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Blood dyscrasias
- 3. BLOOD DYSCRASIAS Presented By: Dr. PearlBhardwaj PG I year. Guided By: Dr H. S. Grover and the Faculty
- 4. Introduction • Dyscrasia (ordyskrasia) is a concept from ancient Greek medicine, meaning bad mixture.
- 5. History • The conceptof dyscrasia was developed by the Greek physician Galen (130–199 AD), who elaborated a model of health and disease as a structure of elements, qualities, humors, organs, and temperaments. Health was understood in this perspective to be a condition of harmony or balance among these basic components, called eucrasia. Disease was interpreted as the disproportion of bodily fluids or four humours: phlegm, blood, and yellow and black bile. The imbalance was called dyscrasia.
- 6. Blood Blood Plasma Formed Elements RBCs WBCs Platelets Blood is abodily fluid that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from the cells.
- 7. Erythrocytes or RBCs •Circular, biconcave cell. • Doesn’t contain a nucleus, ribosomes, or mitochondria. • Contains Hb which is interwoven in the stroma. • The functions of RBCs include: • Carriage of O2 from the lungs to peripheral tissues. • Carriage of CO2. • Approx. 5 x 106 /cumm • Approx. life span- 120 days.
- 8. Haemoglobin • Presentin RBCs. • Required for: • O2 transport • Co2 transport • Acts as a blood buffer • Each molecule has 2 ingredients- haem and globin. • Haemoglobin is a conjugated protein.
- 9. Leucocytes or WBCs •Leukos= white + cytes= cells • Their fundamental job is to: • provide defence against: • Bacteria • Viruses • Fungi • Parasites • Remove debris(dead cells) and foreign protein. • Produce chemicals that produce inflammation.
- 10.
- 11. Classification Blood dyscrasias Disorders of blood cells Redcell disorders White cell disorders Disorders of haemoglobin Haem disorders Globin disorders Disorders of hemostatic mechanisms Bleeding disorders Coagulation disorders
- 12.
- 13. Red Cell Disorders Disordersof RBCs numbers Anaemias Polycythemia Disorder because of defects in the RBC membrane Hereditary Spherocytosis
- 14.
- 15. Anaemias Deficiency Anaemias Iron deficiency anaemia Vitamin B12 deficiency anaemia Folicacid deficiency anaemia Hemolytic Anaemias Haemoglobino p-athies Thalassaemias Erythrocyte Membrane Defects Acquired hemolytic Anaemias Others Aplastic anaemia Fanconi’s Anaemia Associated with systemic diseases
- 16. Iron Deficiency Anaemia •Most common anaemia. • Microcytic hypochromic type of anaemia. • Women are affected 4 times more frequently than men. CAUSES • Nutritional Deficiencies • Chronic Blood loss • Malaria
- 17. Systemic features • Nonein the early stages Cardiorespiratory • Dyspnoea • Tachycardia • Palpitations Cutaneous • Pallor • Koilonychia- spoon shaped nails
- 18.
- 19. General Management • Administrationof 300 mg ferrous sulphate orally, 3 times daily for 10 days. • Mild gastrointestinal intolerance is the side effect. • Takes 10 to 20 days for the hematocrit to normalize. • Parenteral administration reserved for patients who do not respond to oral therapy.
- 20. Dental Considerations • LocalAnesthesia adequate for pain control. • Atrophic glossitis – soreness of tongue with depapillation or colour change- best known effect of severe anaemia. • Candidiasis may be a presenting feature caused by candida albicans which may be promoted by anaemia itself.
- 21. Periodontal Manifestations • IDA-CPpatients exhibited a higher gingival index, bleeding on probing, probing pocket depth, and percentage (%) of sites with a clinical attachment loss (CAL) of ≥6 mm than CP patients. • In patients with significant anaemia, elective periodontal surgical procedures should be avoided as they may cause bleeding problems and also poor wound healing. Impact of iron deficiency anemia on chronic periodontitis and superoxide dismutase activity: a cross-sectional study. Souvik Chakraborty, Shikha Tewari1, Rajinder Kumar Sharma, Satish Chander, Narula1, Pratap Singh Ghalaut, Veena Ghalaut. Journal of Periodontal Implant Sci 2014;44:57-64.
- 22.
- 23. Vitamin B12 Deficiency Anaemia CAUSES •Auto immune- Pernicious Anaemia (due to atrophy of gastric mucosa). • Poor Intake- specially in vegetarians. • Malabsorption • Congenital • Gastrectomy • Ileal disease • Drugs (rare) • Colchicine • Neomycin
- 24. General Features Slow todevelop because liver stores last upto 3 years. • Weakness • Fatigue • Light-headedness, • Rapid heartbeat • Rapid breathing • Pale color to the skin • In addition, neurological symptoms particularly paresthesia of the extremities may develop.
- 25. Oral Manifestations • Aphysically normal, sore or burning tongue can be caused by early Vitamin B12 deficiency. • Tongue may appear fiery or beefy red and the tip and margins show papillary atrophy. • Candidiasis can be precipitated by anaemia. • Apthous stomatitis occasionally presenting feature.
