Hemorrhagic characters and hemostatic disorders have been investigated in 106 children with acute leukemia at the National Hospital of Pediatrics from September 1st 2002 to August 30th 2004. Results showed that: bleeding signs on admission were found in 49.1%. With subcutaneous hemorrhages, petechie and bruise in skin were most common, and with mucosa hemorrhages, epistaxis and alveolar hemorrhage were more common but gastrointestinal mucosa hemorrhages and urinary hemorrhages were rare. Thrombocytopenia, prolonged APTT, hypofibrinogenemia, hypoprothrombinemia, and disseminated intravascular coagulation accounted for 91.5%, 47.2%, 23.6%, 18.9% and 2.8%, respectively. These disorders were often combined together. Hemorrhagic status related closely with thrombocytopenia. Prolonged APTT, decreased prothrombine rate, and hypofibrinogenemia were contributed to increase of hemorrhagic rate.
Hemostatic Disorders ; Child ; Leukemia

Multiple myeloma is a monoclonal plasma cell proliferation disorder with various symptoms and signs caused by paraproteinemias. Among these signs, a bleeding tendency is one of the major fatal causes. However, significant severe bleeding is rare in most cases. In this study, we report a case of multiple myeloma in a patient who had a severe recurrent bleeding tendency due to platelet dysfunction caused by paraproteins. After being treated with therapeutic plasma exchange and chemotherapy, the patient's monoclonal protein level decreased and the bleeding stopped.
Blood Platelets ; Hemorrhage ; Hemostatic Disorders ; Humans ; Multiple Myeloma ; Paraproteinemias ; Paraproteins ; Plasma Cells ; Plasma Exchange ; Plasmapheresis ; Platelet Function Tests

Background: Open heart surgery with cardiopulmonary bypass (CPB) causes haemostatic abnormalities which result in postoperative excessive bleeding. Objectives: To investigate haemostatic disorders before - after CPB and postoperative bleeding. Subjects and methods: Sixty congenital cardiac patients with and without cyanosis were recruited consecutively. Hematology and coagulation tests were done 1 day before operation, 15 minutes after protamine administration, 2 and 6 hours after the operation in the intensive care unit (lCU). Mediastinal chest tube drainage (MCTO) was measured for the first 6h in the ICU. Results: Significant differences between 2 groups could be found for red blood cells, hemoglobin, hematocrit, fibrinogen, D-dimer, fibrinogen degradation products (FOP), von Kaulla and platelet aggregation to epinephrine before operation (p < 0.05). There were not significant differences in platelets, PT, APTT, platelet aggregation to adenosine diphosphate (AOP) in the acyanotic and cyanotic patients (p > 0.05). Most hematology parameters were decreased significantly and hemostasis measurements were prolonged after operation in both groups (p < 0.05). There was also the significant difference in MCTO between 2 groups (p < 0.05). Conclusion: Coagulation, hemostasis and fibrinolysis disorders; thrombocytopenia and acquired transient platelet dysfunction may be responsible for bleeding complications after CBP \r\n', u'\r\n', u'
Heart Defects ; Congenital/ pathology ; epidemiology ; Hemostatic Disorders/ pathology ; diagnosis

Osteogenesis imperfecta is one of the groups of hereditary disorders of connective tissue which includes the Ehlers-Danlos syndrome, the Marfan syndrome, pseudoxanthoma elasticum, and Hurler syndrome. While cardiovascular involvement is associated with each of these disorders, it is least common in osteogenesis imperfecta and is overshadowed by the bony, ocular, otologic, cutaneous, and dental manifestations that are characteristic of the disorder. In evaluating patients with osteogenesis imperfecta, careful attention should be paid to cardiovascular findings and if valvular lesions are noted, patients should be instructed regarding the need for antibiotic prophylaxis for dental and surgical procedures. We report a case of osteogenesis imperfecta associated with aortic regurgitation.
Antibiotic Prophylaxis ; Aortic Valve Insufficiency* ; Connective Tissue ; Ehlers-Danlos Syndrome ; Humans ; Marfan Syndrome ; Mucopolysaccharidosis I ; Osteogenesis Imperfecta* ; Osteogenesis* ; Pseudoxanthoma Elasticum

