Bleeding Disorders Question And Answers

Bleeding Disorders Essay Questions

Question 1. Classify the common bleeding disorders and add a note on oral manifestations in different conditions.
Answer:

Bleeding disorders Classification:

• Coagulation factor deficiencies
• Platelet disorders
• Vascular disorders
• Fibrinolytic defects

Read And Learn More: Oral Medicine and Radiology Question And Answers

Coagulation Factor Deficiencies:

• Congenital
  • Factor 8 and 9 deficiencies (hemophilia A and hemophilia B)
  • von Willebrand disease (deficiency in factor 8 protein complex)
  • Other factor deficiencies (factor 11, 12, 10, 5, 13— rare).
• Acquired
  • Liver disease
  • Vitamin K deficiency
  • Heparin and coumarin therapy
  • Disseminated intravascular coagulation.

Platelet Disorders:

• Quantitative disorders —thrombocytopenia
  • Congenital
    • Immune-mediated
    • Collagen vascular disease
    • Sarcoidosis
  • Acquired
    • Idiopathic thrombocytopenic purpura
    • Disseminated intravascular coagulation
    • Microangiopathic hemolytic anemia
    • Leukemia
    • Myelofibrosis
    • Aplastic anemia
    • Systemic lupus erythematosus
    • Infection — HIV malaria, mononucleosis
    • Cytotoxic chemotherapy
    • Drug-induced — rifampin, quinine.

Qualitative disorder (nonthrombocytopenic or functional platelet defect)

• Congenital
  • Glanzmann’s thrombasthenia
  • Platelet-type von Willebrand disease
  • Bernard-Soulier syndrome
• Acquired
  • Drug-induced
  • Liver disease
  • Alcoholism

Vascular disorders (vessel wall disorders)

• Scurvy
• Purpura
• Hereditary hemorrhagic telangiectasia
• Cushing’s syndrome
• Ehlers-Danlos syndrome

Fibrinolytic defects:

• Disseminated intravascular coagulation
• Streptokinase therapy

Oral manifestations of bleeding disorders:

• Spontaneous gingival bleeding, petechiae, ecchymosis—multiple, small.
  • Characteristic of vascular and platelet disorders.
  • Unusual in coagulation disorders.
• Bleeding from surface injuries and scratches due to:
  • Continuous and profuse bleeding in vascular and platelet disorders.
  • Minimal in coagulation disorders.
• Delayed bleeding, hematomas:
  • Characteristic of coagulation disorders
  • Rare in vascular and platelet disorders.

Question 2. Briefly describe the anemic conditions associated with orofacial manifestations.
Answer:

• Anemic conditions with orofacial manifestations are:
  • Iron-deficiency anemia
  • Plummer-Vinson syndrome
  • Megaloblastic anemia
  • Sickle cell anemia
  • Thalassemia
  • Aplastic anemia

Iron-deficiency Anemia:

• It is the most prevalent type of hematological disorder and manifests in the orofacial region as:
  • Tongue atrophy (atrophic glossitis)
  • Pale mucosa
  • Angular cheilitis
• Atrophic glossitis is characterized by patchy, or diffuse, atrophy of papilla on the dorsal aspect of the tongue (flattening of the tongue papillae), giving the appearance of a smooth, red, and shiny tongue.
• In the advanced state, tenderness and a burning sensation of the tongue will be present.
• Angular stomatitis is a condition characterized by painful fissures at the corners of the lip. The common etiology includes iron deficiency and candidal infection.
• Treatment must focus on iron supplementation and anticandidal therapy. Iron deficiency state may also lead to peeling of superficial peeling of the epithelium at the lips and at the angle of mouth associated with dryness known as Cheilosis.

Plummer-Vinson Syndrome:

• It is also known as Paterson-Brown-Kelly syndrome and sideropenic dysphagia. It is caused by iron deficiency. Women are frequently affected.
• The features of this syndrome include atrophic glossitis, angular cheilitis, and difficulty in swallowing. The formation of an esophageal web (a ring-like membrane of 2-3 mm thick composed of normal esophageal structure (mucosa and submucosa protruding into the esophagus) causes dysphagia.
• Other associated findings include koilonychia (or spoon nails), pharyngoesophageal ulcerations, and white, keratotic lesions in the oral mucosa.
• This syndrome may rarely lead to carcinoma of the esophagus and throat due to the increased turnover rate of epithelial cells in the upper digestive tract. A barium swallow study and fluoroscopic evaluation will confirm the diagnosis.

