Gastrointestinal System Involvement Research Articles

Primary extramedullary plasmacytoma of the sigmoid colon with perforation: a case report

BackgroundExtramedullary plasmacytomas account for 4% of all plasma cell tumors and occur mainly in the upper respiratory tract; gastrointestinal system involvement is rare. Extramedullary plasmacytoma of the colon with perforation has not been reported.Case presentationA 77-year-old woman with a 1-year history of lower abdominal pain and nausea was admitted to our hospital. An abdominal computed tomography scan revealed a sigmoid tumor with perforation. The patient underwent emergency surgery. Pathological examination led to a diagnosis of plasmacytoma of the colon. The patient did not undergo postoperative adjuvant chemotherapy. She has had no recurrence in 14 months of regular follow-up.ConclusionsWe have herein described a rare case of extramedullary plasmacytoma of the gastrointestinal tract with perforation involving the sigmoid colon.

A CASE OF BEHCET 'S DISEASE WITH UPPER EXTREMITY THROMBOSIS

Behcet's disease (BD) is a multi-system vasculitis in which recurrent oral and genital ulcers, uveitis as well as musculoskeletal, neurologic and gastrointestinal system involvement are observed. In previous studies, vascular involvement rate ranges between 1-38%. Most common vascular complication is superficial vein thrombosis of the extremities, followed by deep vein thrombosis. In this study, we will present a case of BD with upper extremity thrombosis.

Five-year Retrospective Study on Primary Hyperparathyroidism in South India: Emerging Roles of Minimally Invasive Parathyroidectomy and Preoperative Localization with Methionine Positron Emission Tomography-Computed Tomography Scan.

Background:Primary hyperparathyroidism (PHPT) is a common endocrine disease with a variable clinical presentation. PHPT is usually symptomatic at presentation in majority of the patients, especially in developing countries. As the accessibility to investigations, advanced imaging methods and surgical procedures are improving, the clinical profile of the patients with PHPT has undergone a palpable change compared to the earlier description. Hence we decided to look for a change in clinical, imaging and surgical outcomes of PHPT patients from South India.Methods:We collected the data on clinical presentation, biochemistry, radiological features and operative findings of patients with PHPT treated in our hospital from 2011-2015. Cases of PHPT were identified from the laboratory values using the biochemical criteria, after the exclusion of secondary and tertiary hyperparathyroidism cases.Results:Our study identified 54 patients (19 males and 35 females) with age ranging from 16 to 71 years. A Significant proportion(38.9%) of the patients were asymptomatic. Musculoskeletal symptoms (40.7%), renal manifestations (27.7%) and gastrointestinal system involvement (27.7%) constituted the other common modes of presentation. CNS involvement was seen in 3 patients. A palpable nodule in the neck was detected in 4 patients. Interestingly 4 patients were managed for parathyroid crisis at presentation. Biochemical features included hypercalcaemia (100%) and hypophosphatemia (59%) with a mean intact PTH level of 602.0±721.3 pg/ml. Sensitivity of Ultrasonography and Tc99M Sestamibi was 72% and 70.6% respectively for detecting a parathyroid adenoma. Sensitivity of C11 methionine PET-CT was 71.4% in those patients who were negative for other imaging modalities. Forty three patients (79.6%) underwent minimally invasive parathyroidectomy.Conclusion:In South India we have a notable change in the clinical presentation of PHPT from a symptomatic to an asymptomatic state. C11 Methionine PET - CT is an emerging modality for preoperative localisation especially when other imaging modalities are negative and when a minimally invasive parathyroidectomy is desired.

Study of association of mortality with electrolyte abnormalities in children admitted in pediatric intensive care unit