- 26. Periodontal Manifestations • Markedpallor of the gingiva.
- 27. General Management • Parenteralcourse of Vitamin B12 sufficient to provide daily requirement of 2 to 5 µg until abnormality is corrected. • This regimen is followed by monthly intra muscular injections ( 50 to 100 µg) for the rest of patients life.
- 28. Dental Considerations • Localanaesthesia satisfactory. • Conscious sedation- Nitrous oxide is contraindicated. • General anaesthesia should be postponed until a low haemoglobin has been remedied.
- 29. Folic Acid DeficiencyAnaemia • Needed by the body to • synthesize and breakdown amino acids • Synthesize DNA / RNA • Essential for the maturation of erythrocytes. • Laboratory picture is that of megaloblastic anaemia.
- 30. Folic Acid DeficiencyAnaemia CAUSES • Mostly dietary deficiency • Malabsorption • Coeliac disease and Crohn’s disease • Increased demands • Pregnancy • Patients under going long term renal dialysis • Drugs • Alcohol- interferes with metabolism and absorption of folic acid. • Barbiturates • Phenytoin
- 31. General Features • Folatedeficiency leads to anaemia • fatigue • dizziness • feeling cold • irritability • headache • difficulty breathing • pale skin • GI symptoms such as • Nausea, vomiting • Abdominal pain • Diarrhea, especially after meals • Anorexia also is common. • In combination with the above symptoms, may lead to marked weight loss.
- 32. Oral Manifestations • Inearly deficiencies • Soreness of tongue without depapillation or colour change • In severe anaemia • Atrophic Glossitis is the best known effect • Angular Stomatitis is also a well known sign
- 33. General Management • Oralfolic acid (1 mg tablet daily) is effective in reversing megaloblastic anaemia caused by folate deficiency. • If the underlying cause of deficiency is irreversible (e.g. intestinal disease), maintenance therapy is necessary. • The patient should be given education emphasizing the origin of the dietary deficiency.
- 34. Dental Considerations • Localanaesthesia is satisfactory. • Conscious sedation can be given if the haemoglobin level is only moderately depressed and supplemental oxygen can be given. • General Anaesthesia should be postponed until a low haemoglobin has been remedied.
- 35. Aplastic anaemia • Pancytopeniawith a non functioning bone marrow. • Rare disease causing leucopenia, thrombocytopenia and refractory anaemia. CAUSES • Idiopathic • Chemicals such as benzene • Drugs • Hepatic viruses • Irradiation
- 36. General features Features ofanaemia • Purpura – usually the first manifestation • Suseptibility to infection • Suseptibility to bleeding
- 37. Oral manifestations • Ulceration •Haemorrhagic tendencies • Suseptibility to infections • Oral lichenoid reactions ulceration Bleeding tendencies Lichenoid reaction
- 38. General Management • Firstline treatment for aplastic anemia consists of immunosuppressive drugs, typically either anti- lymphocyte globulin or anti-thymocyte globulin, combined with corticosteroids and cyclosporine.
- 39. Dental Considerations • Treatmentmodifications need to take into consideration: • Anaemia • Haemorrhagic tendencies • Susceptibility to infections • Effects of corticosteroid therapy • Hepatitis B and other viral infections • Local anaesthesia is satisfactory. • Conscious sedation and general anaesthesia should be avoided
- 40. Periodontal Considerations • Gingival enlargementis a complication of cyclosporine therapy.
- 41. Polycythemia • Polycythemia maybe defined as an abnormal increase in the erythrocyte count in the peripheral blood, usually accompanied by an increase in hemoglobin. • Opposite of anaemia • RBC count and Hb concentration both rise.
- 42. Polycythemia • It canbe: • Primary aka Polycythemia Vera • Due to malignant changes in erythrocyte producing cells. • Secondary • Where there is an excess need of RBCs for survival. Eg at high altitudes, COPD and cyanotic heart diseases.
- 43. Systemic features • Headache •Dizziness • Vertigo • Tinnitus • Hemorrhages • Ruddy cyanotic skin seen on the face and extremities • Splenomegaly is a common finding on physical examination.
- 44. General Management • Goalof medical therapy is to reduce erythroid over production and to lower blood viscosity. • When the hematocrit rises above 60%, blood is removed usually about 500 ml initially, by phlebotomy every 2 to 3 days until hematocrit is controlled. • Thereafter ,maintenance therapy consists of long term phlebotomy or myelosuppressive therapy. • Long term myelosupression by chemo therapy or irradiation is efficient.
- 45. Oral manifestations • Facialplethora (red florid complexion) is a cardinal sign particularly evident around the ears, nose and lips. • Oral mucosa may have cyanotic or purplish appearance.
- 46. Oral manifestations • Bleeding tendenciesare evident as • petechiae, • hemorrhages • or prolonged bleeding following extractions or periodontal surgery.
- 47. Periodontal Manifestations • Thegingivae appears congested and edematous and may bleed spontaneously because of hyperviscosity and hypertension. • Gingival enlargement may be seen.
- 48.