Megaloblastic Anemia:

• It is caused by vitamin B12 and folic acid deficiency and is a slowly progressing condition. The oral manifestations include glossitis, stomatitis, and recurrent aphthous ulcers. These findings are associated with the altered- metabolic status of oral epithelial cells.
• Magenta tongue or Beefy red tongue is due to decreased epithelial thickness and is a characteristic of B12 deficiency. Oral changes are the early manifestations and occur before the sign of anemic symptoms or macrocytosis.

Sickle Cell Anemia:

• It is a hereditary disorder with the presence of abnormal hemoglobin S. There is a genetic replacement of valine by glutamic acid of the P-polypeptide chain of DNA at the sixth position.
• This change alters hemoglobin and subsequently the structure of red cells which acquires a sickle shape.
  • Hemoglobin value in sickle cell patients is reduced and is in the range between 5-9 g/dL. The white blood cell count range is elevated and between 12,000-20,000 cells/ mm³.
  • The life expectancy is variable because these patients may undergo sickle cell crises due to microvascular occlusion and multisystem complications.
  • Facial changes include increased thickening of the skull and osteoporotic changes, the tendency for a protruding maxilla and mandible, osteomyelitis of the mandible, and mental nerve paresthesia.
  • Oral and dental findings include pale mucosa, late eruption, malocclusion, hypoplastic enamel, midline diastema, and asymptomatic pulpal necrosis. The radiographic changes comprise coarse- trabecular pattern of the mandible, a step-ladder appearance of the alveolar bone.

Radiographic findings include:

• The sparse and coarse trabecular pattern gives rise to the generalized radiolucent appearance of jaw bones.
• The inferior border of the mandible appears thin.
• Generalized osteoporosis is commonly caused by salmonella infections.
• Step-ladder appearance in the inter¬dental bone is due to horizontal trabeculation.

Thalassemia: Thalassemia is a hereditary hemolytic anemia. There is a defective synthesis of α or β poly-peptide chains of hemoglobin (α-thalassemia, β-thalassemia).

Thalassemia Classification:

1. Homozygous thalassemia
2. Homozygous β-thalassemia (Cooley’s anemia or Mediterranean anemia, or thalassemia major) is prevalent among the Mediterranean population (Greece, Turkey, Cyprus, and Southern Italy).
3. β-thalassemia is characterized by significant orofacial malformations and severe anemia. Hypoxia and cardiac failure in early infancy lead to a short span of life. To overcome the anemic changes, the bone marrow produces more red cells and shows hyperplasia and expansion.
4. The marrow changes result in the following facial malformations:
   • The affected individuals have a path-monic chipmunk face due to larger and prominent maxilla, frontal bossing, and prominent malar processes.
   • The maxilla-mandible discrepancy leads to class II malocclusion with increased overjet and spacing between maxillary teeth.
5. Heterozygous thalassemia (thalassemia minor)
6. Heterozygous thalassemia is a minor form and is asymptomatic. Peripheral smear analysis, it shows a hypochromic and microcytic pattern.
7. Compound heterozygous thalassemia

Question 3. Classify hematological malignancies of white blood cells. Describe the etiopathogenesis, oral manifestations, investigations, and dental consideration for leukemia.
Answer: Hematologic malignancies are leukemia, lymphoma, and plasma cell tumors.

Leukemia:

• Leukemia is associated with excessive exposure to ionizing radiation, certain chemicals (benzene), and some viral infections (for example Epstein-Barr virus, human lymphotropic virus).
• The result is the abnormal proliferation of white blood cell-forming tissues which leads to an increased number of immature and abnormal white blood cells in the circulation.

Classification of leukemia: Based on the type of hematopoietic stem and progenitor cells affected:

1. Acute myelogenous leukemia (AML)
2. Acute lymphocytic leukemia (ALL)
3. Chronic myelogenous leukemia (CML)
4. Chronic lymphocytic leukemia (CLL)

Leukemia Oral manifestations:

• Gingival enlargement:
  • Leukemic cells infiltrate into the gingival tissue and cause gingival hyperplasia. The inter¬dental papillae, marginal, and attached gingiva become progressively enlarged.
  • In severe form, the crowns are covered by gingiva. Gingival hyperplasia is more prevalent in acute leukemia than the chronic variety. Leukemic chemotherapy will help to control the hyperplastic growth to a greater extent.
  • Gingiva appears swollen, pale pink to purple color, and with loss of stippling.
  • Ulceration: Atypical mucosal ulcers are present.
  • Unprovoked bleeding from gingiva and petechiae.
  • Herpetic infections and candidiasis.