Background : Electrolyte abnormalities are common in critically ill children needing admission in intensive care unit. In view of the importance of electrolyte homeostasis and its significant impact on the final outcome of the patient, the present study was undertaken. Since sodium and potassium are the electrolytes that are most frequently deranged in critically ill patients, we have studied these electrolytes in our study. Methods : The study was carried out in the Pediatric Intensive Care Unit at a tertiary care hospital. The study enrolled all the patients admitted in PICU of age 29 days to 12 years during April 2015 to September 2017. Hyponatremia and hypernatremia was defined as serum sodium concentration below 135mEq/L and above 145mEq/L respectively. Hypokalemia and hyperkalemia shall be defined as potassium level below 3.5mEq/L and above 5.5mEq/L, respectively. Results : The prevalence of electrolyte abnormality in terms of sodium or potassium abnormality in our study was 44.31% (323 out of 729). Hyponatremia (27.43%) was the most common electrolyte abnormality in our study followed by hypokalemia (13.99%). The mortality in children with electrolyte abnormality was found to be 28.8% that was higher as compared to the mortality in the group without electrolyte abnormality which was 19.7%. This association was statistically significant (p value=0.004; Odd’s ratio- 1.640; 95% confidence interval 1.169-2.322).The mortality in the patients with hyponatremia and hypernatremia was 32.5% and 38.5% respectively, which was significantly higher than the mortality in the normonatremic patients which was 19.5%, the association being statistically significant. The mortality in the group with hypokalemia and hyperkalemia was found to be 24.5% and 30.4% which was higher than the group with normokalemia which was 23.1%. However, the association was not statistically significant. The association of primary organ system involvement with the presence of electrolyte abnormality was found statistically significant. Maximum number of study participants with hyponatremia had central nervous system involvement (48.5%) and those with hypernatremia had gastrointestinal system involvement (65.4%). Hypokalemia was most frequently observed in patients with gastrointestinal involvement (54.9%) and hyperkalemia with renal involvement (34.8%). Conclusion : Our study has demonstrated significant association of electrolyte abnormalities at admission in PICU with mortality and primary organ system involvement. Close monitoring and correction of electrolyte abnormalities is essential to reduce morbidity and mortality.

Gastritis Associated with Initially Pediatric Crohn's Disease and Ulcerative Colitis.

PurposeThe aim of this study is to determine the involvement of the upper gastrointestinal system (GIS) in patients diagnosed with Crohn's disease (CD), ulcerative colitis (UC), and non-inflammatory bowel disease (IBD) and to compare their differences.MethodsThis study included patients aged between 2 and 18 years who underwent colonoscopy and esophagogastroduodenoscopy (EGD) for the first time due to the prediagnosis of IBD. In EGD, samples were taken from duodenum, antrum, corpus, and esophagus; and gastritis, duodenitis, and esophagitis were identified through histopathologic examination. The data gathered the ends of the research were compared between IBD with non-IBD groups and between CD-UC with non-IBD groups, and the presence of significant differences between groups were determined.ResultsIn our study, 16 patients were diagnosed with CD, 13 with UC, 3 with undeterminate colitis, and 13 with non-IBD. In the histopathological examination of the groups, GIS involvement was found in 94.1% of patients diagnosed with IBD and in 38.5% of non-IBD patients. Moreover, the difference was found to be statistically significant (p=0.032). No significant difference was found between the CD and UC groups. Gastritis was mostly observed in 93.8% of CD-diagnosed patients, 76.8% of UC-diagnosed patients, 81.2% of IBD-diagnosed patients, and 38.5% of non-IBD-diagnosed patients. On the other hand, significant differences were found between CD and non-IBD groups (p=0.03), UC and non-IBD groups (p=0.047), and IBD and non-IBD groups (p=0.03).ConclusionThe results of the study show that gastritis was highly observed in UC- and CD-diagnosed patients than in non-IBD-diagnosed patients.

Progress of gastrointestinal involvement in systemic sclerosis

Systemic sclerosis is an autoimmune disease characterized by skin thickening and tightness. Digestive system is the most commonly damaged system secondary to skin, and as to unspecific clinical manifestations, this complication is often ignored in the early, the prognosis and quality of life of patients will be affected. This article overviews the recent research developments on pathology, clinical features, management of systemic sclerosis with digestive system involvement, to draw attention to early diagnosis and treatment of this condition for the improvement of the prognosis. Key words: Scleroderma, systemic; Digestive system; Disease attributes; Diagnosis; Therapeutics

