ABSTRACT
Myeloproliferative neoplasms (MPNs) are clonal disorders of hematopoietic stem cells. The malignant clones produce cytokines that drive self-perpetuating inflammatory responses and tend to transform into more aggressive clones, leading to disease progression. The progression of MPNs follows a biological sequence from the early phases of malignancy, polycythemia vera, and essential thrombocythemia, to advanced myelofibrosis and leukemic transformation. To date, the treatment of MPNs has focused on preventing thrombosis by decreasing blood cell counts and relieving disease-related symptoms. However, interferon (IFN) has been used to treat MPNs because of its ability to attack cancer cells directly and modulate the immune system. IFN also has the potential to modulate diseases by inhibiting JAK2 mutations, and recent studies have demonstrated clinical and molecular improvements. Long-acting IFN is administered less frequently and has fewer adverse effects than conventional IFN. The current state of research on long-acting IFN in patients with MPNs is discussed, along with future directions.
ABSTRACT
Castleman’s disease is a rare non-neoplastic lymphoproliferative disorder of unknown origin. It is classified into unicentric or multicentric based on its anatomical distribution. Multicentric Castleman’s disease can be subdivided according to the presence of human herpesvirus-8 (HHV-8) infection. Castleman’s disease has a rare incidence, and HHV-8-positive multicentric Castleman’s disease is even rarer. There are several types of natural course for this disease, and the rapidly progressing type can lead to death within a few weeks, emphasizing the need for prompt diagnosis and treatment. We report a recent case from Korea, presenting with multiple lymphadenopathies, confirmed as HHV-8-positive multicentric Castleman’s disease through biopsy, and achieving complete response with rituximab monotherapy.
ABSTRACT
Myeloproliferative neoplasms (MPNs) are clonal disorders of hematopoietic stem cells;these include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). MPNs are inflammatory cancers, wherein the malignant clone generates cytokines that sustain the inflammatory drive in a self-perpetuating vicious cycle. The course of MPNs follows a biological continuum, that is, from early cancer stages (ET/PV) to advanced myelofibrosis as well as impending leukemic transformation. MPN-related symptoms, e.g., fatigue, general weakness, and itching, are caused by inflammatory cytokines. Thrombosis and bleeding are also exacerbated by inflammatory cytokines in patients with MPN. Until recently, the primary objective of ET and PV therapy was to increase survival rates by preventing thrombosis. However, several medications have recently demonstrated the ability to modify the course of the disease; symptom relief is expected for most patients. In addition, there is increasing interest in the active treatment of patients at low risk with PV and ET. This review focuses on the ET/PV treatment strategies as well as novel treatment options for clinical development.
ABSTRACT
Objective@#Bone marrow (BM) examinations are performed to evaluate hematological abnormalities. Focusing on patients with cytopenia, we aimed to determine the circumstances under which a BM examination can assist in the diagnosis of hematologic diseases. @*Methods@#The medical records of 738 patients who underwent BM examination from March 2011 to March 2019 at Soonchunhyang University Seoul Hospital were reviewed. In total, 234 patients underwent a BM examination to identify the cause of cytopenia. Excluded from the analysis were BM examinations performed to diagnose specific diseases and evaluate disease status. @*Results@#Results suggesting suboptimal outcome (n=6) or BM invasion of solid tumors (n=13) were excluded. Immune thrombocytopenic purpura patients (n=52) with normal BM examination results were also excluded. One hundred sixty-three patients who underwent BM examination to determine the cause of cytopenia were included in the analysis. A comparison of non-specific results (n=56) to those pointing to an underlying hematologic disease (n=107) showed that patients with severe neutropenia or severe thrombocytopenia were more likely to be diagnosed with a hematologic disease. Specifically, as the number of severe cytopenias increased, the likelihood of a hematologic disease diagnosis was significantly augmented. Patients with end-stage renal disease, autoimmune disease, or liver cirrhosis were more likely to receive non-specific results. @*Conclusion@#In conclusion, seeking the underlying disease or drug should be a primary target for patients with cytopenia. In cases of severe cytopenia in more than one lineage, BM examination should be strongly considered to diagnose an underlying hematologic disease.
