Oral Aspects of Hematological Disorders
Question 1. What is anemia?
Answer. Anemia is a hematological disorder characterized by either decrease in the number of red blood cells (RBCs) or the blood hemoglobin, resulting in a lack of ability for the blood to carry oxygen to body tissues.
Question 1a. Name the most common anemia among Indians.
Answer. Iron deficiency anemia.
Read And Learn More: Oral Pathology Short Notes Question And Answers
Question 1b. What is pancytopenia?
Answer. An abnormal deficiency in all types of blood cells (red blood cells, white blood cells and platelets) is known as pancytopenia. It is usually associated with conditions like bone marrow tumor or with aplastic anemia).
Question 1c. In which organ, heme component of hemoglobin is converted into bilirubin?
Answer. Kidney.
Question 1d. What is pernicious anemia?
Answer. Pernicious anemia is a type of chronic, progressive, megaloblastic anemia characterized by impaired RBC maturation; secondary to insuffiient vitamin B12 due to defective intrinsic factor required for its absorption in the stomach.
Question 2. What are the clinical features of pernicious anemia?
Answer. There are four major cardinal features of pernicious anemia:
- Abnormally large red blood cells
- Hypochlorhydria
- Neurologic and gastrointestinal symptoms
- A fatal outcome, unless the patient receives lifelong injections of vitamin B12.
Question 3. Name the oral manifestations of pernicious anemia.
Answer.
- Beefy red tongue with glossodynia (painful tongue)
- Loss of taste sensation and glossopyrosis (itching and burning tongue)
- Hunter’s glossitis, pallor with burning sensation in the oral mucosa and dysphagia
- Increased susceptibility to oral infections.
Question 4. What is Hunter’s glossitis?
Answer. Pernicious anemia often causes atrophy and inflmmation of the fiiform papillae and produces a bald appearance of the tongue; this condition is often known as Hunter’s glossitis.
Question 5. What is Schilling’s test?
Answer. Schilling test is a medical investigation used for patients with vitamin B12 defiiency. Schilling’s test detects the absence of intrinsic factor in the stomach and helps to determine whether the patient has pernicious anemia.
Question 6. What is the treatment of pernicious anemia?
Answer. Intramuscular injections of vitamin B12.
Question 7. What is iron deficiency anemia?
Answer. Iron deficiency anemia is a chronic, microcytic, hypochromic type of anemia, which occurs either due to inadequate absorption or excessive loss of iron from the body.
Question 8. What are the causes of iron deficiency anemia?
Answer.
- Inadequate absorption of iron in the body
- Excessive blood loss
- Increased demand for RBC
- Decreased intake of iron in the body.
Question 9. Describe the general clinical features of iron deficiency anemia.
Answer.
- Fatigue, palpitations, dizziness and sensitivity to cold
- Lemon-tinted palor of the skin and generalized weakness
- Koilonychia
- Splitting of hair is also commonly seen.
Question 10. What is koilonychia?
Answer. Koilonychia (spoon-shaped figer nails) is an important feature in iron deficiency anemia and the patients often exhibit increased brittleness and cracking of the figer nails.
Question 11. What are the oral manifestations of iron deficiency anemia?
Answer.
- Mucosal pallor with loss of keratinization (atrophic mucositis)
- In some patients, atrophic mucositis in the aerodigestive tract or oropharynx may predispose to the occurance of squamous cell carcinoma
- Depapillations and mucosal atrophy produce smooth, bald, red and glazed appearance of the tongue
- Often there is burning sensation in the tongue (glossopyrosis) and glossodynia (painful tongue)
- Angular chelitis, dysphagia (diffiulty in swallowing), recurrent aphthous ulcer and candidiasis of the oral mucosa
- Plummer-Vinson syndrome presents a triad of symptoms comprising of dysphagia, stomatitis (inflmmation of the oral mucosa) and atrophic glossitis.
Question 12. In which age and sex group iron deficiency anemia is more common?
Answer. This syndrome is more prevalent in middle-aged woman and these patients are susceptible to oral cancers and pre-cancers.
Question 13. What is the greatest risk in iron deficiency anemia?
Answer. In Iron deficiency anemia, atrophic mucositis in the aerodigestive tract or oropharynx may predispose to the occurrence of squamous cell carcinoma.
Question 14. Name the common investigations done in iron defiiency anemia?
Answer.
- Peripheral blood smear shows small (microcytic) and pale (hypochromic) red blood cells
- Hemoglobin level is decreased to as low as 3.6 g/100 mL
- Total RBC count is dropped moderately, but rarely does it go down below 3 million per mm3
- MCV, MCH and mean cell hemoglobin concentration (MCHC)—all are reduced
- Serum iron level is decreased to 10 mg (normal 50–150 mg)
- Hemosiderin is completely absent from the bone marrow. If GI tract bleeding is the suspected cause of iron defiiency anemia, then the following additional investigations are to be done:
- X-rays (GI tract series), stool examination for occult blood, esophagoscopy, gastroscopy and sigmoidoscopy, etc.