- 49. Hereditary Spherocytosis • alsoknown as Minkowski– Chauffard syndrome • Intrinsic defect in RBC membrane that renders it spheroidal, less deformable and vulnerable to spleenic sequestration and destruction. • Mutations disrupt vertical interactions between spectrin meshwork and intrinsic membrane proteins.
- 50.
- 51. General features Signs andsymptoms are highly variable • Include mild pallor • Intermittent jaundice • Splenomegaly.
- 52. Oral Manifestations • Manifestin the mucosa as deposition of blood pigments during episodes of jaundice or in acute infections. • Severe dental infection may trigger an attack requiring hospitalisation.
- 53. General Management • Splenectomyis frequently performed and is invariably successful in correcting the haemolysis of erythrocytes. • Gall Bladder is also removed if symptomatic or diseased. (affected in about half of the patients).
- 54. Disorders of Hb Hbabnormalities Hereditary Hemoglobinopathies Porphyrias Thalassemias Acquired Carboxy hemoglobenemia Methemoglobenemia
- 55. Haemoglobinopathies • They aregenetically determined disorders of haemoglobin production. • Each of the haemoglobin peptide (globin) chains has a unique amino acid sequence that can be altered as a result of DNA mutations. • Haemoglobinopathies are mostly seen in non – caucasians.
- 56. Sickling Disorders • Itis an autosomal recessive disorder. • Erythrocytes distort into cresent or sickle shape. • HbS haemoglobin present instead of HbA. • 2 α chains as usual but in the β chains, in position 6, valine instead of glutamic acid is present. • Sickling of RBCs leads to stasis, hemolysis and microthrombosis of blood.
- 57. General Features 6 mainproblems: • Painful Crises • Usually due to infarction brought on by infection, dehydration, hypoxia, acidosis or cold. • Form mainly in spleen, bones and joints, brain, kidney, lungs, retinae and skin. • Haematological crises • Caused by parvovirus infection. • Can be: haemolytic, aplastic or sequestration. • Chronic Anaemia • Chronic hyperbilirubinaemia • Infections- pnuemococci, meningococci, haemophilus and salmonellae • Sequestration syndromes
- 58. Oral Manifestations • Pallorof the oral mucosa. • Slow tooth development and eruption. • Hypoplasia of enamel secondary to anemia.
- 59. Radiographically, • “Step ladder” trabecularpattern • Lamina Dura is more distinct • Calcified pulp canals • Hypercementosis may be seen.
- 60. General Management • Painfuland febrile crises of sickle cell anemia are managed with analgesics, sedatives, bed rest and fluids. • When vaso- occluson develops, transfusion therapy is advocated. • Whole blood or packed cells are used to increase the erythrocyte count and to promote tissue oxygenation.
- 61. Dental Considerations • Sicklecell anaemia with Hb less than 11 gm/dl presents a hazard for general anaesthesia. • Local anaesthesia is the preferred mode for pain control. • Conscious sedation can be used safely. • Preferable to avoid Prilocaine which in overdose may cause methaemoglobinaemia. • Aspirin is best avoided- may cause acidosis in large doses. • Prophylactic antibiotics should be given and infections may be treated vigorously. • Elective surgery should be carried out in the hospital and the anaemia should be corrected pre operatively with the Hb brought upto 10gm/dl.
- 62. Thalassaemias • These areautosomally dominant, inherited disorders in which either alpha or beta globin chains are synthesized at a low rate, thereby lessening the production of Hb A. • Characterized by hypochromic microcytic anaemia. • They may be • Severe- major thalassemia • Mild – minor thalassemia • May affect • alpha chain- alpha thalassemia • Beta chain- beta thalassemia
- 63. Beta- Thalassemia • Resultsfrom depressed production of beta chains. • Homozygous beta thalassemia (Cooley’s anaemia or thalassemia major) is characterized by: • Increasingly severe anaemia • Hepatosplenomegaly • Skeletal Abnormalities • Heterozygotes for beta thalassemia may be asymptomatic.
- 64. Oral manifestations • Marked overdevelopmentof maxilla and mandible. • The osseous changes cause • prominent cheekbones, • sunken root of the nose, • labial inclination of the maxillary incisor, • inadequate lip seal. These lead to the description “chipmunk” or “rodent facies”. • The oral mucosa may be pale, owing to anaemia or yellow tinged due to jaundice.
- 65. General Management • Treatmentincludes blood transfusions to temporarily control anemia. • Repeated transfusions accelerate development of electromyocardial instability. • Iron chelating agents have been used to improve serum iron balance in these patients. • In select cases, splenectomy may be helpful. • Prognosis for thalassemia major is unfavorable and no successful therapy is available. • Death usually occurs early. • Thalassemia minor often requires no specefic therapy unless haemoglobin levels drop.
- 66. Dental Considerations • Patientsundergoing repeated transfusions are at a risk of Hepatitis B, C and HIV carriage. • Since splenectomy results in an immune defect, it is prudent to cover surgical procedures with prophylactic anti- microbials. • Local anaesthesia is safe. • Conscious sedation may be given with oxygen levels not less than 30%. • General anaesthesia may be complicated by enlargement of the maxilla (difficulty in entubation).