ABSTRACT
Objective@#Eosinophilia in patients on hemodialysis has already been reported. It has been associated with allergy to dialyzers and exaggerated activation of complement during hemodialysis. Its etiology, however, remains unknown. In addition, there are not enough studies on eosinophilia in patients on hemodialysis in Korea. Therefore, we performed this retrospective study to find out the prevalence and possible etiologic factors of blood eosinophilia in patients undergoing hemodialysis. @*Methods@#Between January 2013 to December 2015, the patients hospitalized for hemodialysis at Soonchunhyang University Hospital and National Health Insurance Service Medical Center (Ilsan Hospital) were included in this study. Eosinophilia was defined when absolute eosinophil count was greater than 500/μL, respectively. We retrospectively reviewed the medical records of patients about parasite infection, other malignancies, and history of kidney transplantation. @*Results@#Of the 2,155 patients hospitalized for hemodialysis at two centers, 1,057 patients (49%) were found to have eosinophilia. We investigated 1,199 patients’ information (Soonchunhyang University Hospital) by the medical records. Two hundred two patients (16.8%) had no identifiable and/or possible causes. Only two patients complained of symptoms such as itching. Steroids were administered to control symptoms, and both patients had normal eosinophil levels, and steroids were discontinued. Other patients did not complain of specific symptoms associated with eosinophilia and did not take medication such as steroids. Eosinophilia was improved in 49% of patients without special treatments. @*Conclusion@#We found that the eosinophil counts in patients with end stage renal disease on hemodialysis were frequently elevated. However, in most cases, eosinophilia was not clinically relevant.
ABSTRACT
Background/Aims@#A better understanding of cancer cell biology has led to the discovery and development of several new targeted agents for cancer. These drugs are widely used in cancer treatment and have good toxicity profiles. However, some patients are extremely sensitive to these drugs and can develop severe toxicities. Among the toxicities, pulmonary complications are infrequent with most targeted therapies. This study aimed to identify the radiologic pulmonary complications in various targeted therapies and to analyze the characteristics of patients with pulmonary toxicity. @*Methods@#We retrospectively reviewed the medical records and chest image findings of 644 patients who were treated with targeted antineoplastic agents at Soonchunhyang University Hospital between May 2005 and September 2014. @*Results@#Of these 644 patients, 90 (14.0%) developed pulmonary complications as noted on chest computed tomography. Among these patients, 15 (2.3%) developed drug-related pulmonary toxicities. Treatment with targeted agents was discontinued in all patients, while 11 patients were simultaneously treated with glucocorticoids. Three patients died of drug-related pulmonary toxicity. @*Conclusions@#During targeted therapy, clinicians should assess for pulmonary toxicities and symptoms that occur with dyspnea. If drug-induced pulmonary toxicities are suspected, imaging studies should be performed immediately, and the possibility of variable radiological patterns should be considered. Discontinuing the use of implicated causative agents and treatment with glucocorticoids resulted in an improvement in both symptoms and imaging findings, but some patients still experienced fatal pulmonary toxicities.
ABSTRACT
Myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell malignancies.Chronic inflammation and a dysregulated immune system are central to the pathogenesis and progression of MPNs. Interferon alpha (IFNα) was first used for the treatment of MPNs approximately 40 years ago. It has significant antiviral effects and plays a role in anti-proliferative, pro-apoptotic, and immunomodulatory responses. IFNα is an effective drug that can simultaneously induce significant rates of clinical, hematological, molecular, and histopathological responses, suggesting that the disease may be cured in some patients. However, its frequent dosage and toxicity profile are major barriers to its widespread use. Pegylated IFNα (peg-IFNα), and more recently, ropeginterferon alpha-2b (ropeg-IFNα-2b), are expected to overcome these drawbacks. The objective of this article is to discuss the clinical role of IFNα in Philadelphia-negative MPNs through a review of recent studies. In particular, it is expected that new IFNs, such as peg-IFNα and ropeg-IFNα-2b, with lower rates of discontinuation due to fewer adverse effects, will play important clinical roles.
