Coagulation

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Coagulation disorders are those who affects your blood's ability to clot. It decreases the blood’s ability to clot. As a result, bleeding lasts longer than usual.

HAEMOPHILIA:

HAEMOPHILIA

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Factor VIII & IX deficiencies are the most common severe inherited disorders. In mid 20 th century whole blood or plasma used for Rx of it.

pathophysiology:

pathophysiology

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Management of Acquired Hemophilia in the Emergency Department  Acquired hemophilia A is a rare but potentially life-threatening autoimmune bleeding disorder that results from the development of autoantibodies that are directed against, and interfere with, the activity of clotting Factor VIII. Inhibition of Factor VIII, which functions as a cofactor for Factor IXa in the enzymatic activation of Factor X, leads to a reduction in the generation of thrombin on the surface of activated platelets. Patients who develop autoantibodies against Factor VIII may present with severe and sometimes catastrophic bleeding episodes, despite having no previous history of a bleeding diathesis. Spontaneous ecchymoses, retroperitoneal and cerebral hemorrhages, gross hematuria, muscle bleeding, and intractable epistaxis are common in the patients with acquired hemophilia. This pattern of bleeding is in contrast to patients with hemophilia A, which is more often characterized by bleeding into the joints or soft tissues. Retrospective studies of patients with acquired hemophilia have reported mortality rates between 8% and 22%. A recent 2-year study of all patients with acquired hemophilia A in the United Kingdom (n=172) found that bleeding was the cause of death in 9% of the patients. Please note that the courses are accredited only for physicians (MD, DO, or equivalent). All other participants receive a certificate of completion. Available Courses A 54-Year-Old Woman With Rheumatoid Arthritis, Bruising, Swelling, and Pain

VON WILLE BRAND’S DISEASE:

VON WILLE BRAND’S DISEASE

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It is a family of bleeding disorders caused by an abnormality of VWF. most common hereditary bleeding disorder. autosomal dominant Here – 1. a platelet disorder 2. low or abnormal VWF (either quantitative or qualitative) 3. secondary reduction of plasma VIII level

pathophysiology:

pathophysiology VWF is a large multimeric glycoprotein, synthesized in platelets and endothelial cells & stored in alpha granules & weilbel Palade bodies respectively. VWF acts as carrier protein for FVIII & also required for normal platelet adhesion. In primary hemostasis: VWF attaches to platelets by its specific rc to GP IB on platelet surface and acts as an adhesive bridge between platelets and damaged subendothelial matrix at site of vascular damage. In secondary hemostasis: it protects FVIII from degradation and delivers it to the site of injury.

Causes :

Causes Congential: If there is low or defective vWF, platelets cannot properly grip onto injured area and bleeding does not stop quickly. Acquired von Willebrand disease: 1. patients with autoantibodies . 2. aortic valve stenosis , leading to GI bleeding ( Heyde's syndrome ). 3. Wilms' tumour , 4. hypothyroidism 5. mesenchymal dysplasias.

Classification:

Classification A. hereditary /congenital/ inherited : depending on qualitative and quantitative defects of vWF Tble: type 1:autosomal dominant trait 70-80% partial quantitative decrease of qualitatively normal VWF and FVIII. mild clinical symptoms reduction in VWF activity, VWF antigen, and FVIII exists. type 2: 15-20% qualitative defects of VWF. either autosomal dominant or recessive. Of the 5 known type 2 VWD subtypes (ie, 2A, 2B, 2C, 2M, 2N), type 2A VWD is by far the most common. Type 2A VWD autosomal dominant trait and is characterized by normal-to-reduced plasma levels of factor VIIIc (FVIIIc) and VWF. Analysis of VWF multimers reveals a relative reduction in intermediate and high molecular weight multimer complexes. The multimeric abnormalities are commonly the result of in vivo proteolytic degradation of the VWF. The ristocetin cofactor activity is greatly reduced, and the platelet VWF reveals multimeric abnormalities similar to those found in plasma. Type 2B VWD also is inherited as an autosomal dominant trait. This type is characterized by a reduction in the proportion of high molecular weight VWF multimers, while the proportion of low molecular weight fragments are increased. Patients with type 2B VWD have a hemostatic defect caused by a qualitatively abnormal VWF and intermittent thrombocytopenia. The abnormal VWF has an increased affinity for platelet glycoprotein Ib. The platelet count may fall further during pregnancy, in association with surgical procedures, or after the administration of desmopressin acetate (DDAVP). Although some investigators found DDAVP to be clinically useful in persons with type 2B VWD, studies directed at excluding the 2B variant should be completed before DDAVP is used therapeutically. Measurements of FVIIIc and VWF in plasma are variable; however, studies involving the use of titered doses of ristocetin reveal that aggregation of normal platelets is enhanced and induced by unusually small amounts of the drug. In patients with the rare type 2M VWD, laboratory results are similar to those of certain patients with type 2A VWD. Type 2M VWD is characterized by a decreased platelet-directed function that is not due to a decrease of high–molecular weight multimers. Laboratory findings show decreased VWF activity, but VWF antigen, FVIII, and multimer analysis are found to be within reference range. Type 2N VWD is also rare and is characterized by a markedly decreased affinity of VWF for FVIII, resulting in FVIII levels reduced to usually around 5% of the reference range. Other VWF laboratory parameters (ie, VWF antigen [VWF:Ag], ristocetin cofactor activity) are usually normal. The FVIII-binding defect in these patients is inherited in an autosomal recessive manner. Evaluate patients with FVIII deficiency and a bleeding disorder that is not clearly transmitted as an X-linked disorder or those who respond incompletely to hemophilia A therapy for type 2N VWD. Unfortunately, the confirmatory test for type 2N VWD is not routinely available, likely resulting in an underestimate of the true frequency of this subtype. type 3 most severe marked deficiencies of both VWF and FVIIIc in the plasma, absence of VWF from both platelets and endothelial cells, and a lack of response to DDAVP. severe clinical bleeding autosomal recessive trait. Consanguinity is common. Less severe clinical and laboratory abnormalities B. acquired vWD

Clinical manifestation :

Clinical manifestation Race: not associate Sex: M:F equal Age: any age History: mostly asymptomatic. Diagnosed during routine preoperative screening (eg, prolonged BT). There may be H/O followings: Increased or easy bruising Recurrent epistaxis Menorrhagia

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positive family H/O a bleeding diathesis Bleeding from wounds Gingival bleeding Postpartum bleeding Postoperative bleeding (particularly after tonsillectomy or dental extractions) interference with clotting after taking aspirin or similar medications . Physical exam: Normal or may be present with: -- Increased bruises Mucosal bleeding

Chart with inv and type:

Chart with inv and type

Treatment:

Treatment Rx depends on type & severity of vWD. Even needed only before a surgical or dental procedure Treatment to controls bleeding may include: Desmopressin —in mild vWD 1 -- Nasal spray (Stimate) -- Injection (dDAVP) b) IV infusions: Factor VIII conc. vWF Cryoprecipitate c) Birth control pills— to control heavy menstrual periods in vWD 1. d) Antifibrinolytic e) High dose IV gamma globulin & anti vWf Ab.

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Risk Factors: having family members with it Genetics: chromosome 12 Prevention: no guidelines available. Genetic counseling

DISSEMINATE INTRAVASCULAR COAGULATION:

DISSEMINATE INTRAVASCULAR COAGULATION (CONSUMPTION COAGULOPATHY )