Leukemia Lab investigations:

• The peripheral granulocyte count is strikingly increased in chronic leukemia, whereas in acute leukemia, the count may either increase (with numerous blast forms), decrease, or normal.
• Atypical hematopoietic cells in the bone marrow.
• Cytogenetic analysis of chromosomal abnormalities.

Oral complications in leukemic patients:

• Spontaneous, severe bleeding gums.
• Pulpal and periodontal infections may lead to osteonecrosis of the jaw bones.
• Corticosteroid therapy may results in opportunistic candidal infection.

Dental care for leukemic patients:

• A detailed-history, comprehensive oral examination and full mouth radiographic examination are necessary for treatment planning. Treatment should be carried out after consulting the hematologists.
• Preventive dental care needs to be started prior to leukemic treatment (chemotherapy or radiotherapy).
• Patients in long-term remission can go for any dental treatment, but patients in severe or relapse conditions should be provided with palliative or emergency dental treatment alone.

Question 4. Dental treatment for patients with bleeding disorders.
Answer:

• The severity of the bleeding disorder and the invasiveness of the dental procedure will determine the course of dental treatment in these patients.
• If the dental treatment is minimally invasive and the bleeding disorder is a milder form, only minor or no changes are needed.
• For patients with severe bleeding disorders, revising the hemostatic system to a satisfactory level and hemostatic measures using local and adjunctive methods are necessary. The hematologist should be consulted before planning for invasive dental procedures.
• Oral surgical procedure: For coagulation disorders, transfusion of the right factors with, 50-100% of normal levels is mandatory.
  • Local hemostatic techniques like pressure, surgical packs, sutures, and surgical stents may also be applied independently or in combination.
• Periodontal procedures: Gingival probing and supragingival scaling can be carried out safely. Subgingival scaling and root planning may also be carried out carefully without inducing bleeding. Factor replacement is necessary only for extensive periodontal surgery and to give nerve blocks.
  • Bleeding can be controlled at the surgical site by using local hemostatic materials and conventional mouthguards. Postoperative bleeding can be controlled with the use of a hemostatic mouth rinse containing tranexamic acid or aminocaproic acid.
• Restorative and endodontic procedures: Dental restorations do not carry the bleeding risk. Appropriate care should be taken to avoid soft-tissue injury when using instruments and rubber dams. Routine endodontic therapy can be performed without any special attention but, surgical endodontic procedures require factor replacement therapy.
• Prosthodontic procedures: Careful post-insertion adjustments will minimize trauma. Oral tissue should be handled carefully during prosthesis fabrication to reduce the risk of ecchymosis.
• Orthodontic procedures: Orthodontic treatment can be carried out without any modification. The appliances should not impinge on soft tissues, and good oral hygiene practices should be encouraged.

Bleeding Disorders Medical management:

• Nonsteroidal anti-inflammatory drugs should not be prescribed for patients with antiplatelet and anticoagulant medications because of their increased risk of bleeding.
• Penicillins, erythromycin, metronidazole, tetracyclines, and miconazole potentiate the action of warfarin.

A physician’s advice is mandatory before prescribing these drugs.

• Patients on antiplatelet drugs: In most cases, no need to stop antiplatelet drugs like aspirin (ASA), clopidogrel, and dipyridamole.
• Local hemostatic measures are adequate to control bleeding. Before modifying the drug regimen, the patient’s physician should be consulted.

Patients on anticoagulant drugs:

• For patients taking warfarin, the international normalized ratio (INR) should be checked before invasive procedures. The normal range is 2.0-3.0. When INR is below 3.0, dental surgery can be carried out routinely, and no need to revise the warfarin dose.
• Heparin is administered to end-stage renal patients undergoing dialysis. Dental procedures should be carried out on the days when the patient is not posted for dialysis.

Question 5. Describe the pathogenesis and orofacial manifestations of lymphomas.
Answer:

• Lymphoma is a solid malignancy of the reticuloendothelial system which cares for the body’s immune mechanism. Lymphocytes are the affected cells.
• Lymphomas are of two types: Hodgkin’s and non-Hodgkin’s. About 90% of cases are non-Hodgkin’s type, while about 10% is Hodgkin’s type.
• In non-Hodgkin’s type, both B and T lymphocytes are affected, whereas, in Hodgkin’s lymphoma, only an abnormal type of B lymphocyte named Reed- Sternberg cells are involved.