Crohn Hastalığının Üst Gastrointestinal Sistem Tutulumu: Tek Merkez Deneyimi

Background and Aim: The frequency of upper gastrointestinal system involvement in Crohn’s disease is 0.5%–4%. Treatment and follow-up strategies of these patients differ from those of ileocolonic disease. Here we present our patients who had Crohn’s disease with upper gastrointestinal system involvement. Materials and Methods: We retrospectively investigated our patients who had Crohn’s disease with upper gastrointestinal system involvement. Results: Upper gastrointestinal system involvement was detected in 10 patients. The median age of patients was 40.5 years (31-53 years); 6 patients were male and 4 patients were female. The median follow-up period after diagnosis was 29 (1-228) months. Four patients had isolated upper gastrointestinal system involvement, while 6 patients had ileocolonic disease. The frequency of organ involvement was 10% (1 patient) for the esophagus, 20% (2 patients) for the stomach, and 70% (7 patient) for the duodenum. Endoscopic appearances varies among patients; cobble stone appearances were detected in 2 patients, ulcers were detected in 7 patients and nodulariy were detected in 4 patients. Granulomas were detected histopathologically in 3 patients (30%). Conclusion: Complaints like epigastric pain, nausea and vomiting, and/or dysphagia must warrant clinicians to investigate the upper gastrointestinal system in Crohn’s disease. Early diagnosis and appropriate treatment are necessary to prevent complications.

Behçet's syndrome: providing integrated care.

Behçet’s syndrome (BS) is a multisystem vasculitis that presents with a variety of mucocutaneous manifestations such as oral and genital ulcers, papulopustular lesions and erythema nodosum as well as ocular, vascular, gastrointestinal and nervous system involvement. Although it occurs worldwide, it is especially prevalent in the Far East and around the Mediterranean Sea. Male gender and younger age at disease onset are associated with a more severe disease course. The management of BS depends on the severity of symptoms. If untreated, morbidity and mortality are considerably high in patients with major organ involvement. Multidisciplinary patient care is essential for the management of BS, as it is for other multisystem diseases. Rheumatologists, dermatologists, ophthalmologists, neurologists, cardiovascular surgeons and gastroenterologists are members of the multidisciplinary team. In this study, we reviewed the epidemiology, etiology, diagnostic criteria sets, clinical findings and treatment of BS and highlighted the importance of the multidisciplinary team in the management of BS.

Gastrointestinal system involvement in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multisystem disorder which can affect the gastrointestinal (GI) system. Although GI symptoms can manifest in 50% of patients with SLE, these have barely been reviewed due to difficulty in identifying different causes. This study aims to clarify clinical characteristics, diagnosis and treatment of the four major SLE-related GI system complications: protein-losing enteropathy (PLE), intestinal pseudo-obstruction (IPO), hepatic involvement and pancreatitis. It is a systematic review using MEDLINE and EMBASE databases and the major search terms were SLE, PLE, IPO, hepatitis and pancreatitis. A total of 125 articles were chosen for our study. SLE-related PLE was characterized by edema and hypoalbuminemia, with Technetium 99m labeled human albumin scintigraphy (99mTc HAS) and alpha-1-antitrypsin fecal clearance test commonly used as diagnostic test. The most common site of protein leakage was the small intestine and the least common site was the stomach. More than half of SLE-related IPO patients had ureterohydronephrosis, and sometimes they manifested as interstitial cystitis and hepatobiliary dilatation. Lupus hepatitis and SLE accompanied by autoimmune hepatitis (SLE-AIH overlap) shared similar clinical manifestations but had different autoantibodies and histopathological features, and positive anti-ribosome P antibody highly indicated the diagnosis of lupus hepatitis. Lupus pancreatitis was usually accompanied by high SLE activity with a relatively high mortality rate. Early diagnosis and timely intervention were crucial, and administration of corticosteroids and immunosuppressants was effective for most of the patients.

Clinical Predictors of Outcome in Patients with Anti-neutrophil Cytoplasmic Autoantibody-related Renal Vasculitis: Experiences from a Single-center.

