Multiple myeloma (MM), a hematological malignancy, is characterized by malignant plasma cell proliferation in the bone marrow. Recent treatment advances have significantly improved patient outcomes associated with MM.In this study, we aimed to develop comprehensive, evidence-based guidelines for the diagnosis, prognosis, and treat‑ ment of MM. We identified 12 key clinical questions essential for MM management, guiding the extensive literature review and meta-analysis of the study. Our guidelines provide evidence-based recommendations by integrating patient preferences with survey data. These recommendations include current and emerging diagnostic tools, thera‑ peutic agents, and treatment strategies. By prioritizing a patient-centered approach and rigorous data analysis, these guidelines were developed to enhance MM management, both in Korea and globally.

Purpose: The role of allogeneic stem cell transplantation (alloSCT) in multiple myeloma (MM) treatment remains controversial. We conducted a retrospective, multicenter, nationwide study in Korea to evaluate the outcomes of alloSCT in Asian patients with MM. Materials and Methods: Overall, 109 patients with MM who underwent alloSCT between 2003 and 2020 were included in this study. Data were collected from the Korean Multiple Myeloma Working Party Registry. Results: The overall response rate and stringent complete response plus complete response (CR) rates were 67.0 and 46.8%, respectively, after alloSCT. At a median follow-up of 32.5 months, the 3-year probability of progression-free survival (PFS) and overall survival (OS) rates were 69.3% and 71.8%, respectively. The 3-year probabilities of OS rates in the upfront alloSCT, tandem auto-alloSCT, and later alloSCT groups were 75.0%, 88.9%, and 61.1%, respectively. Patients who achieved CR before or after alloSCT had significantly longer OS (89.8 vs. 18 months and 89.8 vs. 15.2 months, respectively). Even though patients who did not achieve CR prior to alloSCT, those who achieve CR after alloSCT had improved PFS and OS compared to those who had no achievement of CR both prior and after alloSCT. Patients who underwent alloSCT with 1-2 prior treatment lines had improved PFS (22.4 vs. 4.5 months) and OS (45.6 vs. 15.3 months) compared to those with three or more prior treatment lines. Conclusion AlloSCT may be a promising therapeutic option especially for younger, chemosensitive patients with earlier implementation from relapse.

Metformin, commonly prescribed for type 2 diabetes, is considered safe with minimal side-effect. Acute pancreatitis is rare but potentially fatal adverse side-effect of metformin. We report a patient on hemodialysis with metformin-related acute pancreatitis and lactic acidosis. A 62-year-old woman with diabetic nephropathy and hypertension presented with nausea and vomiting for a few weeks, followed by epigastric pain. At home, the therapy of 500 mg/day metformin and 50 mg/day sitagliptin was continued, despite symptoms. Laboratory investigations showed metabolic acidosis with high levels of lactate, amylase at 520 U/L (range, 30-110 U/L), and lipase at 1,250 U/L (range, 23-300 U/L). Acute pancreatitis was confirmed by computed tomography. No recognized cause of acute pancreatitis was identified. Metformin was discontinued. Treatment with insulin and intravenous fluids resulted in normalized amylase, lipase, and lactate. When she was re-exposed to sitagliptin, no symptoms were reported.
Acidosis ; Acidosis, Lactic* ; Amylases ; Diabetes Mellitus ; Diabetic Nephropathies ; Female ; Humans ; Hypertension ; Insulin ; Lactic Acid ; Lipase ; Metformin* ; Middle Aged ; Nausea ; Pancreatitis* ; Renal Dialysis* ; Sitagliptin Phosphate ; Vomiting