Non-Hodgkin’s lymphoma (NHL):

• Non-Hodgkin’s lymphoma is the second most prevalent oropharyngeal tumor next to squamous cell carcinoma. It occurs at an extranodal site in 40% of cases and 2-3% of these cases occur in the mouth and jaw bones.
• Waldeyer’s ring, salivary glands, and maxilla are the common sites, although any region may be affected. Oral lesions appear as a red and painless enlargement. Surface ulceration occurs when subjected to trauma. However, deep oral ulcerations are rare. The mobility of teeth is common in the affected jaw, and rarely paresthesia will occur.
• The characteristic symptoms include a fever of unknown origin (>38°C), weight loss (>10% over 6 months), drenching night sweats, visceral pain, and malaise.
  • Radiographic findings include genera¬lized bone resorption and loss of the lamina dura. The loss of the lamina dura in lymphoma patients may be related to the effects of tumor cell infiltration and in some cases may be because of the release of osteoclast-activating factors from the lymphoid cells.
  • Non-Hodgkin’s lymphoma is classified as indolent, aggressive, and highly aggressive.
    • Indolent lymphoma accounts for 40% of all NHL with the most common type being follicular lymphoma. Aggressive lymphoma approximately represents 50% of cases and occurs as diffuse large B-cell lymphoma and T-cell natural killer (NK) cell lymphoma.
    • The highly aggressive form includes Burkitt’s lymphoma and lymphoblastic lymphoma.

Burkitt’s lymphoma: It is a fast¬growing tumor. Three types are recognized:

1. Endemic (African): Primarily affects African children in the age group of 4-7 and has a preference for boys with a 2:1 ratio.
2. Sporadic (non-African): It occurs worldwide and accounts for 1-2% of adult lymphoma cases.
3. Immunodeficiency-associated: This variant of Burkitt’s lymphoma occurs in HIV or AIDS patients.

It represents 30-40% of non-Hodgkin’s lymphoma in HIV- patients and is also an AIDS-defining disease.

Lymphoma Symptoms: The symptoms of Burkitt’s lymphoma vary among subtypes. The endemic variant starts with tumors involving the jaw or other facial bones.

Lymphoma Investigations:

• Computed tomography (CT) and magnetic resonance imaging (MRI): If neurologic signs or symptoms are present.
• Bone scanning and plain radiography: When associated with bone symptoms.

Lymphoma Treatment:

• Intensive chemotherapy is the main treatment.
• Cyclophosphamide
• Cytarabine
• Doxorubicin
• Etoposide
• Methotrexate
• Vincristine

Hodgkin’s lymphoma (Hodgkin’s disease):

• The frequent manifestation is cervical lymphadenopathy and extranodal Hodgkin’s lymphoma is extremely rare. The tumor spreads via the lymphatic channels in a characteristic pattern and hence the treatment results in a good prognosis.
• If extranodal Hodgkin’s lymphoma occurs, it presents as swelling and ulcers on the oral mucosa of the palate, tonsils, floor of mouth, vestibule, and alveolus

Bleeding Disorders Short Notes

Question 1. International normalized ratio (INR).
Answer:

• The prothrombin time (PT) is highly variable because different thromboplastins derived from different sources are nonstandardized.
• Prothrombin time performed on the same sample at different laboratories revealed different results. To standardize the PT, the INR was introduced by World Health Organization (WHO) in 1983.

International normalized ratio Method:

• The INR is a ratio and has no units.
• The first point in the INR calculation is to normalize the PT by comparing it with the mean standard prothrombin time (MNPT— the mean prothrombin time of healthy adult individuals).
• In the next step, the ratio is raised to a power nominated by the international sensitivity index (ISI). The ISI is a function of the thromboplastin reagent. Two data groups are used to obtain the ISI:
• Healthy adult people.
• Patients stabilized on warfarin.
• The normal INR is typical of 1.0 but varies from 0.9-1.1. During Warfarin administration, the INR increases between 2 and 3.5.
• This test assesses the extrinsic pathway of coagulation and measures factors 1, 2, 5, 7, and 10.

