BACKGROUND: Therapeutic plasma exchange (TPE) is an effective and practical treatment for separation and removal of harmful antibodies or pathogenic substances from the blood. The volume of plasma removed must be replaced by a replacement fluid such as 4~5% albumin solution or Fresh frozen plasma (FFP). We conducted a study of coagulopathy using albumin solution and checked the chemical composition of fresh frozen plasma. METHODS: We measured pre- and post-TPE PT/aPTT for evaluation of the effect of albumin replacement on coagulation from 192 TPE sessions of 19 patients. We also investigated routine chemistry test items including glucose and electrolytes from 10 randomly selected FFP. RESULTS: The post PT and aPTT within four hours after TPE were prolonged due to a transient decrease in coagulation factors, but were normalized within 2 days after TPE. All coagulation time was corrected to the level of the pre-TPE status within four hours before the next TPE except the patients who received TPE 6 times or more. FFP showed higher level in glucose, sodium and inorganic phosphate. CONCLUSION: Albumin exchange produces temporary coagulation factor deficiency. However, this transient factor deficiency rarely causes clinical problems and the factors are rapidly corrected by redistribution and resynthesis. We should be careful about hypocalcemia, hyperglycemia, and hypernatremia when using FFP replacement.
Antibodies ; Blood Coagulation Factors ; Chemistry ; Electrolytes ; Glucose ; Humans ; Hyperglycemia ; Hypernatremia ; Hypocalcemia ; Plasma ; Plasma Exchange* ; Sodium

The Mycobacterium avium-intracellulare complex (MAC) is the most common pathogen in pulmonary disease caused by a nontuberculous mycobacteria. Patients with MAC pulmonary disease tend to be older, are more likely to have underlying lung disease than tuberculosis patients. The insidious nature of MAC pulmonary disease has been emphasized in many reports because symptoms may be present for months or years before a diagnosis can be made. Most patients experience chronic coughing, which is usually productive of purulent sputum. A MAC pulmonary infection is rarely accompanied by acute respiratory symptoms and lobar pneumonic consolidation on chest radiography. We report a very rare case of M. intracellulare pulmonary disease presenting as acute pneumonia.
Cough ; Diagnosis ; Humans ; Lung Diseases ; Mycobacterium avium Complex* ; Mycobacterium avium-intracellulare Infection ; Mycobacterium* ; Nontuberculous Mycobacteria ; Pneumonia* ; Radiography ; Sputum ; Thorax ; Tuberculosis

Thrombotic thrombocytopenic purpura is a rare but fatal complication of systemic lupus erythematosus. The diagnosis of thrombotic thrombocytopenic purpura as a syndrome distinct from systemic lupus erythematosus may be challenging particularly when thrombotic thrombocytopenic purpura is presented concomitantly with systemic lupus erythematosus. Early diagnosis and aggressive treatment including plasmapheresis would be required. However, recent reports have suggested that the use of cyclophosphamide may have a role. We describe a patient with systemic lupus erythematosus who was first presented with severe thrombotic thrombocytopenic purpura. Diagnosis was based on typical clinical features of thrombotic thrombocytopenic purpura and laboratory findings of active lupus nephritis. Renal biopsy also confirmed the coexistence of thrombotic thrombocytopenic purpura and diffuse proliferative lupus nephritis. Although prompt extensive plasmapheresis and high dose steroid therapy were performed, oliguric renal failure and thrombocytopenia persisted. After addition of cyclophosphamide to the treatment with plasmapheresis and steroid, clinical manifestations of thrombotic thrombocytopenic purpura and lupus nephritis were markedly improved.
Biopsy ; Cyclophosphamide* ; Diagnosis ; Early Diagnosis ; Humans ; Lupus Erythematosus, Systemic ; Lupus Nephritis* ; Plasmapheresis ; Purpura ; Purpura, Thrombotic Thrombocytopenic* ; Renal Insufficiency ; Thrombocytopenia