- 67. Periodontal Considerations • Severegingivitis is evident if the patient is subjected to splenectomy. • The colour of gingiva sometimes tends to be dark due to high ferritin levels in the blood. • Periodontitis, gingivitis etc. are very dangerous foci of infection in these immune impaired individuals. This situation is worsened in the presence ofDental considerations in Thalassemic patients Dr Sakshi Madhok, Dr Saksham Madhok. (IOSR- JDMS)Volume 13, Issue 6 Ver. IV (Jun. 2014), PP 57-62.
- 68.
- 69.
- 70. Leukaemias • Leukemias arepotentially lethal diseases in which there is neoplastic proliferation of bone marrow WBCs. • They are classified by: • Clinical course- acute or chronic • Cell of origin- lymphoblast or non lymphoblast • Dental management of such patients is often complicated by bleeding tendencies and susceptibility to infection.
- 71. Acute Lymphoblastic Leukemia • Mostcommon malignant disease of children. • Usually in childhood but can affect any age group. CAUSES • Ionizing radiation • Exposure to benzene
- 72. Clinical features • Anaemia •Lymphadenopathy • Splenomegaly • Infections • Fever • Bruising • Bleeding tendencies
- 73. Oral manifestations • Oralbleeding and petechiae are typical manifestations • Oral pallor • Gingival swelling (sometimes) • Mucosal or gingival ulceration • Pericoronitis • Cervical lymphadenopathy • Oral and Peri- oral Herpetic infections • Candidiasis caused by Candida albicans
- 74. General Management • Treatmentof choice is with prednisolone. • Other anti leukemic agents like anthracycline antibiotic are added if needed.
- 75. Dental considerations • Preventiveoral health care is essential and conservative dental treatment may be possible. • Surgery should be deferred except in emergencies . • Regional local anaesthetics contra indicated if there is severe haemorrhagic tendencies. • Conscious sedation is usually possible. • Anaemia is a contradiction to general anaesthesia however iv sedation may be used. • Because of dangers of infection and hemorrhage desmopressin or platelet or blood infusions may be needed pre operatively. • Antibiotic coverage should be started pre operatively and continued till the wound heals. • Any extraction sockets should not be packed as they predispose to infection. • Aspirin and other NSAIDs should not be given since they can aggravate bleeding.
- 76. Acute Myeloid Leukemia •Most common leukemia in adults CLINICAL FEATURES • Anaemia • Lymphadenopathy • Splenomegaly • Infections • Fever • Bruising and bleeding tendencies • Night sweats • Lethargy • Weight loss
- 77. Oral manifestations • Oralbleeding and petechiae are typical manifestations • Mucosal pallor • Mucosal or gingival ulceration • Pericoronitis • Cervical lymphadenopathy • Gingival swelling (sometimes)- occurs due to abnormal response to dental plaque
- 78. General Management • Lessresponsive to therapy and continues to be lethal for the majority affected. • Remission is the result of intensive treatment with cytosine arabinoside, together with anthracycline antibiotic, daunorubicin and sometimes methotrexate.
- 79. Dental considerations • Preventiveoral health care is essential and conservative dental treatment may be possible. • Surgery should be deferred except in emergencies . • Antibiotic coverage should be started pre operatively and continued till the wound heals.
- 80. Chronic Lymphocytic Leukemia• Primarydisease of older persons. • The cause of CLL remains unknown. CLINICAL FEATURES • Many are asymptomatic. • In others, • Fatigue • Fever • Weight loss • Anorexia • Lymphadenopathy • Haemorrhage
- 81. Oral manifestations • Gingivalbleeding • Oral petechiae • Oral ulceration • Herpes simplex infection may occur • Candidiasis is common • Bleeding tendencies • Liability to infections
- 82. General Management • Thechemotherapeutic agent used in the treatment of CLL is the alkylating agent chlorambucil. • This agent may be given with prednisone, especially if autoimmune hemolytic anemia and thrombocytopenia are present. • This therapy reduces nodal and spleen size and improves the quality of life.
- 83. Dental considerations • Localanaesthesia is satisfactory. • Conscious sedation may been given. • General anaesthesia should be carried out in a hospital.
- 84. Chronic Myeloid Leukemia •Most dramatic white cell disorder. • Proliferation of myeloid cells in the bone marrow, peripheral blood and other tissues. CLINICAL FEATURES • Anaemia • Weight loss • Joint pains • Splenomegaly • Hepatomegaly • Rarely lymphadenopathy
- 85. Oral manifestations • Pallorof mucosa due to anaemia • Disturbance of taste • Xerostomia • Oral hemorrhages • Swelling of salivary glands
- 86. General Management • Treatmentof CML is with the alkylating agent busulfan or with hydroxyurea. • These cytotoxic drugs reduce peripheral blood counts and spleen size, making the patient less symptomatic.
- 87. Dental considerations • Treatmentplanning should take into consideration: • Bleeding tendencies • Liability to infection • Anaemia • Susceptibility to hepatitis B, C and HIV infection.