International normalized ratio Purpose:

• The International normalized ratio is helpful in evaluating the effectiveness of warfarin. Warfarin therapy is indicated to prevent clot formation and at the same time to avoid excess bleeding.
• The INR is used to adjust a person’s warfarin dose and to bring the PT into an acceptable range.
• International normalized ratio Inference:
• No dental surgical procedure is recommended when INR is >3.5-4.
• For full mouth extraction, INR should be <2-3.
• For flap surgery and multiple trans alveolar extractions, INR should be <1.5.

Question 2. Outline the medical consideration for dental patients with liver diseases.
Answer:

• In liver diseases, the production of clotting factors (factor 2, 7, 9, and 10, vitamin K) is reduced due to parenchymal damage and obstruction. These patients exhibit bleeding tendency depending on the extent of their liver disease.
• Viral hepatitis patients are a source of cross-infection, and so necessary precautions should be taken during procedures.

Management of hemostatic defects in liver disease:

• Vitamin K and freshly frozen plasma infusion for prolonged prothrombin time and partial thromboplastin time.
• Cryoprecipitate is the choice for a replacement for factor 8 deficiency and disseminated intravascular coagulation.
• The impaired liver function necessitates dose modification before drug administration in these patients. The patient’s physician should be consulted for the same.

Question 3. Dental consideration for patients with platelet disorders.
(or)
Dental management for patients with thrombocytopenic purpura.
Answer:

• Thrombocytopenia is a platelet disorder that manifest due to the following abnormalities:
• Decreased bone marrow production of platelets
• Increased platelet destruction
• Platelet sequestration due to enlarged spleen.

There are two types of platelet disorders:

1. Idiopathic thrombocytopenic purpura (ITP).
2. Thrombotic thrombocytopenic purpura.

Idiopathic thrombocytopenic purpura is characterized by mild to a severe decrease in platelet count, with bone marrow in relatively healthy condition, and by mucocutaneous bleeding with the absence of other abnor¬malities.

Thrombocytopenia Pathogenesis: The reason for platelet reduction is idiopathic (unknown). The autoimmune reaction of platelet destruction seems to participate in this process.

Thrombocytopenia Signs and Symptoms:

• An important general symptom is the presence of petechiae which indicates capillary bleeding.
• Oral manifestations—in advanced or severe stage:
  • Gingival bleeding is the first indicator of thrombocytopenia many times. Profuse bleeding occurs either spontaneously, or following a minor trauma induced by tooth brushing and flossing.
  • Hematomas and hemorrhagic bullae.
  • Petechiae and ecchymosis in the soft palate and buccal mucosa.

Thrombocytopenia Investigations:

• The complete blood count (CBC) is useful in the diagnosis.
• The reduced number of platelets supports the diagnosis of thrombocytopenia.
  • When platelet levels are between 50,000/ mm3 and 100,000/mm³, mild clinical manifestations can occur because the platelets still maintain the capacity for hemostatic equilibrium.
  • When the platelet number is below 50,000/mm3, it suggests purpuric manifestations.
  • Below 30,000/mm3, the symptoms and signs are more intense; when between 10,000/mm3 and 5,000/mm³, the hemorrhagic phenomena can be critical.
  • Platelet function is evaluated by bleeding time (BT) and is prolonged in thrombo¬cytopenia (>5 min). Clot retraction may be deficient or absent with only a slight absorption of prothrombin 1 hour after coagulation.

Thrombocytopenia Dental Consideration:

• Dental treatment of ITP patients should correlate with the presentation of the disease and physicians’ help should be taken to proceed.
  • Dental treatment is indicated when the platelet counts above 50,000/mm³.
  • If the platelet count is very low (10,000- 20,000/mcL), dental treatment is contraindicated. In emergency situations, the patient should be supported with 4 Immunoglobulin (IVIg) combined with 4 methylprednisolones to provide the needful assistance.
  • In routine conditions, prednisone 1-2 mg/kg/day is the initial treatment option.

Question 4. What is a tourniquet test?
(or)
Capillary fragility test
(or)
Rumpel-Leede phenomenon.
Answer: The tourniquet test is a marker of capillary fragility.

• Tourniquet test Method:
  • Record of the patient’s blood pressure by covering 2/3rd of the arm with a sphygmomanometer.
  • Calculating the mean blood pressure— systole+diastole/2.
  • The cuff should be retained at mean blood pressure for 5 minutes.
  • About 2 minutes following cuff deflation and removal, petechiae should be counted in the 4 cm distal surface of the ante- cubital fossa.
  • A positive test is 10 or more petechiae per 1 square inch.
• In normal conditions, for men, the number of petechiae is up to 5, whereas in women and children up to 10 petechiae appear but do not go beyond 10.

