Some problems in patients with hemostasis disorder

Posted by Specialist Doctor II Pham Tien Ngoc - Emergency Doctor - Emergency Department - Vinmec Central Park International General Hospital

Hemostasis disorder is a dangerous disease with many different causes and different clinical manifestations. Each cause will have different specific treatment and supportive measures. Therefore, learning about the problems on patients with hemostasis disorder will help doctors know the medical history and have the right treatment, limiting the risk of complications.

The doctor needs to find out about the patient's history of hemostasis disorder:
The patient has umbilical cord bleeding at birth, bleeding in many places on the body, bleeding early in childhood or later. In addition, there are other cases of bleeding in the same family, bleeding at the site: skin, mucous membranes, lungs, digestive, urinary, genital, without showing local lesions. Unexplained bleeding. The times and amount of blood transfusion (day, quantity) mainly after surgery or procedure, or miscarriage, postpartum,... Bleeding after tooth extraction or baby tooth loss Bleeding 15 minutes after intravenous injection Intensity of anemia after surgery, number of bleeding times

There are 5 clinical features that lead to bleeding, specifically:
How to start: The patient bleeds spontaneously and repeatedly, with no obvious traumatic cause, or just a minor trauma (concussion) light touch, intramuscular injection,...) Bleeding site: The patient has bleeding in many places, which can be both in the skin and in the mucosa, with or without external bleeding. In addition, bleeding can also be at a single place but with a special location such as: joint hemorrhage, neonatal umbilical cord bleeding. Clinical features: ecchymosis of the skin or mucous membranes Recurrence in one patient Similar case in the family

The main factors involved in blood clotting are as follows:
Factor 1 (fibrinogen): established in the liver (in case of liver disease, reducing fibrinogen in circulating blood, preventing blood clotting). Factor II (prothrombin): unstable protein, can cleave smaller platelets (thrombin, produced by the liver) affecting blood clotting, inhibiting blood clotting. Factor III (thromboplastin): replaces platelet phospholipids (participates in exogenous coagulation, also has anti-infective effects. Factor IV (Ca 2+) Factor V (proaccelerin): inactivates when Ca 2 deficiency occurs. + Factor VII (proconvertin): plasma activity is retained on the asbestos filter. Factor VIII (anti-hemophilic A): synthesis of liver, spleen and in the reticuloendothelial system, inactivation in the absence of Ca 2+ (synthesized by many genes in different chromosomes) Factor IX (antihemophilic B) Factor X (Stuart): in plasma inactive form, used in endogenous coagulation, vice versa weak Factor X is lost when tissue thromboplastin is donated during exogenous coagulation. Factor XI: initiates endogenous coagulation Factor XII: activates the coagulation system Factor XIII: fibrin stabilizer

The inhibitors of coagulation are as follows:
Protein C: vitamin K dependent serine protease is activated by thrombin to APC (activated protein C) which degrades factors Va, VIIIa, lacking in quantity or quality of blood clotting factors. one of the above anticoagulants causes hypercoagulability, (e.g., V Leiden or high levels of factor VIII cause hypercoagulability) Antithrombin III defect Factor V mutation Leiden Protein S: plays a role in supporting protein C in inactivation Two important factors are activated factors V and VIII. TFPI (tissue factor pathway inhibitor): inhibits factor VIIa-related activation of factors IX and X after activation of this activation Antiphospholipid antibodies Hyperhomocysteinemia

5.1 Antithrombin Antithrombin is antithrombin, preventing the conversion of fibrinogen to fibrin.
5.2. Heparin Produced by mast cells and basophils, acts:
Inhibits the formation of prothrombinase Inhibits the effect of thrombin Promotes the interaction between thrombin and antithrombin, causing thrombin to become inactive Anticoagulant effect lasting 3-4 hours, then heparin is destroyed by the enzyme heparinaz in the blood or phagocytosed. 5.3. Antithromboplastin Antithromboplastin is available in normal human blood, but in high concentrations in the blood of patients with hemophilia.
5.4 Sodium citrate Dissolves in blood, combines Ca 2+ into an undissociated complex, preventing the action of Ca 2+ in the chain of clotting reactions
5.5. Potassium oxalate Combines Ca 2+ to precipitate calcium oxalate causing blood to not clot
5.6. Dicoumarin Dicoumarin is similar to vitamin K, competes with vitamin K, and prevents the formation of factors (II, VII, IX, X) by the liver. Dicoumarin only works in the body.
5.7. High concentration salt solutions High concentration salt solutions (NaCL, Na2so4...)
5.8. Other methods Other methods such as: vitamin K, fresh plasma, coagulation factors, protamine sulfate, fresh tissue sections,... can also be used.

Some features that distinguish primary hemostasis and plasma coagulation disorders are explained in the following image:

Each certain situation of hemostasis disorder has its own way of management. General principles of medical management of hemostatic disorders are:
Cut off the sources of coagulation disorders: anticoagulants, antiplatelet drugs, anti-inflammatory drugs, infections, DIC,. .. Alternative treatment: blood transfusion and deficiency of clotting factors In order to properly assess the patient's blood clotting status, doctors will assign you to perform tests to evaluate clotting factors blood. This helps the patient avoid risk factors for the disease.