No abstract available.
Anemia* ; Diagnosis*

The Korean venous thromboembolism (VTE) registry, which was initiated by the Working Parties of Korean Society on Thrombosis and Hemostasis, and the Korean Society of Hematology, is a web-based multicenter registry (http://kdvt.chamc.co.kr) for recruiting consecutive VTE patients. The aim of the registry is to prospectively collect data on the epidemiology and clinical outcomes of VTE from a large, unselected cohort of patients, and to provide data on the true incidence and management of VTE in the real-world. By the end of 2007, the starting year of the registry, 840 patients were registered. By the end of 2008, 1,121 were registered, with 1,289 by the end of 2009, and 1,463 by April 2010 from 11 hospitals. The first report on the epidemiologic characteristics of 596 consecutive VTE patients was released in October 2007.
Cohort Studies ; Female ; Humans ; Incidence ; Male ; Middle Aged ; *Registries ; Republic of Korea ; Risk Factors ; Venous Thromboembolism/*epidemiology

No abstract available.
Diagnosis, Differential* ; Hemorrhage*

Acquired hemophilia A (AHA) is a bleeding disorder caused by the development of an auto-antibody against endogenous factor VIII (FVIII). In this study, the epitope of the autoantibody was identified in a 67-year-old female patient with AHA. A prolonged activated partial thromboplastin time (77.4 s) that failed to correct in an incubation mixing test (68.2 s), a decreased FVIII activity, and a high FVIII inhibitor (14.6 Bethesda units/mL) were observed. Enzyme-linked immunosorbent assay demonstrated that the antibody belonged to the immunoglobulin G4 subclass. An immunoblotting assay revealed the light chain (A3/C1/C2 domain) of FVIII as the binding region of the antibody. The bleeding experienced by our patient resulted from the interference of FVIII binding to both FIX by anti-A3 antibodies and phospholipids and von Willebrand factor by anti-C2 antibodies. To the best of our knowledge, this is the first study in Korea characterizing an autoantibody in the context of AHA.
Antibodies ; Enzyme-Linked Immunosorbent Assay ; Factor VIII ; Female ; Hemophilia A ; Hemorrhage ; Humans ; Immunoblotting ; Immunoglobulins ; Korea ; Light ; Partial Thromboplastin Time ; Phospholipids ; von Willebrand Factor

Acquired hemophilia A (AHA) is a bleeding disorder caused by the development of an auto-antibody against endogenous factor VIII (FVIII). In this study, the epitope of the autoantibody was identified in a 67-year-old female patient with AHA. A prolonged activated partial thromboplastin time (77.4 s) that failed to correct in an incubation mixing test (68.2 s), a decreased FVIII activity, and a high FVIII inhibitor (14.6 Bethesda units/mL) were observed. Enzyme-linked immunosorbent assay demonstrated that the antibody belonged to the immunoglobulin G4 subclass. An immunoblotting assay revealed the light chain (A3/C1/C2 domain) of FVIII as the binding region of the antibody. The bleeding experienced by our patient resulted from the interference of FVIII binding to both FIX by anti-A3 antibodies and phospholipids and von Willebrand factor by anti-C2 antibodies. To the best of our knowledge, this is the first study in Korea characterizing an autoantibody in the context of AHA.
Antibodies ; Enzyme-Linked Immunosorbent Assay ; Factor VIII ; Female ; Hemophilia A ; Hemorrhage ; Humans ; Immunoblotting ; Immunoglobulins ; Korea ; Light ; Partial Thromboplastin Time ; Phospholipids ; von Willebrand Factor

Pneumatosis intestinalis (PI) is a rare condition characterized by multiple pneumocysts in the submucosa or subserosa of the bowel. Here, we report a rare case of asymptomatic PI after chemotherapy induction in an 18-yr-old man with B lymphoblastic leukemia with recurrent genetic abnormalities. The patient was treated conservatively and recovered without complications. The possibility of PI should be considered as a complication during or after chemotherapy for hematologic malignancies. Conservative treatment should be considered unless there are complications, including peritonitis, bowel perforation, and severe sepsis.
Adolescent ; Hematologic Neoplasms ; Humans ; Peritonitis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Sepsis