Question 5. Enumerate dental management for hemophilic patients.
Answer:

• Hemophilia A—Invasive dental procedures need the physician’s assistance. Factor 8 replacement may be necessary.
  • Desmopressin (DDAVP) is valuable to achieve a transient rise in factor 8 level. This drug is adequate to meet hemostasis in mild hemophilia. Desmopressin is administered along with antifibrinolytic agents to enhance its effectiveness.
  • Hemostatic mouth rinse is helpful in postoperative care. Epsilon aminocaproic acid and tranexamic acid are the frequently used antifibrinolytic hemostatic oral rinses.
• Hemophilia B is a factor 9 deficiency disease. Replacement therapy manages it with highly purified, virally inactivated factor 9 concentrates.

Question 6. Briefly describe the systemic management of patients with bleeding disorders.
Answer: For irreversible coagulopathies, systemic replacement of missing factors may be necessary.

The standard agents used are:

1. Platelets

• Platelets: 1 unit = 50 mL; and raise count by 6,000.
• Indications: Platelet count:
  • It is less than 10,000 in nonbleeding individuals.
  • It is less than 50,000 in the presurgical level.
  • It is less than 50,000 in actively-bleeding individuals.
  • Nondestructive thrombocytopenia.

2. Fresh Frozen Plasma: Fresh frozen plasma: 1 unit = 150-250 mL, contains factors 2, 7, 9, 10, 11, 12, 13, and heat-labile 5 and 7.

Fresh Frozen Plasma Indications:

• Undiagnosed bleeding disorder with active bleeding.
• Severe liver disease.
• When transfusing is greater than 10 units of blood.
• Immune globulin deficiency.

3. Cryoprecipitate:

• Cryoprecipitate: 1 unit = 10-15 mL.
• Indications:
  • Hemophilia A, von Willebrand disease
  • When specific factor concentrates are unavailable.
  • Fibrinogen deficiency.

4. Factor 8 Concentrate:

• Factor 8 concentrate: 1 unit raises factor 8 level by 2%
• Indications:
  • Hemophilia A with active bleeding state or before surgery.
  • von Willebrand disease.

5. Factor 9 Concentrate:

• Factor 9 concentrate: 1 unit raises factor 9 level by 1-1.5%.
• Indications: Hemophilia B with active bleeding or before surgery.

6. Desmopressin:

• Synthetic analog of antidiuretic hormone, —intranasal application mode.
• Indications:
  • Active bleeding state in von Willebrand disease or before surgery
  • Uremic bleeding in liver disease
  • Bleeding in esophageal varices.

7. Tranexamic Acid:

• It has 4.8% mouth rinses for antifibrinolytic effect following oral surgical procedures.
• Oral dose: 25 mg/kg 8 hourly.
• Indications: To maintain clot formation.

Question 7. Discuss local hemostatic agents.
Answer:

• Absorbent gelatin sponge material
• Microporous polysaccharide hemispheres
• Oxidized cellulose
• Fibrin sealant (glue)
• Topical thrombin
• Tranexamic acid
• Epsilon-aminocaproic acid
• The use of absorbable hemostatic materials helps in clot formation and maintaining clot stability. But these materials should be avoided in immunosuppressed patients as they carry a risk of infection and delay the healing period.
• Topical thrombin products fasten the hemostasis when applied at the surgical site. They convert soluble fibrinogen to insoluble fibrin and arrest bleeding.
• When using thrombin products in conjunction with absorbable collagen or cellulose (gel form) or with fibrinogen (fibrin glues), the hemostasis is very efficient and this minimizes the chances for factor replace¬ment.

Question 8. Multiple myeloma.
Answer:

Multiple myeloma (MM) is a malignant neoplasm of plasma cells characterized by the synthesis of pathologic M proteins, bony lesions, kidney disease, hyperviscosity of blood, and hypercalcemia.

Multiple myeloma Oral manifestations:

• The patient may experience pain, swelling, and numbness of the jaws, gingival epulis, and mobility of the teeth.
• More than 30% of MM patients develop osteolytic jaw lesions. These lesions are more common in the mandible than in the maxilla and occur in the posterior teeth region, ramus, and condylar process, because of the increased hematopoiesis in these sites.