ABSTRACT
Background/Aims@#A better understanding of cancer cell biology has led to the discovery and development of several new targeted agents for cancer. These drugs are widely used in cancer treatment and have good toxicity profiles. However, some patients are extremely sensitive to these drugs and can develop severe toxicities. Among the toxicities, pulmonary complications are infrequent with most targeted therapies. This study aimed to identify the radiologic pulmonary complications in various targeted therapies and to analyze the characteristics of patients with pulmonary toxicity. @*Methods@#We retrospectively reviewed the medical records and chest image findings of 644 patients who were treated with targeted antineoplastic agents at Soonchunhyang University Hospital between May 2005 and September 2014. @*Results@#Of these 644 patients, 90 (14.0%) developed pulmonary complications as noted on chest computed tomography. Among these patients, 15 (2.3%) developed drug-related pulmonary toxicities. Treatment with targeted agents was discontinued in all patients, while 11 patients were simultaneously treated with glucocorticoids. Three patients died of drug-related pulmonary toxicity. @*Conclusions@#During targeted therapy, clinicians should assess for pulmonary toxicities and symptoms that occur with dyspnea. If drug-induced pulmonary toxicities are suspected, imaging studies should be performed immediately, and the possibility of variable radiological patterns should be considered. Discontinuing the use of implicated causative agents and treatment with glucocorticoids resulted in an improvement in both symptoms and imaging findings, but some patients still experienced fatal pulmonary toxicities.
ABSTRACT
Myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell malignancies.Chronic inflammation and a dysregulated immune system are central to the pathogenesis and progression of MPNs. Interferon alpha (IFNα) was first used for the treatment of MPNs approximately 40 years ago. It has significant antiviral effects and plays a role in anti-proliferative, pro-apoptotic, and immunomodulatory responses. IFNα is an effective drug that can simultaneously induce significant rates of clinical, hematological, molecular, and histopathological responses, suggesting that the disease may be cured in some patients. However, its frequent dosage and toxicity profile are major barriers to its widespread use. Pegylated IFNα (peg-IFNα), and more recently, ropeginterferon alpha-2b (ropeg-IFNα-2b), are expected to overcome these drawbacks. The objective of this article is to discuss the clinical role of IFNα in Philadelphia-negative MPNs through a review of recent studies. In particular, it is expected that new IFNs, such as peg-IFNα and ropeg-IFNα-2b, with lower rates of discontinuation due to fewer adverse effects, will play important clinical roles.
ABSTRACT
Myeloproliferative neoplasms (MPNs) are classified as chronic myeloid leukemia (CML) and Philadelphia chromosome-negative MPN. In MPN cases, the presence of a BCR-ABL1 translocation with a coexisting mutation is exceptionally rare. Herein, we report the first documented patient with CML harboring CALR mutation in Korea. A 33-year-old woman was referred to our hospital in February 2015 with splenomegaly, leukocytosis, and thrombocytosis. She was diagnosed with CML and started receiving nilotinib. In October 2015, a major molecular response was observed, but thrombocytosis persisted. A repeat bone marrow (BM) examination revealed no specific findings. However, as thrombocytosis worsened, we changed nilotinib to dasatinib. In May 2019, owing to persistent thrombocytosis, we repeated the BM examination and found CALR mutation (15.97%) on the MPN–next generation sequencing (NGS) test. We then retrospectively performed repeat MPN-NGS testing using the BM aspirate sample obtained in 2015 and found CALR mutation (10.64%).
ABSTRACT
OBJECTIVE@#To prevent invasive fungal disease (IFD) in acute myeloid leukemia (AML) patients, the use of posaconazole as a prophylactic antifungal agent has become standard in patients undergoing induction chemotherapy. However, there are few data comparing itraconazole and posaconazole as prophylactic antifungal agents in the real world.@*METHODS@#Patients at the Soonchunhyang University Seoul Hospital, who were treated with itraconazole or posaconazole for preventing IFD during induction chemotherapy for AML from January 2009 to April 2018, were included in the study. The collected clinical data were reviewed, and IFD was diagnosed using the revised definition of IFD from the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group.@*RESULTS@#A total of 53 patients were recruited to receive either posaconazole (n=29) or itraconazole (n=24). IFD occurred in seven patients (29.1%) who used posaconazole and in six patients (20.6%) who used itraconazole for antifungal prophylaxis (P=0.475). The 100-day mortality rate was 4 (13.8%) in the posaconazole group and 2 (8.3%) in the itraconazole group (P=0.535).@*CONCLUSION@#There was no significant difference in the incidence of IFD and 100-day mortality between the patients with induction chemotherapy for newly diagnosed AML who received posaconazole and itraconazole as prophylactic antifungal agents. These results suggest that it would be worthwhile to ascertain whether posaconazole is widely known as a better approach than itraconazole as prophylactic antifungal agents in the real-world.
