemotherapy response examined by immunohistochemistry using APEX1 and Jagged1 antibody, and protein expression level was scored for staining intensity and percent positive cell. RESULTS: The result of MTT assay after APEX1 knockdown showed that strong coexpression of APEX1 and Jagged1 cell line (SNU-245, SNU-1079, and SNU-1196) showed a greater decrease in IC₅₀ of chemotherapeutic agent (5-fluorouracil, gemcitabine and cisplatin). The Western blot analysis of APEX1 and Jagged1 expression in biliary cancer cell lines after APEX1 knockdown definitively demonstrated decreased Jagged1 expression. The APEX1 and Jagged1expression level of immunohistochemistry represented that chemorefractory patients had higher than chemoresponsive patients. CONCLUSION: These results demonstrate that simultaneous high expression of APEX1 and Jagged1 is associated with chemoresistance in biliary cancer and suggest that is a potential therapeutic target for chemoresistance in advanced biliary cancer.

Spontaneous Remission of Pernicious Anemia / 대한내과학회지

Pernicious anemia is a macrocytic anemia that is caused by vitamin B12 deficiency, itself a result of the absence of intrinsic factors due to autoimmune destruction of parietal cells. We report here the case of a 43-year-old female with spontaneous remission of pernicious anemia. The patient presented with fatigue. Her serum vitamin B12 level was low, hemoglobin level was 7.6 g/dL, and serologic tests for anti-intrinsic factor and anti-parietal cell antibodies were positive. We diagnosed her with pernicious anemia, but did not administer vitamin B12 because her hemoglobin level increased spontaneously. Since then, the patient's hemoglobin and serum vitamin B12 levels have been within the normal range.

Pseudomyxoma Peritonei in a Patient with History of Breast Cancer / 대한소화기학회지

Pseudomyxoma peritonei is a very rare condition, and even rarer in patients with history of cancer. A 70-year old woman with a history of breast cancer was admitted with abdominal pain and distention. Abdominal computed tomography revealed ascites collection, diffuse engorgement and infiltration of the mesenteric vessel, suggesting peritonitis or peritoneal carcinomatosis. Diagnostic paracentesis was attempted several times, but a sufficient specimen could not be collected due to the thick and gelatinous nature of the ascites. Therefore, the patient underwent diagnostic laparoscopy for tissue biopsy of the peritoneum, which indicated pseudomyxoma peritonei. However, the origin of the pseudomyxoma peritonei could not be identified intraoperatively due to adhesions and large amount of mucoceles. Systemic chemotherapy was performed using Fluorouracil, producing some symptomatic relief. After discharge, abdominal pain and distention gradually worsened, so at 18 months after initial diagnosis the patient received palliative surgery based on massive mucinous ascites and palpable mass at the omentum. The patient expired after surgery due to massive bleeding.

A Case of Surgical Resection in Hepatocellular Carcinoma with Pulmonary Metastasis

Hepatocellular carcinoma (HCC) is well known malignancy with poor prognosis, even after resection of the primary tumor. Sorafenib is the first-line treatment in advanced HCC, but the disease control rate of sorafenib is only 43%. Pulmonary metastasectomy in patients with pulmonary metastasis from HCC has been reported to increase long-term survival compared with systemic chemotherapy. Video-assisted thoracic surgery is considered a reliable approach to the diagnosis and treatment of pulmonary diseases with low complication rate. Pulmonary metastasectomy is not universally accepted because of frequent local recurrence, an uncontrollable primary tumor, and frequent multiple pulmonary metastases in HCC, but outcome of pulmonary metastasectomy and adjuvant sorafenib therapy has not been studied. We experienced a patient who had advanced HCC with pulmonary oligometastasis and received surgical resection of the metastatic pulmonary nodule and sorafenib chemotherapy. In advanced HCC with pulmonary oligometastasis, surgical resection of pulmonary metastasis and sorafenib chemotherapy should be considered.

Takayasu arteritis and antiphospholipid antibody syndrome in an elderly woman

No abstract available.

Analysis of Histologic Features Suspecting Anaplastic Lymphoma Kinase (ALK)-Expressing Pulmonary Adenocarcinoma