Multiple myeloma Radiographic features:

• Normal bone pattern
• Multiple radiolucent lesions
• Diffuse rarefaction
• Osteoporotic changes.

Skull lesions frequently occur more than jaw lesions. Multiple punched-out radiolucent lesions of varying size with ill-defined margins is pathognomonic and suggest the diagnosis.

Multiple myeloma Dental treatment:

• Dental procedures are routinely indi¬cated for myeloma patients on the recovery phase. For patients in the active phase or relapse state, hemato-oncologist guidance is mandatory to proceed with dental treatment.
• It is advisable to start dental treatment before chemo-or radiotherapy.
• If a hematopoietic cell transplant is planned for a patient, dental procedures should not be carried out in the immediate pre and post-procedure days (minimum of 72 hours) to avoid tran-sient bacteremia.

Question 9. Basic physiology of hemostasis.
Answer: Hemostasis is a physiological process of arresting the blood flow or blood loss following an injury (hemo = blood; stasis = standing).

It has four basic steps:

1. 1. Vascular phase
   2. Platelet phase
   3. Coagulation cascade
   4. Fibrinolytic phase

• When a blood vessel is injured, the first 3 phases play a major role to stop bleeding and prevent blood loss.
  • Vasoconstriction—Vasoconstrictors like serotonin are released from platelets.
  • Platelet plug formation—The platelets attach to the collagen in the damaged vessels. These attached platelets release an enzyme, adenosine diphosphate (ADP) which attracts more platelets and form a plug that temporarily stops bleeding.
  • Blood coagulation—fibrin deposition will reinforce the loose plug into a definite plug.

Physiology of coagulation:

• The clotting process is known as coagulation and involves the following steps:
  • Formation of fibrin from fibrinogen.
  • Formation of unstable fibrin mesh— fibrin monomers polymerize to form a loose mesh.
  • Formation of intact fibrin mesh (clot stabilization)—formation of covalent cross-linkages within fibrin mesh will result in a dense and stable mesh.
• Fibrinolytic system: Once healing occurs, the clot formed within the vessel is disposed of fibrinolysis which involves the dissolution of the clot. This step needs the presence of a proteolytic enzyme called plasmin.

Bleeding Disorders Multiple Choice Questions

Question 1. Platelet count and bleeding time are used to check the

1. Vascular spasm
2. Primary hemostasis
3. Clot formation
4. Secondary hemostasis
   (Note: In primary hemostasis, the platelets adhere to the damaged endothelium and form a platelet plug).

Answer: 2. Primary hemostasis

Question 2. Spontaneous clinical hemorrhage occurs when the platelet count is

1. 10,000-20,000/mm³
2. Below 50,000/mm³
3. 50,000-1,00,000/mm³
4. Below 10,000/mm³

Answer: 1. 10,000-20,000/mm³

Question 3. Surgical or traumatic hemorrhage occurs when the platelet count is below

1. 50,000/mm³
2. 1,00,000/mm³
3. 10,000/mm³
4. 20,000/mm³

Answer: 1. 50,000/mm³

Question 4. Normal bleeding time by modified Ivy’s test is

1. Between 10 minutes and 15 minutes
2. Between 1 second and 6 seconds
3. Between 10 seconds and 15 seconds
4. Between 1 minute and 6 minutes

Answer: 4. Between 1 minute and 6 minutes

Question 5. Bleeding time is considered as prolonged if it is greater than

1. 5 minutes
2. 15 minutes
3. 5 seconds
4. 15 seconds

Answer: 2. 15 minutes

Question 6. Platelet transfusion is given

1. 1 hour before surgery
2. 1 day before surgery
3. 30 minutes before surgery
4. 4 hours before surgery

Answer: 3. 30 minutes before surgery

Question 7. The most common inherited coagulopathy is

1. Hemophilia C
2. Hemophilia A
3. von Willibrand disease
4. Stuart disease

Answer: 3. Von Willibrand disease

Question 8. The rate-limiting step in clotting is

1. Endothelial damage
2. Platelet aggregation
3. Thrombin activation
4. Fibrinolysis

Answer: 4. Fibrinolysis

Question 9. The normal range of prothrombin time is

1. 11-13 minutes
2. 1-7 minutes
3. 11-13 seconds
4. 1-7 seconds

Answer: 3. 11-13 seconds

Question 10. Vessel wall integrity or platelet disorders is diagnosed by

1. Tourniquet test
2. Coagulation test
3. Hematocrit
4. IVY method

Answer: 1. Tourniquet test

Question 11. The rumple-Leede phenomenon is used for measuring

1. Bleeding time
2. Capillary fragility
3. Factor deficiency
4. Clot integrity
   (Note: Tourniquet test uses the Rumple-Leede phenomenon for measuring capillary fragility).