- 88. Periodontal Considerations • Periodontalmanifestations consist of leukemic infiltration, gingival bleeding. Their expression is more common in acute forms of leukemia than in chronic forms. LEUKEMIC INFILTRATION • Leukemic cells can infiltrate the gingiva and less frequently the alveolar bone. • Gingival infiltration results in leukemic gingival enlargement. • Clinically, the gingiva appears: • Bluish red and cyanotic • Rounding and tenseness of the gingival margin • Increase in size, mostly in the interdental papilla & partially covering the crowns of the teeth..
- 89. Periodontal Considerations BLEEDING • Gingivalhemorrhage is a common finding in leukemic patients. • It is caused by the thrombocytopenia resulting from replacement of bone marrow cells by leukemic cells and from the inhibition of normal stem cell function by leukemic cells
- 90. Leukemic gingival enlargement• Mostimportant oral sign of leukemia. • True enlargement occurs in acute leukemia and seldom in chronic leukemia. • It may be • diffuse or marginal • localized or generalized. • Normal contours of gingiva are lost. • It may appear as • A diffuse enlargement • An oversized extension of the margins • A discrete tumor like interproximal mass
- 91. Leukemic gingival enlargement • Stipplingis replaced by spongy, swollen tissue that tends to peel away from the teeth and bleed easily. • The consistency is moderately firm, bit there is a tendency towards friability. • Progressive disease produces purple, shiny and hemorrhagic gingiva. • Purpuric features like petechial lesions and ecchymosis are common.
- 92. LYMPHOMAS • Malignant lymphomasare solid neoplastic growths of lymphoid cells that arise in reticuloendothelial and lymphoid tissue. • They spread to distant lymphoid sites, such as liver, spleen and bone marrow. • Originate in the lymph nodes but can arise extra nodally. • Several types of malignant lymphomas occur, however those of primary interest are • Hodgkin’s Lymphoma • Non – Hodgkin’s Lymphoma • Burkitt’s Lymphoma
- 93. Hodgkin’s Lymphoma • Itis a neoplastic disorder originating in lymphoid tissue. • The disease classically begins as a painless, enlarging mass that appears in the neck or mediastinum of adults. • Initially arises at a single site and subsequently spreads to contiguous lymphoid structures. • Characterized by a neoplastic proliferation of large multi nucleated reticulum cells known as Reed Stenberg Cells.
- 94. • Progressive painless swellingof the cervical lymph nodes. • Generalized itching • Cyclic elevations of body temperature • Chills and night sweats • Weight loss
- 95. Non-Hodgkin’s Lymphoma • Heterogenous collectionof 10 different malignant lymphoid diseases. • Produce numerous atypical lymphocytes that obliterate normal lymph nodal architecture.
- 96. General Features • Painless lymphadenopathy– presenting feature. • Enlarged lymph nodes are firm, rubbery and mobile. • Rapid nodal growth may impair lymphatic drainage, resulting in lymphadema, pain, parasthesia and skin infiltrations
- 97. Oral Manifestations • Lymphoidtissue of Waldeyer’s Ring frequently involved site. • Infiltration results in non tender enlargements of tonsillar tissue or the posterior palate. • The surface of these swellings may be erythematous, ulcerated or necrotic.
- 98. Oral Manifestations • Whenpalate is the sole site of primary lymphoma, it is sometimes referred to as lymphoproliferative disease of the palate. • The enlargement is slow growing, soft, spongy and relatively painless. • The surface is non ulcerated and often lumpy, with red surface telangiestasia. • As the disease progresses the colour changes from pink to blue purple
- 99. Periodontal Manifestations • Inrare cases, oral Non- Hodgkins Lymphoma can mimic other, more common disorders and can appear as a gingival mass. • Lymphomas of the gingiva are frequently treated for months as a pulpal or periodontal problem before the correct diagnosis is made.
- 100. Dental Considerations • Preventivedental measures are most important in patients with lymphoma. • Generally speaking, patients in a state of remission can receive routine dental care. • However, patients with acute signs or symptoms of lymphoma should receive only conservative emergency dental care. • When invasive procedures are required in the patient with lymphoma, a bleeding time determination and consultation with oncologist are recommended. • Patient undergoing chemotherapy or radiation therapy should be observed for xerostomia or radiation caries.
- 101.
- 102. Haemostasis • Normal Haemostasis dependson a complex interaction of the blood vessels, platelets, fibrin coagulation and deposition and fibrinolytic proteins. • Heamostasis consists of 3 reactions: • Primary- vascular phase • Secondary- platelet phase • Tertiary- coagulation phase All of which occur at the same time.
- 103. Primary Haemostasis • Primaryhaemostasis is by vasoconstriction after injury. • This reduces the outflow of escaping blood after injury. • Circulating platelets are exposed to subendothelial collagen, which causes the release of intra- cytoplasmic granules that stimulate platelet aggregation and helps to form the soft primary plug. Abnormalities in primary haemostasis • Abnormal platelet number or function • Abnormal von willebrand factor
- 104. Secondary Haemostasis • Theaggregation of platelets is the second phase of haemostasis. • Therefore it is aka ‘the platelet phase’. Abnormalities in secondary haemostasis • Defects in the coagulation cascade cause more serious bleeding than do defects of primary haemostasis. • They include bleeding into cavities (chests, joints, cranium).
