HEMOSTASIS

  1. GENERAL:

 Hemostasis is the formation of a solid clot from fluid blood while maintaining blood flow through a
damaged vessel, resulting in repair.  There are four components that contribute to this healing are the blood vessels themselves, platelets, the coagulation system and the fibrinolytic system.

Signs of abnormalities seen in animals with bleeding disorders include:

  • Prolonged bleeding from minor trauma or surgery

  • Petechiae, ecchymoses and purpura

  • Hematomas and hemarthroses

  • Nonspecific bleeding, such as epistaxis, melena, hematemesis and hematuria

 II          PHYSIOLOGY

 The blood vessels act to prevent bleeding by three mechanisms—vasocontriction, diversion and activation.  A transient vasospasm, especially in the capillaries, is the first response of a blood vessel to injury.  Blood is diverted from the injured vessel to collateral circulation, where the vessels are intact.

 The inner surface of normal vasculature is smooth and coated with anticoagulant factors.  When the endothelium is damaged, collagen fibers are exposed and thromboplastin and vonWillebrand factor leak from the injured cells.

 Activation occurs due to the contact of platelets with these collagen fibers and the released vonWillebrand factor.  The coagulation system is activated when blood proteins contact tromboplastin leaking from damaged tissue cells.

Platelets (thrombocytes) are cellular fragments of megakaryocytes, mature cells located in the bone marrow.  As these cytoplasmic fragments are released, they enter the circulation, where their mean survival time is approximately ten days.   Mammalian platelets, like erythrocytes, are anucleate.  The cytoplasm is pale blood and the cells are approximately one to four microns in diameter (although platelets the size of an erythrocyte can be seen).  Platelets vary in shape and are usually round or oval.  The cell margins, however, are not well defined and tails are frequently seen.

The function of the platelet is primary hemostasis—the formation of a platelet plug.  This is initiated by adhesion.  The platelets adhere to the site of injury, in response to contact with collagen and vonWillebrand factor.  These activated platelets aggregate, attracted to each other and sticking together.  The cell membranes dissolve and viscous metamorphosis occurs, as a fragile jellylike plug is formed (imagine sneezing right after a nosebleed stops—it’s the platelet plug that comes out). 

This platelet mass is the substrate on which the coagulation system reacts to form a fibrin clot (scab).  

The coagulation system is a series of enzymatic reactions that results in the formation of an insoluble fibrin mesh around the platelets.  This series of reactions is called the coagulation cascade.  Most of the coagulation factors are protein-based enzymes that circulate in inactive form. 

The coagulation factors were numbers by order of discovery, not by their place in the coagulation cascade.  Don’t learn the names of these factors, but for information, they are:

      Factor I                              fibrinogen
Factor II                             prothrombin
Factor III                            tissue thromboplastin
Factor IV                            calcium ion
Factor V                             proaccelerin
Factor VII                           proconvertin
Factor VIII                          a-hemophiliac factor
Factor VIIIa                        vonWillebrand factor
Factor IX                            Christmas factor
Factor X                             Stuart factor
Factor XII                           Hageman factor
Factor XIII                           fibrin stabilizing factor

vonWillebrand factor (factor VIIIa) is part of factor VIII, but functions independently of the coagulation cascade.  It is an adhesive protein produced by the endothelial cells and the megakaryocytes.  It facilitates the attachments of platelets to collagen fibers.

 There are three coagulation pathways, two that initiate coagulation (the intrinsic and extrinsic pathways) and the common pathway that completes the fibrin clot formation.  The intrinsic pathway is initiated by the contact of blood components with abnormal endothelial surfaces (i.e. collagen fibers).  This is a slow process and generally takes several minutes to complete.  The extrinsic pathway is very rapid, completing its action within seconds.  This occurs when shed blood contact thromboplastin in the tissues surrounding the damaged blood vessel.  The intrinsic and extrinsic pathways merge to form the common pathway.  This part of the cascade converts fibrinogen to fibrin, forming a protective scab.

Coagulation must be followed by fibrinolysis—the breakdown of fibrin—or we (and our animals) would be giant walking scabs.  The fibrinolytic system replaces fibrin with collagen, re-establishing the normal channel.  As fibrinolysis occur, fibrin degradation products are formed, coating platelets and interfering with further clotting.