Answer: 2. Capillary fragility

Question 12. Scurvy results when the daily intake of vitamin C falls below 10 mg/day.

1. 90 mg/day
2. 75 mg/day
3. 500 mg/day
4. 10 mg/day

Answer: 4. 10 mg/day

Question 13. Hypermobility of the temporomandibular joint is a feature of

1. Temporomandibular dysfunction syndrome
2. Laskin’s syndrome
3. Ehlers-Danlos syndrome
4. Myasthenia gravis

Answer: 3. Ehlers-Danlos syndrome

Question 14. Stunted teeth with pulp stones is a finding in

1. Taurodontism
2. Regional odontodysplasia
3. Klinefelter syndrome
4. Ehlers-Danlos syndrome

Answer: 4. Ehlers-Danlos syndrome

Question 15. Glanzmann’s thrombasthenia is a qualitative defect of

1. Platelets
2. Factor 9
3. Thrombocytes
4. Factor 2
   (Note: Deficiency in platelet membrane phos¬pholipids).

Answer: 1. Platelets

Question 16. Bernard-Soulier syndrome is a qualitative disorder of

1. Platelets
2. Factor 9
3. Thrombocytes
4. Factor 2
   (Note: Defect in platelet membrane glycoprotein).

Answer: 1. Platelets

Question 17. The expected complications of using cryoprecipitate and fresh frozen plasma are the

1. Hypersensitive reaction
2. Hypothermia and bradycardia.
3. Transmission of viral infection
4. Transfusion hemolysis,

Answer: 3. Transmission of viral infection

Question 18. Ischemic tissue injury is a characteristic of

1. Thalassemia major
2. Intravascular coagulation disorders
3. Herpes zoster infection
4. Sickle cell anemia

Answer: 4. Sickle cell anemia

Bleeding Disorders  Viva Voce

Question 1. What is the range of normal platelet count?
Answer: The normal range is 1,50,000-4,50,000/mm

Question 2. What are the guidelines for treating dental patients on warfarin?
Answer: For patients taking warfarin, their INR should be checked before any invasive procedure. The therapeutic range is 2.0-3.0. Many of the minor surgical procedures can be carried out without any alteration in warfarin dose when INR is less than 3.0.

Question 3. What are the guidelines for treating dental patients on heparin?
Answer: Heparin is given for end-stage renal disease patients on hemodialysis. Heparin has a short half-life of 5 hours, and patients can be treated safely on the days between dialysis.

Question 4. Name the inherited coagulopathies.
Answer: von Willibrand disease (deficiency of von Willibrand factor), hemophilia A, and hemophilia B.

Question 5. What are the complications of factor replacement therapy?
Answer:

• Allergic reactions.
• Viral disease (hepatitis B and C, HIV, cytomegalovirus).
• Development of antibodies to factor concentrates.
• Disseminated intravascular coagulation.
• Thromboembolic diseases (especially for factor 9 complex concentrate).

Question 6. How aspirin affects hemostasis?
Answer:

• Aspirin is an antiplatelet drug that causes a functional defect in platelets as follows:
• Aspirin inactivates the enzyme prostaglandin synthetase resulting in the inactivation of the cyclooxygenase catalytic pathway.
• This decreases the biosynthesis of prostaglandin and thromboxanes which are essential for the interactions between platelets and endothelium for hemostasis.

Question 7. What is pagophagia?
Answer: Pagophagia is a craving for ice chewing or iced drink. It is an occasional symptom of Plummer- Vinson syndrome (iron-deficiency anemia) due to pharyngoesophageal ulceration.

Bleeding Disorders Highlights

• This chapter deals with the management of dental patients with various bleeding disorders. Hemostatic disorders have characteristic findings in the mouth and facial structures.
• Many of these signs are nonspecific, but instruct the dental surgeon about the chances for a coexisting hematological disease.
• It is essential in dental practice to understand the risk of bleeding when performing semiological and surgical procedures with consideration of their medication and provide safe care.