Polycystic kidney disease (PCKD) is the most common life-threatening genetic disease that causes kidney failure worldwide. Patients with autosomal dominant PCKD notice an increase in abdominal size as the kidney cysts grow and present with gastrointestinal and pulmonary symptoms. Surgical therapy, percutaneous drainage, sclerotherapy, cyst decompression, and laparoscopic fenestration have been used to treat the symptoms, but the results are often unsatisfactory. We recruited five patients with PCKD. Each patient complained of severe abdominal discomfort, and had a poor quality of life. In these patients, we performed renal artery embolization. After the procedure, all of the patients were discharged without severe complications. Follow-up abdominal computed tomography was performed 3-6 months after the procedure, and we were able to confirm a reduction in the size of both kidneys. In addition, the clinical symptoms improved in all five patients.
Decompression ; Drainage ; Follow-Up Studies ; Humans ; Kidney ; Polycystic Kidney Diseases* ; Quality of Life ; Renal Artery* ; Renal Insufficiency ; Sclerotherapy

Anemia is a common cause of referrals to gastroenterologists. Only a small number of anemia cases result from vascular abnormalities. Visceral artery aneurysms and pseudoaneurysms are rare forms of vascular disease that have significant potential for rupture, resulting in potentially life-threatening hemorrhaging. We present the case of a 70-year-old female patient with a pseudoaneurysm of the superior mesenteric artery complicated with rupture, who had no abdominal pain and only anemia.
Abdominal Pain ; Aged ; Anemia* ; Aneurysm ; Aneurysm, False* ; Arteries ; Female ; Humans ; Mesenteric Artery, Superior* ; Referral and Consultation ; Rupture* ; Vascular Diseases

BACKGROUND/AIMS: The gastrointestinal (GI) tract often becomes involved in patients with systemic amyloidosis. As few GI amyloidosis data have been reported, we describe the clinical features and outcomes of patients with pathologically proven GI amyloidosis. METHODS: We identified 155 patients diagnosed with systemic amyloidosis between April 1995 and April 2013. Twenty-four patients (15.5%) were diagnosed with GI amyloidosis using associated symptoms, and the diagnoses were confirmed by direct biopsy. RESULTS: Among the 24 patients, 20 (83.3%) had amyloidosis light chain (AL), three (12.5%) had amyloid A, and one (4.2%) had transthyretin-related type amyloidosis. Their median age was 57 years (range, 37 to 72), and 10 patients were female (41.7%). The most common symptoms of GI amyloidosis were diarrhea (11 patients, 45.8%), followed by anorexia (nine patients, 37.5%), weight loss, and nausea and/or vomiting (seven patients, 29.2%). The histologically confirmed GI tract site in AL amyloidosis was the stomach in 11 patients (55.0%), the colon in nine (45.0%), the rectum in seven (35.0%), and the small bowel in one (5.0%). Patients with GI involvement had a greater frequency of organ involvement (p = 0.014). Median overall survival (OS) in patients with GI involvement was shorter (7.95 months; range, 0.3 to 40.54) than in those without GI involvement (15.84 months; range, 0.0 to 114.53; p = 0.069) in a univariate analysis. A multivariate analysis of prognostic factors for AL amyloidosis revealed that GI involvement was not a significant predictor of OS (p = 0.447). CONCLUSIONS: The prognosis of patients with AL amyloidosis and GI involvement was poorer than those without GI involvement, and they presented with more organ involvement and more advanced disease than those without organ involvement.
Adult ; Aged ; Amyloid Neuropathies, Familial/*diagnosis/immunology/mortality/pathology/therapy ; Biomarkers/analysis ; Biopsy ; Female ; Gastrointestinal Diseases/*diagnosis/immunology/mortality/pathology/therapy ; Gastrointestinal Tract/immunology/*pathology ; Humans ; Immunoglobulin Heavy Chains/analysis ; Immunoglobulin Light Chains/analysis ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Multivariate Analysis ; Predictive Value of Tests ; Prognosis ; Proportional Hazards Models ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Serum Amyloid A Protein/analysis ; Time Factors