Gray platelet syndrome (GPS) is one of primary hemostatic disorders with characteristics of moderate bleeding tendency, thrombocytopenia, gray platelet on Wright-Giemsa stained smear and absence of platelet -granule. It is known to be mostly inherited by autosomal dominance but not all. We report a case of gray platelet syndrome diagnosed in a woman with bleeding tendency such as easy bruise and evaluate clinical usefulness of mean platelet component (MPC), mean platelet volume (MPV) and platelet component distribution width (PCDW) using ADVIA 120 (Bayer Diagnostics, NY, USA).
Blood Platelets ; Contusions ; Female ; Gray Platelet Syndrome* ; Hemorrhage ; Hemostatic Disorders ; Humans ; Mean Platelet Volume ; Thrombocytopenia

Spinal subdural hematoma (SSDH) is an extremely uncommon condition. Causative factors include trauma, anticoagulant drug administration, hemostatic disorders, and vascular disorders such as arteriovenous malformations and lumbar punctures. Of SSDH cases, those that do not have any traumatic event can be considered cases of nontraumatic acute spinal subdural hematoma, which is known to have diverse clinical progress. Treatment typically consists of surgical decompression and cases in which the condition is relieved with conservative treatment are rarely reported. We report two nontraumatic acute spinal subdural hematoma patients who were successfully treated without surgery.
Arteriovenous Malformations ; Decompression, Surgical ; Hematoma, Subdural, Spinal* ; Hemostatic Disorders ; Humans ; Spinal Puncture

The biocompatibility of dialysis membranes has significantly reduced adverse responses to dialysis, such that nowadays they are rarely reported. We report the case of a patient diagnosed and subsequently treated for thrombocytopenia caused by a dialysis reaction, as an example of a hemostatic disorder mistaken for an immature arteriovenous fistula. The peridialysis pattern of the platelet count helped to confirm the diagnosis. Further studies of the negative effects of dialysis are needed, including risk factors, predictors, treatment, and prevention.
Arteriovenous Fistula ; Diagnosis ; Dialysis ; Hemostatic Disorders ; Humans ; Membranes ; Platelet Count ; Renal Dialysis ; Risk Factors ; Thrombocytopenia

Intraoperative massive bleeding requires fluid therapy and blood transfusion. But transfusion may elicit infection, hemolytic reaction, hemostatic disorder and other complication. Individuals often produce antibodies to the alleles which lack in ABO and Rh system. Such antibodies are responsible for the most serious reaction to transfusions. Antibodies may occur "naturally" or in response to sensitization from a previous transfusion or pregnancy. We report a case of severe anemic patient who had Anti Ce due to previous transfusion and was not transfused for several hours because of incompatible cross-matching. His Rh phenotype is revealed cDE.
Alleles ; Anemia* ; Antibodies ; Blood Transfusion ; Fluid Therapy ; Hemorrhage ; Hemostatic Disorders ; Humans ; Phenotype ; Pregnancy

M protein is often expressed in multiple myeloma and also can be detected in several lymphoma such as Waldenstrm macroglobulinaemia. M protein level can reflect the malignant degree and even genetic abnormality of multiple myeloma and lymphoma to some extent to predict the progress of the diseases, and the therapeutic response and prognosis of the disease can be evaluated by monitoring the M protein level and its change degree. This article reviews the role of M protein in the progression and prognosis of multiple myeloma and lymphoma, and discusses the differences in M protein expression between multiple myeloma and lymphoma, in order to provide new insights for clinical diagnosis, monitoring and evaluation of therapeutic effect.
Humans ; Lymphoma ; Multiple Myeloma/pathology* ; Prognosis ; Waldenstrom Macroglobulinemia/pathology*

Diseases of the peripheral nervous system are one of the most difficult subjects in neurology partly in that specific etiologies are often not found. Among peripheral neuropathies of unknown causes, an association of monoclonal gammopathies has been observed. Monoclonal gammopathies are disorders characterized by proliferation of a single clone of plasma cell that produces monoclonal proteins. Here we report two cases of chronic sensorimotor polyneuropathy with monoclonal gummopathy of undetermined significance, who showed no evidences of multiple myeloma, macroglobulinemia, systemic amyloidosis, or other neoplasms. One case is also associated with hyperthyroidism and cryoglobulinemia.
Amyloidosis ; Clone Cells ; Cryoglobulinemia ; Hyperthyroidism ; Monoclonal Gammopathy of Undetermined Significance* ; Multiple Myeloma ; Neurology ; Paraproteinemias* ; Peripheral Nervous System ; Peripheral Nervous System Diseases ; Plasma Cells ; Polyneuropathies* ; Waldenstrom Macroglobulinemia