  

III         LABORATORY EVALUATION OF BLEEDING DISORDERS

 Sample collection requires scrupulous cleanliness and attention to detail.  The animal must be handled gently and minimal trauma is essential.  One successful attempt into a large blood vessel with minimal venous occlusion is mandatory.  A new plastic syringe should be used for collection, with the first few drops discarded.  Remember that coagulation is initiated by damage to the blood vessel and surrounding tissue—blood collection puts holes in the blood vessel and overlying tissue, causing damage. 

 Sodium citrate is the anticoagulant of choice.  It has a bright blue top.  Plastic tubes are preferred over glass tubes, because plastic inhibits the coagulation cascade.  Plasma should be harvested within thirty minutes of collection and, if shipped, should be with dry ice.

 Inadequate platelet numbers (thrombocytopenia) is the most common acquired coagulopathy in small animal practice, and it is important to be able to accurately assess platelets.  The reference range for platelets in all species is 200,000 – 500,000/ul.  It is easy to estimate platelet numbers and the results are fairly accurate an animal’s platelet count is relatively normal.  All animals should have three to thirty platelets per oil immersion field.

A manual platelet count can be obtained by using Unopette® 5855.  The accuracy and precision are only fair, however.  Generally, automatic blood cell counters are a more accurate method to enumerate platelets, but you should check your machine.

Bleeding time is a method that evaluates both the quantity and function of platelets.  The protocol requires the incision of the buccal mucosa.  The incision should be blotted every 30 – 60 seconds without actually touching the skin.  If there are sufficient platelets that are capable of forming a platelet plug, clotting should occur in one to five minutes.

 Some automatic blood cell counters can determine the mean platelet volume (MPV).  Like the MCV, this measures the volume of the average platelet.  Platelets that have recently been released from the bone marrow are larger than other platelets.  Some machines will also calculate the platelet distribution width (PDW). 

 A decrease in the number of functional platelets is called thrombocytopenia.  Hemorrhage is usually not associated with thrombocytopenia, especially if the condition is chronic.  Clinical signs may occur if the platelet count drops below 30,000/ul. 

 Bone marrow hypoplasia may result in a thrombocytopenia.  Immune-mediated thrombocytopenia (IMTP) may occur; it is rarely the primary problem.  It is often idiopathic, but it may also be associated with infections organisms such as rickettsias, immune-mediate hemolytic anemia, drug-induced (many drugs have been implicated, including prednisolone), and viral infections (such as parvovirus and the retroviruses).  Clinical signs of IMTP are usually minor, although lethargy, anorexia, depression and petechia can occur.  Diagnosis is usually due to exclusion of all other possibilities.  Transfusion of platelets is ineffective for IMTP, as the transfused platelets are destroyed within three or four hours. 

 Thrombocytopathia occurs when there are sufficient numbers of platelets but they are unable to function properly.  These often result in bleeding and are frequently associated with vonWillebrand factor deficiency or viral infections.  Transfusion of platelets is generally the best treatment.

 There are several tests that can be performed to evaluate the coagulation system.  Regardless of the test selected, controls should always be run and used to evaluate the results. 

 The intrinsic coagulation pathway can be evaluated in the clinic with three tests.  The whole blood clotting time (WBCT) is an insensitive (thrombocytopenia can affect the results) but simple screening test.  Blood is placed in a test tube and gently rocked every 30 – 60 seconds.  Dog blood should clot within 2 – 10 minutes and horse blood clots in 4 – 15 minutes. 

 The activated coagulation time (ACT®) test is a modification of the WBCT using silica in a blood collection tube.  This is a simple and rapid test.  Like the WBCT, it is affected by thrombocytopenia and it is not diagnostic if the blood clots WNL (you must perform the following test).

 The activated partial thromboplastin time (APTT) is the most sensitive and specific in-clinic test for the intrinsic coagulation system.  Unlike the WBCT and ACT tests, it is independent of thrombocytopenia.  The patient results are compared to the control; clotting time more than 25% lover than the control is considered abnormal.  The APTT in the dog is 17 – 35 seconds.

The most common in-clinic test for the extrinsic coagulation pathway is prothrombin time (PT).  Like the APTT, it is independent of thrombocytopenia and the results should be within 25% of the control results.  The dog’s APTT averages 7 – 10 second.

Commercial assays exist for all of the factors and fibrin degradation products (FDP).