ABSTRACT
BACKGROUND: Pancreatic cancer is among the most common malignancies associated with venous thromboembolism (VTE). Asian patients are known to have a lower incidence of VTE compared to Caucasian patients. However, few studies have investigated the incidence of VTE in Asian patients with pancreatic cancer. METHODS: This retrospective review of medical records was performed on 505 patients with histopathologically proven advanced stage pancreatic cancer, from January 2006 to December 2012, at Soonchunhyang University Hospitals. RESULTS: Ninety-four patients (18.6%) had at least one pulmonary embolism (PE), deep vein thrombosis (DVT), or splanchnic vein thrombosis (SVT); 38 patients had isolated SVT; and 56 patients (11.1%) had at least one classic VTE (PE and/or DVT of lower extremities). Patients with more advanced stages of pancreatic cancer (distant metastatic stage, recurrence) or who had received chemotherapy had a higher incidence of classic VTE. Patients who were simultaneously diagnosed with pancreatic cancer and classic VTE had a poorer prognosis than patients with subsequent VTEs. There was a significant difference in overall survival (OS) between the presence and absence of a concurrent classic VTE diagnosis (median: OS, 2.1 mo vs. 10.7 mo; P < 0.001). Even when VTE included SVT, the result was similar (P < 0.001). CONCLUSION: In Korean patients with advanced pancreatic cancer, the incidence of VTEs is comparable to that of Caucasian patients. We also found that pancreatic cancer patients with concurrent VTEs had a poor prognosis compared to patients who developed VTEs later.
Subject(s)
Humans , Asian People , Diagnosis , Drug Therapy , Hospitals, University , Incidence , Medical Records , Pancreatic Neoplasms , Prognosis , Pulmonary Embolism , Retrospective Studies , Thrombosis , Veins , Venous Thromboembolism , Venous ThrombosisABSTRACT
The classic presentation of renal cell carcinoma (pain, hematuria, and flank mass) occurs in a minority of patients and often is indicative of advanced disease. Common sites of metastatic renal cell carcinoma are lung, soft tissues, bone, and liver. Paranasal sinus is an unusual site for metastasis of renal cell carcinoma. One 73-year-old male presented to Soonchunhyang University Seoul Hospital with melena. Renal cell carcinoma with metastasis to duodenum was diagnosed by computed tomography (CT). He underwent right radical nephrectomy and Whipple's operation. Positron emission tomography/CT was performed postoperatively, and then metastasis to maxillary sinus was found by accident. He was treated with molecular targeted therapy (pazopanib hydrochloride 800 mg).
Subject(s)
Aged , Humans , Male , Bone and Bones , Carcinoma, Renal Cell , Duodenum , Electrons , Hematuria , Liver , Lung , Maxillary Sinus , Melena , Molecular Targeted Therapy , Neoplasm Metastasis , Nephrectomy , SeoulABSTRACT
BK virus nephropathy has emerged as an important cause of renal allograft dysfunction. Reduction in immunosuppression is the mainstay of BK virus nephropathy treatment. However, decreasing immunosuppressive medications is not sufficient for treatment of BK virus nephropathy. Therefore, there is a need for other treatment strategies such as cidofovir, leflunomide, and intravenous immunoglobulin in combination with immunosuppression reduction. Ciprofloxacin has recently been reported to have antiviral activity and decrease BK viral load in kidney transplant recipients. These findings suggest that the use of ciprofloxacin represents a valuable treatment strategy in patients with BK virus nephropathy. Here, we report on our experience with three patients who developed presumptive BK virus nephropathy after kidney transplantation, who, after 2 months of ciprofloxacin treatment, showed disappearance of BK viremia and improvement in the estimated glomerular filtration rate. Ciprofloxacin may be considered an effective treatment option for BK viremia in kidney transplant recipients.