- 105. Tertiary Haemostasis • Itis the third and final phase. • It is divided into 2 pathways: • The extrinsic pathway • The intrinsic pathway
- 106. Bleeding Disorders CAUSES Defects inhaemostasis, leading to bleeding disorders can comprise: • Platelet activation and function • Anti – thrombin function • The more common causes include • Warfarin- which interferes with clotting factor production • Vitamin K blockage • Aspirin – one tablet of which impairs platelet function for almost 1 week.
- 107. General Features • Deephaemorrhage into muscles, joints or skin. • Bleeding from and into mucosae and skin (‘bruising’)- suggests purpura.
- 108. Localized Oral Purpura •Blood blisters are occasionally seen, typically in older persons in the absence of obvious trauma. • These blood blisters are often in the palate and may sometimes be 1 cm or more in diameter and after rupture may leave a sore area for a time.
- 109.
- 110. Platelet Disorders • Plateletdisorders may be the result of • alteration in platelet numbers, • either decreased (thrombocytopenia) • increased (thrombocythemia), • altered platelet function (thrombocytopathia). • Platelet disorders may be: • Quantitative- Thrombocytopenic purpura • Qualitative- Disorders of platelet function
- 111. Causes • Failed plateletproduction • Thrombocytopenia • von Willebrand’s disease. • Excessive platelet Destruction • Idiopathic Thombocytopenic Purpura • DIC • Abnormal platelet Function • Abnormal platelet Regulation
- 112. Thrombocytopenic Purpura • Thrombocytopeniais a bleeding disorder characterised by a platelet count below the normal range. • Reduced counts may be due to: • Failure of platelet production • Disordered platelet distribution • Increased platelet destruction. • It may be: • Auto- immune or Idiopathic • Drug induced
- 113. Idiopathic Thrombocytopenic Purpura • Akaauto – immune thrombocytopenic purpura. • ITP is one of the most common causes of thrombocytopenia. • Is auto immune and can lead to purpura and prolonged bleeding.
- 114. Drug Induced Thrombocytopenic Purpura •Reactions of drugs or toxins resulting in a low platelet count and bleeding tendency. • Common offenders are: • Quinidine • Quiunine • Sulfonamides • Heavy alcohol consumption.
- 115. General Features • Hallmarksare: • Abrupt appearance of petechial hemorrhages. • Purpura • Bruises after trauma • Petechiae cover extensive surface area appearing rash like, but are tender and do not itch. • Located chiefly on arms, lower leg, upper chest and neck. • In severe cases- mucosal bleeding of the GIT may occur.
- 116. Oral Manifestations • Includepetechiae • Ecchymoses • Post operative haemorrhage. • Sumucous purpura may be conspicous and sometimes seen as “blackcurrant jelly” blood blisters.
- 117. Dental Considerations • Regionallocal anaesthetic block injections can be given. • Major surgery requires platelets more than 75x 10 9 /l. • Conscious sedation can be given. • General anaesthesia can be given in an hospital. • Avoid aspirin and other NSAIDs.
- 118. Periodontal Considerations • Gingivais soft, swollen and friable. • Bleeding occurs on the slightest provocation and is difficult to control. • The severity of the gingival condition is dramatically alleviated by removal of the local factors.
- 119. CONGENITAL COAGULATION DEFECTS Most importanthereditary bleeding disorders include: von Willibrand’s disease Haemophilia A and B
- 120. Haemophilia • X linkeddisorder resulting from a deficiency in clotting factor VIII (Hemophilia A or classic) or factor IX (Hemophilia B or Christmas Disease) . • Affects males, females are carriers. • 3 forms of hemophilia are seen: • Severe- < 1% of normal factor VIII • Moderate – 1% - 5% • Mild – 5% – 25%
- 121. General Features • Characterizedby severe, persistent bleeding. • Hemorrhage into soft tissue, muscles, organs and joints. Clinically these are prominent as hematomas and hemarthroses. • Excessive bleeding after trauma. Intractable oozing or rapid blood loss one hour or more after injury. • Sometimes spontaneous bleeding.
- 122. Oral Manifestations • Petechiae •Ecchymoses • Spontaneous gingival bleeding can occur • Prolonged massive bleeding even from simple tooth exfoliation or extraction. • Associated with anaemia induced pallor and pseudoaphthae.