                                    

IV         HEMOSTATIC DISORDERS

 There are a number of important hemostatic disorders—there are many things that can go wrong.  The disorders can be classified as acquired or hereditary.

Thrombocytopenia is the most common acquired hemostatic disorder of small animals, causing 75% of all small animal coagulopathies. 

When people think of hereditary coagulation disorders, they usually think of hemophilia, especially hemophilia A.  This results from a deficiency of factor VIII and is the most common severe hereditary coagulopathy.  It occurs in most breeds of dogs and cats and in mixed breeds.  It is an X-linked recessive gene, affecting the male offspring of female carries.  Signs vary from mild (trauma results in prolonged bleeding) to severe (excessive bleeding into the tissues).  The lab results are:

  •  APTT-                      prolonged

  • PT-                          WNL

  • Bleeding time-       WNL

 VonWillebrand disease is the most common hereditary coagulation disorder, and results from a deficiency of vonWillebrand factor.  It is seen in at least 54 breeds of dogs, and occurs in swine, cats (rarely) and man.  It is extremely common in Doberman pinschers (70+%) and is also seen in shelties, Scotties, German shepherds, miniature schnauzers, golden retrievers, basset hounds, standard poodles, keeshonds, rotties, dachshunds, Manchester terries, Pembroke Welsh corgis and Chesapeake bay retrievers.  It typically has a high morbidity in the affected breeds, but a low mortality.  Signs are variable; it may be subclinical, there may be inappropriate prolonged bleeding from nail trims or teething, and mucosal bleeding may occur.  Some dogs have an intermittent hemorrhagic diarrhea.  The laboratory results are:

  • APTT-                      WNL

  • PT-                          WNL

  • Bleeding time-       prolonged

  •  vW:ag assay-         low     

 Acquired coagulation disorders are usually associated with vitamin K antagonists (rodenticides and accidental ingestion of coumadin medications) and disseminated intravascular coagulopathy (DIC).

 Rodenticide coagulopathies occur with rat poisons containing warfarin.  The same drug is used in human patients as a blood thinner to prevent clots.  They cause the depletion of vitamin K, which is needed for the synthesis of coagulation factors II, VI, IX and X.  This can result in hemothorax, dyspnea and ventral hematomas.  The lab results are:

  • APTT-                      prolonged

  • PT-                          prolonged

  • Bleeding time-       WNL

 Disseminated intravascular coagulopathy (DIC) results from the simultaneous overstimulation of the coagulation and fibrinolytic systems, causing the formation of microthrombi with concurrent hemorrhage. 

DIC is secondary to something that causes tissue necrosis and/or inflammation, including neoplasias, shock,  infectious diseases (ICH, FIP, heartworm…), noninfectious diseases (pancreatitis, hepatitis, congestive heart failure…), drugs (NSAIDS, steroids, chloramphenicol…), immune mediated diseases, and trauma (GDV, HBC, postsurgical…).  The signs vary with the primary disease, but is identified by increased bleeding, resulting in petechia and echymoses.  Obstruction of capillaries by microthrombi cause damage and failure in the adrenocortical, pulmonary, hepatic, pancreatic and central nervous systems.  The laboratory findings include:

  • APTT-                      prolonged

  • PT-                          prolonged

  • Bleeding time-       prolonged

  • FDP assay-              increased   (most specific test)

The result is DIC  (“death is coming” or “dead in cage”).

 

REVIEW QUESTIONS:

 1.         Define hemostasis and list the four components.
2.         What are common clinical signs of hemostatic abnormalities?
3.         Describe how the blood vessel prevents initiation of or contributes to hemostasis .
4.         Diagram and label the function of the platelet in hemostasis.
5.         Define ‘coagulation system”.
6.         Differentiate between the methods of initiation of the intrinsic vs. extrinsic coagulation systems.
7.         What is fibrinolysis and what is its purpose?
8.         Describe how to collect and handle a specimen obtained for coagulation testing.
9.         Why is it important to test for the number of platelets?
10.        What test can evaluate platelet number AND function?
11.        What should be run with every test specimen in a coagulation system test?
12.        What is most common test for the intrinsic pathway?
13.        What is the most common test for the extrinsic pathway?
14.        What causes vonWIllebrand disease?
15.        What are typical test results for an animal with vonWillebrand disease?
16.        What occurs in DIC and what can cause it?
17.        What are typical test results for an animal with DIC?

 

11/03/2003