Glanzmann's thrombasthenia (GT) is a rare inherited platelet disorder, which is characterized by a complete lack of platelet aggregation due to a deficiency or abnormality of the membrane glycoprotein IIb/IIIa complex. Anti-GPIIb/IIIa antibodies have also been identified to cause platelet dysfunction in patients with a normal platelet count, but this has only been rarely encountered. The condition is also known as acquired GT. Herein, we describe a patient with acquired GT and a history of Evans' syndrome, who presented with severe bleeding and platelet dysfunction, but with a normal platelet count and GP IIb/IIIa expression.
Antibodies ; Blood Platelets ; Hemorrhage ; Humans ; Membrane Glycoproteins ; Platelet Aggregation ; Platelet Count ; Thrombasthenia*

Granulocytic sarcoma is a localized tumor composed of immature cells of the granulocytic series. Most granulocytic sarcomas occur in the course of acute leukemia and the blast crisis of chronic leukemia. Rarely, however, it may present before leukemia becomes clinically apparent. It may also occur in patients with myeloproliferative disorders. It has been reported that it occurs in 3% to 9% of patients with acute myelogenous leukemia (AML) and the incidence of granulocytic sarcoma is reported to be higher in patients with t (8;21). However, epidural granulocytic sarcoma associated with t (8;21) is very rare. In this report, we describe a patient with AML associated with t (8;21) in whom the cord compression occurred due to epidural granulocytic sarcoma. In addition, this case present infiltration of both pleura by blast cells. She was treated with local irradiation and chemotherapy successfully.
Blast Crisis ; Drug Therapy ; Humans ; Incidence ; Leukemia ; Leukemia, Myeloid, Acute* ; Myeloproliferative Disorders ; Pleura ; Sarcoma, Myeloid* ; Spine* ; Sternum*

OBJECTIVE: Previous studies have suggested that hyperhomocysteinemia and methylenetetrahydrofolate reductase (MTHFR C677T) mutations are associated with increased risk of recurrent spontaneous abortion (RSA). Recently, a second site polymorphism in MTHFR, 1298A-->C, which changes a glutamic acid into an alanine residue, was shown to be associated with a decreased enzyme activity. We tested whether the variant alleles of MTHFR C677T and A1298C are risk factor (biomarker) for RSA. MATERIALS AND METHODS: We analyzed DNA from a case-control study in the Korean DNA was extracted from blood samples of 118 patients with RSA and 123 healthy fertile patients as the controls. MTHFR variant alleles were determined by a PCR-restriction fragment length polymorphism assay. RESULTS: We found no evidence for an association between 677TT genotype and risk of RSA (OR=1.95, 95% CI=0.84~4.50, p=0.12). However, the MTHFR 1298AC (OR=0.36, 95% CI=0.20~ 0.63, p=0.0004) and 1298AC+CC (OR=0.35, 95% CI=0.20~0.61, p=0.0002) genotypes were lower among 118 RSA cases compared with 123 controls, conferring a 2.8-fold decrease in risk of RSA, respectively. Moreover, the combined genotypes of MTHFR 677CC/1298AC (OR=0.30, 95% CI= 0.10~0.88, p=0.029) and 677CT/1298AC (OR=0.77, 95% CI=0.60~0.99, p=0.043) also showed significantly lower risk than those with MTHFR 677CC/1298AA type. CONCLUSION: MTHFR 1298AC, MTHFR 677CC/1298AC and 677CT/1298AC genotypes may represent genetic markers for the protection of RSA at least in Korean women.
Abortion, Spontaneous* ; Alanine ; Alleles ; Case-Control Studies ; DNA ; Female ; Genetic Markers ; Genotype ; Glutamic Acid ; Humans ; Hyperhomocysteinemia ; Methylenetetrahydrofolate Reductase (NADPH2) ; Oxidoreductases* ; Pregnancy ; Risk Factors ; Vascular Diseases

In Behcet's disease(BD), there is a marked increase in vascular complication. Venous thrombosis is a major feature of the disease, although arterial thrombosis is rarely described. In Behcet's disease, thrombosis occurs in 20 to 30% of patients. We present two cases of Behcet's disease admitted to our hospital whose chief complaint was progressive increases in swelling and pain in their legs. In the first case, routine coagulation tests and sero-immunological tests were within normal limits, however, protein C and S activity were significantly decreased in the patient. So these findings suggest that auto-immune acquired protein S deficiency may be involved in the pathogenesis of thrombotic events in BD.
Humans ; Leg ; Protein C ; Protein S Deficiency ; Thrombosis ; Venous Thrombosis*