- 123. Dental considerations • Localanaesthetic injections or surgery can be followed by persistent bleeding for days. • The hemorrhage cannot be controlled by pressure alone. • Bleeding tendency may be aggravated by NSAIDs. • In all but severe haemophiliacs, non surgical dental treatment can usually be carried out under antifibrinolytic cover. • Extractions and dentoalveolar surgery should be carefully planned. A factor VIII level between 50 and 75% is required. • Mild hemophiliacs requiring such surgeries can be managed usually without factor replacements. Desmopressin and tranexamic acid are primary alternative. • Post operative suturing is desirable. Vicryl sutures are
- 124. Periodontal considerations • Periodontalhealth is of critical importance for the hemophiliac for 2 primary reasons: • Hyperemic gingival contribute to spontaneous and induced gingival bleeding. • Periodontitis is leading cause for tooth morbidity necessitating extractions. • In all but severe hemophiliacs, scaling can be carried out without LA. • Periodontal surgery requires LA and factor VIII replacement to a level between 50 to 75% Naveen Kumar J, Anil Kumar R, Varadarajan R, Sharma N. Specialty dentistry for the hemophiliac: Is there a protocol in place?. Indian J Dent Res 2007;18:48-54
- 125. von Willebrand’s disease •Due to inherited deficiency in vWF. • Most common inherited bleeding disorders. • Affects both males and females.
- 126. General Manifestations • Generally,bleeding manifestations are mild. • Epistaxis • Bleeding from the mucous membrane • Purpura of mucous membrane and skin • Menorrhagia in females
- 127. Oral Manifestations • Gingivalbleeding is the most common finding- in about 30 – 40% of the diseased. • Uncontrollable bleeding during dental procedures like gingival manipulation and pulpectomy.
- 128. Dental Considerations • Aspirinand NSAIDs should be avoided. • Local infilteration anaesthesia should generally be used. • Nerve block anaesthesia is generally contra indicated because injections delivered into loose alveolar tissue (highly vascular regions) may produce bleeding or a dissecting hematoma that could obstruct the patient’s airway. • Conscious sedation can be given, but care must be taken not to damage the vein. • General anaesthesia must be given in hospital. • Intubation is a possible hazard because of the risk of submucosal bleeding in the airway.
- 129. Periodontal Considerations • Gingivalhaemorrhage is more common than in haemophilia. • Even minor gingival manipulation can lead to uncontrollable bleeding. • Cryoprecipitate or other anti fibrinolytic agents shoulld be administered to minimize bleeding. • Post operative diet should be soft and semi solid to minimize trauma to the gingiva.
- 130. GUIDELINES FOR ORAL HEALTHCARE OF PATIENTS WITH HEMATOLOGIC DISEASE
- 134. Guidelines for Hemorrhagic DentalProcedures • Should be avoided when patients conditions are poorly controlled. • Provide prophylactic antibiotics to prevent infection when open wounds are present. • Obtain pre operative CBC, DLC, platelet count, bleeding time, PT and APTT. Any abnormal values mandate a consultation. • Oral surgical procedures should be done only if absolutely necessary and in a hospital setting. • Single dental extractions should be performed as opposed to multiple extractions. • Pay meticulous attention to good technique and primary closure.
- 135. Guidelines for Dental Emergencies •Pallative emergency treatment only. • Dental infections should be treated aggressively with antibiotics and drainage. • Surgical procedures should be avoided until control of disease can be assessed with blood profiles. • Non – Aspirin or Non- NSAID containing drugs should be prescribed as post operational analgesics. • If the patient comes back with bleeding- • Debride the wound site and remove any clot. • Control the bleeding by packing the socket with absorbable microfibrillar collagen or thrombin soaked gauze.
- 136. Conclusion • Recording aproper history including past medical and dental history and family history of the patient goes a long way in helping the clinician in managing patients with bleeding disorders. • It not only helps in reaching to a proper diagnosis of the patient’s condition but also in forming a holistic treatment plan that is beneficial for the patient. • Knowledge of the signs and symptoms, both oral and systemic of these diseases enable the clinician in diagnosing cases in which the patient does not have any information about the presence of such disease.
- 137. References 1. Concise medicalphysiology, Chaudhuri, IV edition. 2. Robbin’s Basic Pathology, Seventh Edition. 3. Medical Problems in Dentistry; Crispian Scully, Roderick A. Cawson; V edition. 4. Carranza’s Clinical Periodontology, X edition. 5. Color Atlas of Oral Diseases; George Laskaris; III edition 6. Oral Diagnosis, Oral Medicine and Treatment Planning, Bricker, Langlais, Miller, II edition. 7. Impact of iron deficiency anemia on chronic periodontitis and superoxide dismutase activity: a cross-sectional study. Souvik Chakraborty, Shikha Tewari1, Rajinder Kumar Sharma, Satish Chander, Narula1, Pratap Singh Ghalaut, Veena Ghalaut. Journal of Periodontal Implant Sci 2014;44:57-64. 8. Naveen Kumar J, Anil Kumar R, Varadarajan R, Sharma N. Specialty dentistry for the hemophiliac: Is there a protocol in place?. Indian J Dent Res 2007;18:48-54 9. Dental considerations in Thalassemic patients Dr Sakshi Madhok, Dr Saksham Madhok. (IOSR-JDMS)Volume 13, Issue 6 Ver. IV (Jun. 2014), PP 57-62.