Background:Primary anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a chronic autoimmune disease associated with multisystem dysfunction. Renal involvement is common and closely associated with outcome. The purpose of this study was to investigate the clinical determinants of mortality of patients with AAV-related renal injury in the first 2 years after diagnosis in a single West Chinese center.Methods:Demographic and laboratory parameters of 123 consecutive patients with AAV-related renal injury diagnosed in Renal Division and Institute of Nephrology, Sichuan Provincial People's Hospital between 2004 and 2012 were collected retrospectively. All patients were followed up for 2 years after diagnosis. Survivors were compared with nonsurvivors to identify the clinical baseline variables associated with mortality. Multivariate Cox regression model was used to determine the independent predictors of mortality.Results:Of the 123 patients, 46 (37.4%) died by the end of 2 years after diagnosis, with 41 (33.3%) patients dying within the first 12 months. In comparison with the survivors, Birmingham Vasculitis Activity Score (BVAS), the incidence of pulmonary hemorrhage and digestive system (DS) involvement, serum creatinine, and erythrocyte sedimentation rate were significantly higher in nonsurvivors, whereas lymphocyte counts, hemoglobin, and complement 3 (C3) were significantly lower. Renal replacement therapy was more common in nonsurvivors. High BVAS (hazard ratio [HR] = 1.058, 95% confidence interval [CI]: 1.002–1.117; P = 0.042), pulmonary hemorrhage (HR = 1.970, 95% CI: 1.033–3.757; P = 0.04), DS involvement (HR = 2.911, 95% CI: 1.212–6.911; P = 0.017), and serum creatinine >400 μmol/L (HR = 2.910, 95% CI: 1.271–6.664; P = 0.012) were independent predictors of death in patients with AAV-related renal injury.Conclusions:Patients with AAV-related renal injury have high early mortality. Those with high BVAS (particularly with pulmonary or DS involvement) and serious renal dysfunction should receive aggressive therapy and careful monitoring to reduce the occurrence of adverse events and improve prognosis.

Clinical differences of influenza subspecies among hospitalized children.

Clinical findings, mortality, and morbidity rates differ among influenza subspecies. Awareness of these differences will lead physicians to choose the proper diagnostic and therapeutic strategies and to foresee possible complications. The aim of this study was to evaluate the clinical differences of influenza subspecies among hospitalized children. Hospitalized children with proven influenza infection by polymerase chain reaction on nasopharyngeal swab specimens in our clinic, between December 2013 and March 2016, were enrolled. These children were divided into 3 groups as Influenza A/H1N1 (n=42), Influenza A/H3N2 (n=23), and Influenza B (n=35). The median age of the children was 51.5 months (range, 3-204 months). The most common presenting symptoms were fever (n=83), cough (n=58), and difficulty in breathing (n=25). The most common non-respiratory findings were lymphadenopathy (n=18) and gastrointestinal system involvement (n=17). Sixty-two percent of the patients (n=62) had chronic diseases. H1N1 and H3N2 were significantly more common among patients with chronic neurologic disorders and renal failure, respectively. Leukopenia (n=32) and thrombocytopenia (n=22) were the most common pathologic laboratory findings. Neutropenia, elevated CRP levels, and antibiotic use were significantly more common among patients with H1N1 infection. Seven patients were transferred to the intensive care unit with diagnoses of acute respiratory distress syndrome (n=4), encephalitis (n=2), and bronchiolitis (n=1). Two patients with chronic diseases and H1N1 infection died secondary to acute respiratory distress syndrome. Influenza A/H1N1 infection represented more severe clinical disease.

Gastrointestinal system manifestations in juvenile systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n=8) and lupus enteritis (n=1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n=3) and hepatic steatosis (n=1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n=3; associated with methotrexate and nonsteroidal anti-inflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n=1), and gastritis associated with corticosteroid (n=1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