Hereditary spherocytosis (HS) is a genetic disorder characterized by the production and destruction of spherocytes due to a deficiency of red cell membrane cytoskeletal proteins, resulting in the clinical presentation of chronic hemolytic anemia. This disease can be accompanied by an aplastic crisis due to parvovirus B19 infection. Parvovirus B19 infection causes diseases such as erythema infectiosum and arthritis, and can also trigger various autoimmune diseases, including autoimmune hemolytic anemia (AIHA). Here, we report a rare case of AIHA developing 3 months after an aplastic crisis due to parvovirus B19 infection in an 11-year-old boy with HS and provide the relevant literature review.
Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune ; Arthritis ; Autoimmune Diseases ; Cell Membrane ; Child ; Cytoskeletal Proteins ; Erythema Infectiosum ; Humans ; Parvovirus ; Spherocytes ; Spherocytosis, Hereditary

Hereditary spherocytosis (HS) is a genetic disorder characterized by the production and destruction of spherocytes due to a deficiency of red cell membrane cytoskeletal proteins, resulting in the clinical presentation of chronic hemolytic anemia. This disease can be accompanied by an aplastic crisis due to parvovirus B19 infection. Parvovirus B19 infection causes diseases such as erythema infectiosum and arthritis, and can also trigger various autoimmune diseases, including autoimmune hemolytic anemia (AIHA). Here, we report a rare case of AIHA developing 3 months after an aplastic crisis due to parvovirus B19 infection in an 11-year-old boy with HS and provide the relevant literature review.
Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune ; Arthritis ; Autoimmune Diseases ; Cell Membrane ; Child ; Cytoskeletal Proteins ; Erythema Infectiosum ; Humans ; Parvovirus ; Spherocytes ; Spherocytosis, Hereditary

A Schwannoma is a benign tumor that originates from Schwann cells in the gastric wall. The tumor is mainly asymptomatic but sometimes can cause bleeding of the upper GI tract. We encountered a Schwannoma that was identified by gastroscopy as a submucosal mass that might be malignant. Therefore, surgery was considered as the primary treatment. The Schwannoma was confirmed retrospectively by a pathologic examination after excising the mass. This case report is a comparative study into Schwanoma with and without central ulceration. Pathologically, atypia of the cell was discovered in the case accompanied by an ulcer, which is a secondary phenomenon caused by the degeneration that does not indicate the malignancy.
Gastroscopy ; Hemorrhage ; Neurilemmoma* ; Retrospective Studies ; Schwann Cells ; Ulcer* ; Upper Gastrointestinal Tract

Primary hepatocellular carcinoma with metastasis to the ovary is a rare malady. To the best of our knowledge, approximately eight cases in the English literature and two cases in the Korean literature have been reported. We report here a case of hepatocellular carcinoma with metastasis to the ovary in a 40-year-old woman who presented with a month long history of lower abdominal discomfort and a palpable mass. She had been diagnosed with hepatocellular carcinoma (HCC) four years prior and subsequently underwent a several courses of transarterial chemoembolization and radio-frequency ablation. The patient had then been seen regularly and was generally well until 4 month ago, when the alpha fetoprotein (AFP) level began to rise to 425 ng/mL. After that time she had been seen and examined every month, however, the AFP level rose to 2,150 ng/mL without evidence of recurrence on the computed tomography (CT) scan of liver, the heapatic artery angiography, PET and so on. On admission, a pelvis MRI revealed mainly solid masses of both ovaries and multiple peritoneal and omental nodules. Transvaginal core biopsy was performed and the histologic examination of the specimen revealed metastatic hepatocellular carcinoma. We report here on one case of ovarian metastasis from hepatocellular carcinoma together with a brief review of the literature.
Adult ; alpha-Fetoproteins ; Angiography ; Arteries ; Biopsy ; Carcinoma, Hepatocellular* ; Female ; Humans ; Liver ; Magnetic Resonance Imaging ; Neoplasm Metastasis* ; Ovary ; Pelvis ; Recurrence