BACKGROUND: Since 2007 when anaplastic lymphoma kinase (ALK) rearrangements were discovered in non-small cell lung cancer, the ALK gene has received attention due to ALK-targeted therapy, and a notable treatment advantage has been observed in patients harboring the EML4/ALK translocation. However, using ALK-fluorescence in situ hybridization (FISH) as the standard method has demerits such as high cost, a time-consuming process, dependency on interpretation skill, and tissue preparation. We analyzed the histologic findings which could complement the limitation of ALK-FISH test for pulmonary adenocarcinoma. METHODS: Two hundred five cases of ALK-positive and 101 of ALK-negative pulmonary adenocarcinoma from January 2007 to May 2013 were enrolled in this study. The histologic findings and ALK immunohistochemistry results were reviewed and compared with the results of ALK-FISH and EGFR/KRAS mutation status. RESULTS: Acinar, cribriform, and solid growth patterns, extracellular and intracellular mucin production, and presence of signet-ring-cell element, and psammoma body were significantly more often present in ALK-positive cancer. In addition, the presence of goblet cell-like cells and presence of nuclear inclusion and groove resembling papillary thyroid carcinoma were common in the ALK-positive group. CONCLUSIONS: The above histologic parameters can be helpful in predicting ALK rearranged pulmonary adenocarcinoma, leading to rapid FISH analysis and timely treatment.

Right Ventricular Rupture Caused by Prolonged Cardiopulmonary Resuscitation after Sudden Cardiac Arrest / 대한내과학회지

A 46-year-old male arrived at the emergency department with acute dyspnea. On the way to the hospital, heart massage was performed in the ambulance due to asystole on electrocardiography. After 2 hr of resuscitation, sinus rhythm was restored. Extracorporeal life support and an intra-aortic balloon pump were applied due to cardiogenic shock, but the patient showed sustained hypotension. Echocardiography showed moderate pericardial effusion with physiological evidence of cardiac tamponade; emergency pericardiocentesis was performed, which produced bloody pericardial fluid. An explorative sternotomy revealed a massive hematoma in the mediastinum and right ventricular (RV) free wall rupture. After primary repair, echocardiography showed improved left ventricular systolic function and the patient was stable clinically. This case presents RV free wall rupture as an unusual complication of prolonged heart massage. Heart rupture should be considered in hemodynamically unstable patients after prolonged heart massage.

Evaluation of risk factors in patients with vitamin K-dependent coagulopathy presumed to be caused by exposure to brodifacoum

BACKGROUND/AIMS: Recently, many cases of vitamin K-dependent coagulopathy of unknown origin have been reported. Such patients lack any relevant family history and have no systemic disease, raising suspicion of superwarfarin intoxication. We evaluated individual risk factors causing coagulopathy and hemorrhagic symptoms in patients with suspected superwarfarin intoxication. In addition, we determined how to effectively treat vitamin K-dependent coagulopathy caused by suspected superwarfarin intoxication. METHODS: Seven patients with suspected superwarfarin intoxication who lacked any definitive history of rodenticide ingestion were included. Thirty-one patients initially diagnosed with rodenticide poisoning were also included. We performed a retrospective chart review of all subjects and examined clinical data including patient demographics and medical histories. RESULTS: Patients initially diagnosed with rodenticide poisoning were divided into two groups, one of which had a laboratory abnormality (prothrombin time [PT] > 13 seconds) and another group with PTs in the normal range. There was no significant difference between the two groups in any of age, gender, the extent of chronic alcohol consumption, the causative rodenticide, psychiatric problems, ingestion of drugs interacting with warfarin, the extent of intoxication, or the type of ingestion attempt. The albumin level of the former group was significantly lower than that of the latter group (p = 0.014). Furthermore, a significant difference between the two groups was evident in terms of simultaneous ingestion of rodenticide and alcohol (p = 0.023). CONCLUSIONS: Most patients with superwarfarin poisoning did not exhibit any complication. When such complications were evident, they were associated with serum albumin level and coingestion of rodenticide and alcohol.

Effect of Cyclophosphamide and Prednisone as a First-line Treatment for Non-transplant Candidates with Multiple Myeloma / 대한내과학회지

BACKGROUND/AIMS: For many years, conventional treatment for multiple myeloma (MM) not ineligible for high-dose therapy has been the combination of oral melphalan and prednisone (MP). However, melphalan-based regimens are associated with numerous complications. Another alkylating agent, cyclophosphamide, has similar effects on MM and is associated with fewer reports of complications. Therefore, cyclophosphamide-based regimens have usually been used as salvage therapy in patients with refractory or relapsed MM, despite the development of newer drugs. The purpose of this report was to evaluate the efficacy and tolerability of cyclophosphamide and prednisone as a first-line therapy for MM. METHODS: For the period January 2002 to June 2012, we retrospectively analyzed 29 patients newly diagnosed with MM who underwent a treatment regimen consisting of intravenous cyclophosphamide (1,000 mg/kg) for 1 day and prednisone (100 mg) for 4 days. RESULTS: The rate of response to this regimen was 31.1 percent. The median progression-free survival (PFS) was 5.5 months and the median overall survival (OS) was 47.3 months. The regimen was well tolerated. Adverse effects of grades above III were as follows: anemia in seven patients (24.1%), neutropenia in five patients (17.2%), and thrombocytopenia in two patients (6.8%). These adverse effects were easily adjusted. No one developed a secondary malignancy or hemorrhagic cystitis. CONCLUSIONS: Although PFS was less than for the MP regimen, median OS was better than for the MP regimen. Furthermore, the cyclophosphamide-prednisone regimen was well tolerated, and the adverse effects that did occur were easily adjusted. The cyclophosphamide-prednisone combination regimen may represent an effective and well tolerated first-line therapy for non-transplant candidates with MM.