Evaluation of Follow-up Results in Children with Henoch-Schönlein Purpura

Objective: The aim of this study was to investigate long-term follow-up results of children Henoch-Schonlein purpura (HSP). Methods: 102 children (66 boys and 36 girls) with HSP followed up at Pediatric Department of Dicle University Faculty of Medicine between 1996 and 2002 were included. Demographic, clinical and laboratory characteristics of children, treatment modalities and complications were reviewed from the follow-up charts of patients. Fourty-eigth children who came to control after a period of 40.8±19.9 months underwent a thorough clinical and laboratory work up. Clinical and laboratory findings accompanying specific organ involvements were investigated. Results: In total, 91.2% had purpuric eruption, 62.7% had gastrointestinal system (GIS) involvement, 28.4% had renal involvement and 43.1% had arthritis. Triggering factors were upper respiratory tract infection in 55%, vaccination in 2 and drugs in 2 patients. Gastrointestinal involvement was significantly more frequent in children with high immunglobulin A levels (p=0.012), renal involvement was significantly more frequent in children with high erythrocyte sedimentation rate (p=0.006). GIS involvement was more frequent in patients over 7 years (p<0.05). Significant correlations were found between renal involvement and leukocytosis, thrombocytosis and high C-reactive protein levels (p<0.05). Mean healing period of the disease was 17.89.2 days. First relapses occurred in 42 patients (41.2%) with skin eruptions and in 33 (32.4%) with abdominal discomfort. Symptoms and signs were relapsed two times in 12 patients and three times in 13. The existence of hypertension, older age, low C3 levels were found to be associated with renal involvement in logistic regression analysis (p<0.05). Conclusion: Increased IgA level and age over 7 were associated with GIS involvement, while high acute phase reactants and older age were found to be related with renal involvement. J Clin Exp Invest 2016; 7(4): 269-277.

Outcomes of high-grade gastrointestinal graft-versus-host disease posthematopoietic stem cell transplantation in children.

We explored the clinical course of acute high-grade gastrointestinal graft-versus-host disease in children in a single center. This was a retrospective analysis of 28 pediatric patients who presented with a clinical diagnosis of stage III and IV acute graft-versus-host disease (aGVHD) of the gastrointestinal system (GIS). Generally, skin involvement was the initial manifestation of aGVHD that began in the first 3 weeks of hematopoietic stem cell transplantation (HSCT); on the other hand, GIS involvement predominated after the second week of HSCT. Reported adult data show a survival rate of only 25%; however, our study showed more favorable outcomes in children with a survival rate of 55%. We monitored levels of albumin and immunoglobulin G and observed low levels overall during treatment of unresponsive patients, although only albumin levels were shown to be significantly different. We observed a significant increase in mortality with the use of antithymocyte globulin in GIS aGVHD, although antithymocyte globulin used for graft-versus-host disease prophylaxis had no demonstrable effect on GIS aGVHD mortality. Whether the significantly lower GIS aGVHD mortality among the children recruited in our study than among their historical adult counterparts is a primary result of the specific attributes of the pediatric GIS, or whether it originated from HSCT kinetics remains to be determined by future studies.

Anafilakside Riskli Grup: İnfant Anafilaksisi

Objective: The infantile group that is accepted to be at risk of anaphylaxis has a special importance since they are unable to express their complaints and also the signs and symptoms of anaphylaxis can be confused with common symptoms of infants such as drooling, vomiting, diarrhea and itching. However, early detection of the signs and symptoms of anaphylaxis is crucial for effective diagnosis and treatment. The aim of study was to draw attention to infants anaphylaxis. Materials and Methods: All of the patients in the infant group (n=15) among a total of 35 anaphylaxis cases that we followed in the Pediatric Allergy Clinic of Dr. Behcet Uz Children’s Hospital between the years 2011-2015 were included in the study. Demographic and clinical features of the patients were evaluated. Results: 9 of 15 (60%) patients involved in the study were male and 6 (40%) were female, and the average age of anaphylaxis was 8.13±6.83 (min 1-max 24) months. In 93% of cases (n=14) the trigger was food and in 7% of cases (n=1) the trigger was not determined. The most frequently responsible foods were respectively cow’s milk (67%) and egg (20%). In 7 patients (47%), there were respiratory system and skin manifestations, in 7 patients (47%) 3 system involvement and in 1 patient respiratory and gastrointestinal system involvement has been observed. In only 3 cases (20%) the blood pressure was measured and also epinephrine was administered to only 3 cases (20%) in the hospital. In only 3 of the patients (20%), an epinephrine injector was prescribed after anaphylaxis. Conclusion: It was detected that the most common triggers are cow’s milk and egg, the subjective symptoms are frequently overlooked, an atopic nature is a risk factor and physicians’ rates of blood pressure measurement, epinephrine administration and EpiPen autoinjector prescription are low.

Cardiopulmonary and Gastrointestinal Manifestations of Eosinophil- associated Diseases and Idiopathic Hypereosinophilic Syndromes: Multimodality Imaging Approach.

