Bleeding Disorders: Types, Causes, Symptoms & Treatment

Bleeding Disorders: Types, Causes, Symptoms & Treatment

Question. Describe various bleeding disorders and their management in detail.
Or
Write notes on bleeding disorders.

Answer.
Etiology Of Bleeding Disorders

Vascular Defects

• Bleeding disorders caused by vascular defects may be caused by structural malformation of vessels.
• Hereditary disorders of connective tissue and acquired connective tissue disorders.
• Vascular defects rarely cause serious bleeding.
• Bleeding into skin or mucous membrane starts immediately alter trauma but ceases within 24 to 48 hours.
• The vascular defects are hereditary hemorrhagic telangectasia, Henoch­Schönlein purpura.

Bleeding Disorders: Types, Causes, Symptoms, and Treatment

Platelet Disorder:

It can be of two types:

• Reduction in number—Thrombocytopenic purpura. If the total number of circulating platelets falls below 50,000 per mm3 of blood the patient can have bleeding.
  In some cases,the total platelet count is reduced by unknown mechanism,this is called primary or idiopathic thrombocytopenic purpura (ITP).
  Chemicals, radiation and various systemic disease (e.g. leukemia) may have direct effct on the bone marrow and may result in secondary thrombocytopenia.
• Defect in quality: Nonthrombocytopenic purpura, e.g.von Willebrand’s disease, Bernard-Soulier disease,Glanzmann’s thrombasthenia von Willebrand’s disease (pseudohemophilia) is the most common inherited bleeding disorder. Unlike hemophilia, it can occur in females. This is a disease of both coagulation factors and platelets. It is caused by an inherited defect involving platelet adhesion. Platelet adhesion
  is affcted because of a defiiency of von Willebrand`s factor.
• Various drugs such as carbamazepine, aspirin, methyl dopa, phenytoin can also lead to platelet disorders.

Types of Bleeding Disorders and Their Causes

Coagulation Defects

• Hemophilia A: It is the most common coagulation defect. It is inherited as X­linked recessive trait.
  The hemostatic abnormality in hemophilla A is caused by a defiiency/defect of factor VIII.
  Until recently, factor VIII was thought to be produced by endothelial cells and not by the liver as most of coagulation factors.
  The defective gene is located on the X­chromosome.
• Hemophilia B (Christmas disease): Factor IX is defiient or defective.
  It is inherited as X­linked recessive trait.
  Like HemophiliaA, the disease primarily affcts males and the clinical manifestations of the two are identical.
• Disseminated intravascular coagulation (DIC): It is a condition that results when the clotting system is activated in all or a major part of vascular system.
  Despite widespread firinproductionthe major clinical problem is bleeding not thrombosis.
  DIC is associated with a number of disorders such as infection, obstetric complications, cancer and snakebite.

Bleeding Disorders Symptoms and Diagnosis

Management of Bleeding disorders

Hereditary Hemorrhagic telangectasia

It is transmittd as autosomal dominanttraitand is characterized by bleeding from mucous membrane.

Management

• In patient having repeated attacks of epistaxis septal dermoplasty should be done. In septal dermoplasty, involved mucosa get removed and skin grafting is done.
• If spontaneous hemorrhages are present or nasal bleeding is present, then it is controlled by giving pressure packs.
• Sclerosing agents,, i.e. sodium tetradecyl sulphate, if injected intralesionally stop bleeding.
• Electrocautery is done. It helps in arresting bleeding.

Idiopathic thrombocytopenic Purpura (ItP)

Steroid Treatment Protocol

• Initial steroid treatment protocol forITP: Initial steroid treatment protocol l mg/kg/day prednisone, PO for 2-6 weeks.
• Subsequent steroid treatment protocol for ITP: Prednisone dose is individualized for every patient.
  Usually, the dose of prednisone is tapered to less than l0 mg per day for 3 months and then withdrawn.
  Splenectomy is done, if discontinuation of prednisone causes a relapse.
• Follow the ’rule of twos’ for major dental treatment and provide extra steroids prior to surgery, if the patient is currently on steroids or has used steroids for 2 weeks longer within the past 2 years.

Idiopathic thrombocytopenic Purpura  Minor Surgery

• Hemostasis after minor surgery is usually adequate, if platelet levels are above 50,000 cells/mm3.
• Platelets can be replaced or supplemented by platelet transfusions; though sequestration of platelets occurs rapidly. Platelet transfusion is indicated for established thrombocytopenic bleeding.
• When given prophylactically platelets should be given half before surgery to control capillary bleeding and half at the end of the operation to facilitate the placement of adequate sutures.
• Platelets should be used within 6–24 hours after collection and suitable preparations include platelet rich plasma (PRP), which contains about 90% of the platelets from a unit of fresh blood and platelet­rich concentrate (PRC), which contains about 50% of the platelets from a unit of fresh whole blood.
• PRC is thus the best source of platelets. Platelet infusions carry the risk of isoimmunization, infection with bloodborne viruses and, rarely, graft­versus­host disease
• Where there is immune destruction of platelets (e.g. in ITP), platelet infusions are less effctive.
• The need for platelet transfusions can be reduced by local hemostatic measures and the use of Desmopressin or tranexamic acid or topical administration of platelet concentrates.
• Absorbable hemostatic agents such as oxidized regenerated cellulose (Surgical), synthetic collagen (lnstat) or microcrystalline collagen (Avitene) may be put in the socket to assist clottng in postextraction socket.
• Drugs that affct platelet function, such as gentamicin, antihistamines and aspirin should be avoided.

Idiopathic thrombocytopenic Purpura  Major Surgery

For major surgery platelet levels over 75,000 cells/mm3 are desirable.

Von Willebrand Disease

• It is the most common inherited bleeding disorder. It is inherited as autosomal dominant but a severe form of disease may be inherited as a sex­linked recessive trait.
• It is caused due to the deficiency or defect in Von Willebrand factor.
• Types of Von-Willebrand diseases are: Type I, Type II A and II B, Type III

Von Willebrand Disease Management

• Surgical procedures can be performed in patients with mild von Willebrand disease by using DDAVP and EACA.
  Patients with severe Von Willebrand disease requires cryoprecipitate and Factor VIII concentrate.
• Bleeding should be controlled by using local measures such as pressure packs, gelfoam with thrombin, tranexamic acid, etc.
• Aspirin and NSAIDs are avoided and acetaminophen can be given to patients.
• In majority of patients with von Willebrand disease hemostatic defect is controlled with desmopressin via nasal spray.
• Type I vonWillebrand disease is treated withdesmopressin while Type II A and B and Type III require clottng factor replacement.

Disseminated Intravascular Coagulation Management

• Correction of hemodynamic instability by flid therapy,transfusion of packed cells or whole blood.
• Factor replacement: This is the specifi therapy, in this fresh frozen plasma, cryoprecipitate, platelet concentrate transfusions are essential. Fresh­frozen plasma is given at the dose of 15 mL/kg. Platelet is transfused at the dosage of 0.1 unit/kg.