Specific Acquired Factor Deficiencies

Alpha2 antiplasmin deficiency most commonly occurs in DIC and acute promyelocytic leukemia. As discussed in chapter 27, rare patients with excessive bleeding and low levels of alpha2 antiplasmin may benefit from antifibrinolytic therapy. Rare cases of acquired alpha2 antiplasmin deficiency associated with severe bleeding have been reported in amyloidosis. Plasminogen activator inhibitor-1 deficiency has infrequently been reported in amyloidoses. Hypofibrinogenemia is most commonly seen in liver...

Tests of Hemostasis and Thrombosis

A routine laboratory test once well-established is often slavishly adhered to, with little further thought about how it originated, why it is done or what it means. To justify it, the phrases for the record and for protection are often heard. Tests done only for these reasons not only are generally a waste of time and money but can also be quite misleading, and may give to the physician a false sense of security, or produce worry and concern over a potentially serious disorder when no disease...

Table 272 Coagulation defects associated with paraproteins

Monoclonal antibodies inhibiting coagulation factors 2. Monoclonal antibodies inhibiting platelet receptors 3. Monoclonal antibodies inhibiting fibrin formation 4. Amyloid deposits adsorbing factor X 5. Amyloid deposits adsorbing alpha2 antiplasmin Dysproteinemia, the abnormal production of immunoglobulin, can affect many steps of the coagulation system and lead to severe bleeding. Multiple coagulation abnormalities have been described in patients with dysproteinemias (Table 27.2). First, the...

Who Gets APLA

Approximately 30-50 of patients with SLE will have APLA. The antibodies can also be found in patients with other autoimmune diseases. Patients without lupus or other autoimmune disease can have symptomatic APLA (Primary APLA Syndrome). Children will often develop transient non-thrombotic APLA after viral infections. This laboratory finding often comes to attention during pre-operative evaluation for tonsillectomy. Up to 30 of patients with HIV infection will also develop APLA. The...

Table 194 APLA diagnosis

Principles of demonstrating lupus inhibitor 1. Prolongation of coagulation based test 2. Failure of correction in 50 50 mix 3. Correction of abnormal test with addition of phospholipid Coagulation-Based Tests for Lupus Inhibitor Dilute Russell viper venom time Hexagonal phospholipid assay and persistence. Given the prevalence of low-titer APLA the significance of these is unknown. One approach is to test for the presence of anti-p2glycoprotein (anti-p2gp) in these patients if they have...

Other Inherited Bleeding Disorders

Inherited platelet defects fall into two general categories congenital defects in platelet function, and the inherited thrombocytopenic disorders. These syndromes cause varying degrees of platelet-type bleeding ranging from minor to severe. Most patients with inherited disorders of platelet function have normal platelet counts. These patients present with signs of platelet-type bleeding such as nosebleeds and easy bruisability. These patients tend to be only mildly symptomatic and may have...

Table 271 Initial evaluation and management of patients with APL

Obtain baseline PT-INR, aPTT, platelet count, fibrinogen, D-dimer. 2. Based on laboratories, replace using following goals - PT-INR lt 2.0 and aPTT gt 1.5 x normal two units of fresh frozen plasma. - platelets under 50,000 l give one plateletpheresis unit or 6-8 platelet concentrates. - fibrinogen under 100 mg dl give 10 units of cryoprecipitate. 3. If coagulation defects are initially present, follow laboratory every six hours. 4. If patient required over 40 units of cryoprecipitate in 12...

Table 173 Evaluation of patients with hypercoagulable states

Activated protein C resistance ratio factor V Leiden Prothrombin gene mutation PCR assay Protein C activity assay Protein S activity assay Antithrombin III activity assay Homocysteine level Antiphospholipid antibody assays Anticardiolipin antibodies Hexagonal phospholipid assay Dilute Russell viper venom time Dysfibrinogenemia evaluation Fibrinogen activity level Fibrinogen antigen level Thrombin time Endogenous erythroid colony assay Limited evaluation for cancer The timing of the laboratory...

The Situations

Patients under 40 and with no other risk factors including a negative family history of deep venous thrombosis or patients undergoing procedures less then 30 minutes long do not need prophylaxis. Patients over 40 years of age undergoing operations over 30 minutes long and with no other risk factors should receive low dose heparin. Heparin, 5,000 units TID, is started 2-8 hours before surgery and given until the patient is ambulatory. Compression stockings or LMW heparin dalteparin 2500 - 5000...

Table 73 Differential diagnosis of isolated thrombocytopenia

Amegakaryocytic thrombocytopenia Carbamazepine Alcohol Sequestration Immune destruction Idiopathic Drug-induced HIV Sepsis Post-transfusion purpura Heparin-induced thrombocytopenia Non-immune destruction Thrombotic microangiopathies Microangiopathic hemolytic anemia DIC reduced platelet production. Late-stage HIV patients may have prolonged thromb-ocytopenia with pyrimethamine and other medicines. Rare patients with autoimmune amegakaryocytic thrombocytopenia may present with severe...