Editor's Notes
- #7 Formed elements(because they have definite form under the microscope)
- #8 No nucleus- cant divide, no ribosome- no prtn sysnthesis, no mitochondria- no TCA cycle so no ATP production
- #9 Haem is an iron containing compound belonging to class of compounds- protoporphyrins. Globin belongs to a class of prtns called globulins. So Hb is a conjugated prtn. Globin molecule has 4 polypeptide chains- 2 alpha chains (141 amino acids) and 2 beta chains (146). With each chain one molecule of haem is attached. Each haem molecule can combine with one molecule of oxygen… thus one hb molecule can combine with 4 molecules of O. In red blood cells, the enzyme carbonic anhydrase catalyzes the conversion of dissolved carbon dioxide to carbonic acid, which rapidly dissociates to bicarbonate and a free proton: CO2 + H2O → H2CO3 → H+ + HCO3-. Histidine residues in hemoglobin can accept protons and act as buffers. Deoxygenated hemoglobin is a better proton acceptor than the oxygenated form.
- #11 Granulocytes- cytoplasm has granules neutrophils-halmark of acute inflammation-attack and destroy the invading bacteria by producing oxidants h2o2; lowering pH within the bacteria - Eosinophils- are immune system components responsible for combating multicellular parasites- coarse eosinophilic granules Basophils- s shaped nucleus, Basophils appear in many specific kinds of inflammatory reactions, particularly those that cause allergic symptom Lymphocyte- include natural killer cells (NK cells) (which function in cell-mediated, cytotoxic innate immunity), T cells (for cell-mediated, cytotoxic adaptive immunity), andB cells (for humoral, antibody-driven adaptive immunity). They are the main type of cell found in lymph, which prompted the name lymphocyte. Monocyte- part of innate immune system-Monocytes have bean-shaped nuclei that are unilobar.
- #12 heamatologic diseases that have potential for significant health problems can be categorised into 3 main categories
- #15 Anemia is the most common disorder of the blood affecting about a quarter of people globally. Anemia is a decrease in amount of red blood cells (RBCs) or the amount of hemoglobin in the blood resulting in a decrease in the oxygen carrying capacity of the blood. The name is derived from Ancient Greek anhaimia, meaning "lack of blood", from an-, "not" + haima, "blood". A pt is considered anemic if his Hb is less than 12 gm% or RBCs are less than 5 million/cumm
- #16 Vit b9 = folic acid Vit b 12= cyanocobalamin
- #19 Carp mouth- narrowed mouth opening
- #23 It is a disease of late adult life resulting from def of vit B 12. It is a form of megaloblastic anaemia. Megaloblastic anemia is an anemia (of macrocytic classification) that results from inhibition of DNA synthesis during red blood cell production.
- #24 Sources- eggs, lean meat, green leafy veggies, milk
- #29 Nitrous oxide administration for more than 12 hrs interferes with vit b 12 metabolism and neurological function
- #39 Thymocytes are hematopoietic progenitor cells present in the thymus.[1] The primary function of thymocytes is the generation of T lymphocytes (T cells) Thymopoiesis is the process in the thymus by which thymocytes differentiate into mature T lymphocytes.
- #44 An ecchymosis is a subcutaneous extravasation of blood larger than 1 centimeter. Purpura measure 0.3–1 cm (3–10 mm), whereas petechiae measure less than 3 mm, and ecchymoses greater than 1 cm.
- #47 An ecchymosis is a subcutaneous extravasation of blood larger than 1 centimeter. Purpura measure 0.3–1 cm (3–10 mm), whereas petechiae measure less than 3 mm, and ecchymoses greater than 1 cm.
- #50 In HS rbcs hav reduced membrane stability and consequently lose membrane fragments after their release in to the periphery while retaining most of its volume. As a result their surface area to volume ratio decreases until they assume the smallest possible diameter for a given volume ie a sphere. Spleenic destruction occurs because normal rbcs have discoid shape which allows changes in cell shape but in HS rbcs are spheroidal and have less membrane plasticity and they cant leave the spleenic cords… whr they are destroyed by macrophages.
- #51 In HS rbcs hav reduced membrane stability and consequently lose membrane fragments after their release in to the periphery while retaining most of its volume. As a result their surface area to volume ratio decreases until they assume the smallest possible diameter for a given volume ie a sphere. Spleenic destruction occurs because normal rbcs have discoid shape which allows changes in cell shape but in HS rbcs are spheroidal and have less membrane plasticity and they cant leave the spleenic cords… whr they are destroyed by macrophages.
- #57 When exposed to hypoxia, it produces crystalls called tectoids, within the RBCs. This leads to increased fragility of the RBC.
- #58 Spleenic infarcts lead to auto spleenectomy Infections due to spleenic infarction Sequestration syndromes in the chest and in spleen
- #63 Alpha th- deficiency of alpha chains.in major alpha thalessemia- No compensatory mech for loss in alpha chain production so tetramers of the beta and gamma chains combine to form Hb H and Barts both of which are ineffective oxygen carriers. Thus this condition is lethal in utero
- #87 Busulfan suppresses platelet count. Less in Hydroxyurea
- #124 Vicryl sutures were used, as it avoids need for the postoperative removal [11] and the possibility of bleeding when the suture is removed. Catgut may react.