Eosinophil-associated diseases (EADs) are a diverse group of disorders characterized by an increase in circulating or tissue eosinophils. Cardiopulmonary and gastrointestinal system involvement can be due to primary EAD with no known cause or can be secondary to known systemic disease. The cardiopulmonary spectrum of EADs comprises simple pulmonary eosinophilia, acute eosinophilic pneumonia, chronic eosinophilic pneumonia, Churg-Strauss syndrome, allergic bronchopulmonary aspergillosis, bronchocentric granulomatosis, parasitic infections, and idiopathic hypereosinophilic syndrome. Eosinophilic gastrointestinal disorders include eosinophilic esophagitis, eosinophilic gastroenteritis, and eosinophilic colitis. Diagnosis is often challenging and requires a combination of clinical and imaging features along with laboratory findings. The absolute eosinophil count in peripheral blood and the percentage of eosinophils in bronchoalveolar lavage fluid are crucial in evaluation of various eosinophilic lung diseases. Although chest radiography is the initial imaging modality used in suspected cases of pulmonary EAD, multidetector computed tomography may demonstrate more characteristic pulmonary patterns, nodules, and subtle parenchymal abnormalities. Barium esophagography is used to assess mucosal abnormalities and the length and diameter of esophageal strictures. Magnetic resonance imaging is superior in providing valuable information in select patients, especially in evaluation of cardiac and gastrointestinal system involvement. Many patients require a multimodality imaging approach to enable diagnosis, guide treatment, and assess treatment response. Knowledge of the clinical features and imaging findings of the spectrum of EADs involving the lungs, heart, and gastrointestinal tract permits optimal patient care.

Behçet's disease in children, an overview.

SummaryBD is a systemic inflammatory disease with a variable vasculitis. Paediatric onset is very rare and carries a strong genetic component. Oral ulcers and fever of unknown origin are frequent at onset and difficult to distinguish from other inflammatory disorders; therefore, expert opinion is still mandatory to recognize the disease early. An international expert consensus has recently proposed new classification criteria for children with BD. The clinical spectrum of BD is heterogeneous and influenced by gender, ethnicity and country of residence. Young males have the worst prognosis with significantly more frequent neurological, ocular and vascular involvement. BD treatment is aimed at alleviating inflammation. Among all drugs, TNFα inhibitors have become a standard to control severe ocular, neurological and digestive system involvement.

Difficult diagnosis of invasive fungal infection predominantly involving the lower gastrointestinal tract in acute lymphoblastic leukaemia

Invasive fungal infections are most commonly seen in immunocompromised patients and usually affect the respiratory system. Gastrointestinal system involvement of mucormycosis and invasive aspergillosis is rarely reported in childhood. Here we describe a 5 year old boy with acute lymphoblastic leukaemia who developed invasive fungal infection particularly affecting the lower gastrointestinal system to emphasise the difficulties in diagnosis and management of invasive fungal infections in immunocompromised patients.

Risk factors for digestive system involvement in systemic lupus erythematosus

Risk factors for digestive system involvement in systemic lupus erythematosus

Clinical Characteristics and Treatment Outcome of Chinese Patients With Systemic Amyloid Light-Chain Amyloidosis: A Retrospective Single-Center Analysis.

Amyloid light-chain (AL) amyloidosis is a disease caused by tissue deposition of light-chain proteins in vital organs that results in progressive organ damage. We analyzed the clinical characteristics of 123 AL amyloidosis patients and performed an overall survival (OS) analysis to identify critical baseline factors. Patients (median age, 54 years) were diagnosed with organ involvement of kidney (98.4%), gastrointestinal (73%), cardiac (56%), liver (13%), or nervous system (10%), and multiorgan involvement was observed in 91% of patients. Treatment regimens of transplantation, bortezomib plus dexamethasone, melphalan plus dexamethasone, and prednisone-based regimens or no treatment resulted in 3-year OS rates of 72%, 60%, 55%, and 41%, respectively. Median OS was 38 months and was affected by age (≥ 65 years), hypoalbuminemia, renal failure, heart involvement, and organ response time (within 3 months). Multivariate analysis indicated that these were independent prognostic factors on OS except for age. The AL amyloidosis patients in this study presented somewhat different features and outcomes compared to others, with younger age and higher rates of organ involvement.