Adenomyoma is a nonneoplastic lesion that can be found anywhere in the gastrointestinal tract, but it's rarely found in the extrahepatic bile duct. To the best of our knowledge, it is a completely benign lesion, but making a clear distinction from malignancy on preoperative evaluation is very difficult. Its clinical importance mainly lies in the possibility that they may be confused with carcinoma, leading to unnecessarily extensive surgical resections. We report here on a case of distal common bile duct adenomyoma that presented with right upper quadrant abdominal pain, and the preoperative examinations could not reveal whether the tumor was benign or malignant. It was finally diagnosed by histological examination after performing pylorus preserving pancreaticoduodenectomy.
Abdominal Pain ; Adenomyoma* ; Bile Ducts, Extrahepatic ; Common Bile Duct Neoplasms ; Common Bile Duct* ; Gastrointestinal Tract ; Pancreaticoduodenectomy ; Pylorus

We report a case of an intravascular hemolytic reaction attributable to anti-Jk(b) antibodies that were not detected using an enzyme phase antibody identification test. A 61-year-old male who had received two units of red blood cells was admitted to the emergency room because his urine was dark. LISS/Coombs gel column agglutination tests suggested the presence of anti-Jk(b) and anti-E antibodies. However, his serum was negative for the Jk(b) antigen when an enzyme phase test was performed. A positive reaction was evident, however, when EDTA-treated plasma was tested; this excluded any possible complement-mediated reaction. The patient was diagnosed with an intravascular hemolytic transfusion reaction, caused by anti-Jk(b), and was later discharged without specific complications after receiving antigen-negative blood transfusions.
Agglutination Tests ; Antibodies ; Blood Group Incompatibility* ; Blood Transfusion ; Edetic Acid ; Emergency Service, Hospital ; Erythrocytes ; Humans ; Kidd Blood-Group System ; Male ; Middle Aged ; Plasma

BACKGROUND: A peripheral extracorporeal membrane oxygenator (p-ECMO) has been developed to support patients who are dying due to a serious cardiopulmonary condition. This analysis was planned to define the clinical situation in which the patient benefits most from a p-ECMO. MATERIAL AND METHODS: Between June 2007 and Aug 2009, a total of 41 adult patients used the p-ECMO. There were 23 males and 18 females (mean age 54.4+/-15.1 years). All patients had very unstable vital signs with hypoxia and complex cardiac problems. We divided the patients into 4 groups. In the first group, a p-ECMO was used as a bridge to cardiac operation. In the second group, patients did not have the opportunity to undergo any cardiac procedures; nevertheless, they were treated with a p-ECMO. In the third group, patients mostly had difficulty in weaning from CPB (cardiopulmonary bypass) after cardiac operation. The fourth group suffered from many complications, such as pneumonia, bleeding, infections, and LV dysfunction with underlying cardiac problems. All cannulations were performed by the Seldinger technique or cutting down the femoral vessel. A long venous cannula of DLP(R) (Medtronic Inc, Minneapolis, MN) or RMI(R) (Edwards Lifesciences LLC, Irvine, CA) was used together with a 17~21 Fr arterial cannula and a 21 Fr venous cannula. As a bypass pump, a Capiox emergency bypass system (EBS(R); Terumo, Tokyo, Japan) was used. We attempted to maintain a flow rate of 2.4~3.0 L/min/m2 and an activated clotting time (ACT) of around 180 seconds. RESULTS: Nine patients survived by the use of the p-ECMO. Ten patients were weaned from a p-ECMO but they did not survive, and the remainder had no chance to be weaned from the p-ECMO. The best clinical situation to apply the p-ECMO was to use it as a bridge to cardiac operation and for weaning from CPB after cardiac operation. CONCLUSION: Various clinical results were derived by p-ECMO according to the clinical situation. For the best results, early adoption of the p-ECMO for anatomical correction appears important.
Adoption ; Adult ; Anoxia ; Catheterization ; Catheters ; Emergencies ; Extracorporeal Membrane Oxygenation ; Female ; Glycosaminoglycans ; Hemorrhage ; Humans ; Male ; Membranes ; Oxygenators, Membrane ; Pneumonia ; Shock, Cardiogenic ; Tokyo ; Vital Signs ; Weaning