Comparison of laboratory characteristics between acute promyelocytic leukemia and other subtypes of acute myeloid leukemia with disseminated intravascular coagulation

BACKGROUND: Acute promyelocytic leukemia (APL) is an acute myeloid leukemia (AML) subtype with distinctive cell morphology, molecular presentation, clinical course, and treatment. About 90% of APL patients present with hemorrhagic complications due to disseminated intravascular coagulation (DIC). When APL is suspected, all-trans retinoic acid (ATRA) treatment is recommended even before confirmation by molecular tests. Specific criteria for differentiating unconfirmed APL from other AML subtypes with DIC are currently lacking. We aimed to achieve the early diagnosis of APL from other AML types with DIC by restricting the DIC criteria. METHODS: We retrospectively analyzed 29 patients newly diagnosed with AML accompanied by DIC from January 2005 to January 2013. RESULTS: Fibrin degradation products (FDP) (77.7 microg/mL vs. 23.7 microg/mL, p=0.026), D-dimer (7,376.2 ng/mL vs. 1,315.2 ng/mL, p=0.018), and TIBC (264.4 microg/dL vs. 206.8 microg/dL, P=0.046) were higher, while fibrinogen (133.8 mg/dL vs. 373.2 mg/dL, p or =27 microg/mL, D-dimer > or =2,071 ng/mL, and fibrinogen < or =279 mg/dL were our threshold values. These markers may be characteristic to APL and helpful in presumptive diagnosis. CONCLUSION: APL may be differentiated from other AML subtypes by core markers of DIC (FDP, D-dimer, and fibrinogen). We suggest that clinicians set new diagnostic thresholds by restricting the DIC criteria. These findings support the early initiation of ATRA, prior to confirmation by PML-RARA molecular testing.

A Case of Cytomegalovirus Infection Presenting as Pericarditis and Lupus Nephritis Flare-up

Cytomegalovirus (CMV) infection is associated with an exacerbations of systemic lupus erythematosus (SLE), but the role of CMV in disease pathogenesis remains unclear. CMV infection is often severe and difficult to diagnose in patients with SLE. Only a few cases of opportunistic CMV interstitial pneumonitis infection occurring in patients with SLE after intensive immunosuppressive therapy have been described. We report a case of CMV infection presenting with massive pericarditis and aggravating lupus nephritis in a patient who did not undergo any kind of immunosuppressive therapy except for low dose steroids to treat concomitant pulmonary tuberculosis. After diagnosis, the patient was successfully treated with immunoglobulin and ganciclovir. This case may support the theory that CMV infection is a potential trigger for SLE.

A Case of Disseminated Nocardiosis by Nocardia brasiliensis after Steroid Injection

Nocardiosis in the immunocompromised host is a rare but life-threatening infection. Nocardia farcinica and N. asteroides have been reported as the most common causative pathogens of disseminated nocardiosis. It is well known that infection of N. brasiliensis can cause cutaneous nocardiosis mainly, but is rarely known to cause disseminated nocardiosis. We report a case of disseminated nocardiosis by N. brasiliensis. A 68-year-old male subject with no underlying disease developed painful swelling on both lower legs, as well as dyspnea. He was diagnosed with intramuscular abscess, pneumonia and brain abscess by N. brasiliensis. He underwent incision and drainage for intramuscular abscess, as well as antibiotic therapy, and was successfully treated.

A Case of Sensorimotor Polyneuropathy in a Patient with Sjogren's Syndrome / 대한내과학회지

Sjogren's syndrome is a chronic inflammatory disorder characterized by lymphocytic infiltration of the exocrine glands, especially the lacrimal and salivary glands. Although it is characterized primarily by a particular form of dry eyes (keratoconjunctivitis sicca) and a dry mouth (xerostomia), this condition may affect a wide variety of organ systems, including the skin, lungs, heart, and kidneys, and it may rarely have neurological manifestations. The peripheral neuropathy results from nerve damage due to vasculitis or nerve sheath demyelination, which adversely affects the ability of nerves to send messages. We report the case of a 71-year-old woman who presented with polyneuropathy of the lower limbs that was diagnosed as Sjogren's syndrome but progressed to muscle atrophy during her treatment.