Fanconi syndrome (FS) is a rare condition that is characterized by defects in the proximal tubular function. A 48-year-old woman was admitted for evaluation of proteinuria. The patient showed normal anion gap acidosis, normoglycemic glycosuria, hypophosphatemia, and hypouricemia. Thus, her condition was compatible with FS. The M peak was found behind the beta globulin region in urine protein electrophoresis. Upon bone marrow examination, we found that 24% of cells were CD138+ plasma cells with kappa restriction. From a kidney biopsy, we found crystalline inclusions within proximal tubular epithelial cells. Thereafter, she was diagnosed with FS accompanied by multiple myeloma. The patient received chemotherapy and autologous stem cell transplantation, and obtained very good partial hematologic response. However, proximal tubular dysfunction was persistent until 1 year after autologous stem cell transplantation. In short, we report a case of FS accompanied by multiple myeloma, demonstrating crystalline inclusion in proximal tubular cells on kidney biopsy.
Acid-Base Equilibrium ; Acidosis ; Beta-Globulins ; Biopsy ; Bone Marrow Examination ; Crystallins ; Drug Therapy ; Electrophoresis ; Epithelial Cells ; Fanconi Syndrome* ; Female ; Glycosuria ; Humans ; Hypophosphatemia ; Immunoglobulin kappa-Chains ; Kidney ; Middle Aged ; Multiple Myeloma* ; Plasma Cells ; Proteinuria ; Stem Cell Transplantation

Cardiac trauma from penetrating chest injury is a life-threatening condition. It was reported that < 10% of patients arrives at the emergency department alive. Penetrating chest injury can cause serious damage in more than 1 cardiac structure, including myocardial lacerations, ventricular septal defect (VSD), fistula between aorta and right cardiac chamber and valves. The presence of pericardial effusion (even a small amount) on the initial echocardiography might be the only clue to serious cardiac damage in the absence of definite evidence of anatomical defect in heart. We here present a case, in which clear diagnosis of VSD and pseudoaneurysmal formation was delayed a few days after penetrating chest injury due to the lack of anatomical evidence of damage.
Aneurysm, False ; Aorta ; Delayed Diagnosis ; Echocardiography ; Emergencies ; Fistula ; Heart ; Heart Septal Defects, Ventricular ; Humans ; Lacerations ; Pericardial Effusion ; Thoracic Injuries ; Thorax

Topiramate is an antiepileptic drug widely used to treat various seizures, mood disorders and migraine based on its various pharmacological mechanisms. Even though nephrolithiasis is listed as one of its side effects, there have been no cases reporting nephrolithiasis caused by use of topiramate on Korean pediatric patients. Since the use of topiramate is increasing in many patients, the possibility of nephrolithiasis after the treatment needs to be considered. Here, we report our experience in correcting neprholithiasis by simply discontinuing topiramate without administering any additional treatments.
Anticonvulsants ; Fructose ; Humans ; Migraine Disorders ; Mood Disorders ; Nephrolithiasis ; Seizures

In this case report, we describe a 71-year-old woman with right conal coronary artery-to-pulmonary trunk fistula. She visited the outpatient clinic of the nephrology department for long-term management of renal dysfunction. On transthoracic echocardiography (TTE) conducted as a part of cardiac evaluation, an abnormal Doppler color flow taking a course toward echocardiographic probe was incidentally detected outside the main pulmonary trunk, giving an impression of congenital coronary arteriovenous (AV) fistula. Computed tomography coronary angiography confirmed the presence of congenital coronary AV fistula from a conal branch of the right coronary artery to the main pulmonary trunk in the form of a ground cherry. Although the direction of Doppler color flow is not usual (i.e. toward, not away from, echocardiographic probe) in this case, congenital coronary AV fistula should be in the first priority among potential diagnoses when an abnormal Doppler color flow was found near the main pulmonary trunk on TTE.
Aged ; Ambulatory Care Facilities ; Arteries ; Arteriovenous Fistula ; Coronary Angiography ; Coronary Vessel Anomalies ; Coronary Vessels ; Echocardiography ; Female ; Fistula ; Humans ; Nephrology ; Physalis