Subject(s)
Humans , Allografts , BK Virus , Ciprofloxacin , Glomerular Filtration Rate , Immunoglobulins , Immunosuppression Therapy , Kidney Transplantation , Kidney , Transplantation , Viral Load , ViremiaABSTRACT
Although the prognosis of papillary thyroid cancer (PTC) is extremely good, locoregional recurrences after initial treatment occur. Thyroglobulin (Tg) is a reliable tumor marker to detect recurrence or persistence of PTC. However, occasionally serum Tg may miss the detection of a recurrence. We report a 54-year-old female presented with hoarseness due to cervical recurrence without concomitant elevation of serum Tg and anti-Tg antibody, in contrast to extremely increased needle-washout Tg, who had undergone a total thyroidectomy and radioiodine ablation as initial therapies for PTC. Several factors causing such discrepancy between needle-washout Tg and serum Tg can be suggested including site of recurrence, volume of tumor, interference by some kind of plasma antibodies other than anti-Tg antibody, and any conformational defect of Tg protein. Among them, the most convincing explanation is that any conformational defect of Tg may lead to impaired secretion of Tg to blood. We suggest that more studies are needed to find the cause for potential mechanisms involved in PTC recurrences without increased serum Tg.
Subject(s)
Female , Humans , Middle Aged , Antibodies , Hoarseness , Plasma , Prognosis , Recurrence , Thyroglobulin , Thyroid Neoplasms , ThyroidectomyABSTRACT
Hepatic portal venous gas (HPVG) is a rare disease presenting as acute abdomen. The presence of the air in the portal vein has been associated with a mortality rate of more than 75%. Because of high mortality rate, most HPVG requires emergent surgical interventions and intensive medical management. HPVG is most commonly caused by mesenteric ischemia but may have a variety other causes. Clostridium perfringens is the most common pathogen of gas forming bacteria that can cause of HPVG, but Clostridium perfringens blood stream infection with HPVG is not yet reported in Korea. We experienced a case of HPVG caused by Clostridium perfringens blood stream infection at mesenteric venous hemangioma with portal hypertension due to mesenteric arteriovenous malformation.
Subject(s)
Humans , Abdomen, Acute , Arteriovenous Malformations , Bacteria , Clostridium perfringens , Hemangioma , Hypertension, Portal , Ischemia , Korea , Mortality , Portal Vein , Rare Diseases , RiversABSTRACT
Herbal remedies and health foods are widely used, and their side effects have been reported. Glucosamine is a naturally occurring amino-monosaccharide and a safe health food; rarely, however, it can cause cholestatic and hepatocellular hepatitis. We describe a case of drug-induced autoimmune hepatitis after ingestion of glucosamine. A middle-aged woman who had no history of liver disease complained of jaundice after taking glucosamine. The diagnosis of drug-induced acute autoimmune hepatitis was made using the Roussel Uclaf Causality Assessment Method score based on the patient's history and laboratory data, and percutaneous liver biopsy. After supportive care and administering prednisolone and azathiprine, the patient showed rapid improvement in clinical symptoms and laboratory findings.
Subject(s)
Female , Humans , Biopsy , Diagnosis , Chemical and Drug Induced Liver Injury , Eating , Glucosamine , Food, Organic , Hepatitis , Hepatitis, Autoimmune , History , Jaundice , Liver , Liver Diseases , PrednisoloneABSTRACT
Herbal remedies and health foods are widely used, and their side effects have been reported. Glucosamine is a naturally occurring amino-monosaccharide and a safe health food; rarely, however, it can cause cholestatic and hepatocellular hepatitis. We describe a case of drug-induced autoimmune hepatitis after ingestion of glucosamine. A middle-aged woman who had no history of liver disease complained of jaundice after taking glucosamine. The diagnosis of drug-induced acute autoimmune hepatitis was made using the Roussel Uclaf Causality Assessment Method score based on the patient's history and laboratory data, and percutaneous liver biopsy. After supportive care and administering prednisolone and azathiprine, the patient showed rapid improvement in clinical symptoms and laboratory findings.