Question 15. What is aplastic anemia?
Answer. Aplastic anemia is a rare life threatening hemorrhagic disease characterized by general lack of bone marrow activity; that results in decreased formation of RBC, WBC and platelet cells.
Question 16. What the bone marrow exhibits in aplastic anemia?
Answer. Bone marrow shows lack of maturation of the hemopoietic stem cells.
Question 17. What are the causes of aplastic anemia?
Answer.
- Hereditary factor e.g. Fanconi’s syndrome
- Drugs and chemicals such as chloramphenicol, phenylbutazone, sulfonamides, DDT and benzene, etc.
- Radiation
- Infections—tuberculosis, viral hepatitis
- Idiopathic.
Question 18. Name the general manifestations of aplastic anemia.
Answer.
- Weakness, fatigue, marked pallor of the skin with petechiae
- Frequent episodes of epistaxis and severe, fatal hemorrhages
- Bruises, numbness and tingling of the extremities
- Generalized edema of the body, dyspnea and tachycardia
- Fever and severe infections due to neutropenia.
Question 19. Describe the oral manifestations of aplastic anemia.
Answer.
- Petechiae, ecchymoses, extreme pallor and frank hematoma in the oral mucosa
- Spontaneous gingival bleeding and occasional gingival hyperplasia
- Epistaxis with fatal hemorrhage following minor oral surgical procedures
- Multiple areas of ulcerations in the oral mucosa, gingiva, and pharynx. Periphery of the ulcer shows minimal erythema
- Persistent oral infections including candidiasis.
Question 20. What are the laboratory fidings in aplastic anemia?
Answer.
- RBC count is usually below 1 million/mm3
- WBC count may be as low as 2000/mm3
- Platelet count may fall below 20000/mm3
- Bone marrow is fatty and contains only few developing blood cells
- Bleeding time prolonged, but clotting time is normal.
Question 21. What is hemolytic anemia?
Answer. Anemia occurring due to increased hemolysis in the body which the bone marrow cannot compensate.
Question 22. How the bone marrow reacts in case of recurrent hemolysis?
Answer. A compensatory erythroid hyperplasia of the bone marrow occurs, which enhances the rate of erythropoiesis or the production of RBC.
Question 23. What are the hereditary causes of hemolytic anemia?
Answer. Hereditary conditions causing destruction of RBC and leading to the development of hemolytic anemia as follow:
- Hereditary spherocytosis
- Glucose 6–phosphate dehydrogenase defiiency (G6PD)
- Sickle-cell anemia
- Thalassemia.
Question 24. What are the acquired causes of hemolytic anemia?
Answer.
- Antibody-mediated hemolytic disorders:
- Acquired autoimmune hemolysis
- Paroxysmal cold hemoglobinuria
- Erythroblastosis fetalis
- Hemolysis due to physical, chemical or biological agents
- Physical agents, e.g. prolonged physical exercise
- Chemical agents and drugs
- Biological agents.
Question 25. Name some chemical agents, which cause hemolysis.
Answer.
- Arsenic, lead, potassium chlorate and naphthalene
- Sulfonamides, methyldopa and mefenamic acid.
Question 26. Name few microorganisms which can cause hemolysis.
Answer.
- Plasmodium falciparum
- Clostridia
- Streptococcus pyogenes.
Question 27. What is thalassemia?
Answer. Thalassemias are a group of inherited, chronic, hemolytic disorders which are characterized by the production of extremely thin, fragile erythrocytes.
Question 28. What are the target cells?
Answer. In thalassemia extremely thin and fragile erythrocytes are produced which are called target cells.
Question 29. Why anemia develops in thalassemia?
Answer. In thalassemia, the fragile erythrocytes in the peripheral circulation are readily destroyed by the spleen within few days after their formation and as a result anemia develops.
Question 30. What is the basic underlying pathology in thalassemia?
Answer.
- In thalassemia, there is insuffiient synthesis of α- and β-polypeptide chains of hemoglobin which lead to the formation of very fragile RBCs (target cells) and as these cells are readily destroyed by the spleen, patients often develop microcytic hypochromic type of anemia
- Moreover, as the spleen is always overburdened with the job of destroying the abnormal RBCs the organ quickly becomes inactive
- Repeated blood transfusions are necessary in patients with thalassemia and that itself can cause excessive iron deposition in various body tissues (hemosiderosis)
- The later may lead to dysfunction of many glands and other vital organs.
Question 30a. What are the characteristics of RBCs in thalassemia?
Answer. The RBCs are generally microcytic and hypochromic in nature.
Question 31. Name the different types of thalassemias.
Answer. In thalassemia either α or β chain synthesis is disturbed in the blood hemoglobin of the patient; on the basis of that thalassemias are divided in two types:
- α-thalassemia: If α-chain synthesis is disturbed (also called thalassemia minor)
- β -thalassemia: If β-chain synthesis is disturbed (also called thalassemia major)
- Thalassemia intermedia: A rare type with features of intermediate severity.
Question 32. What is Cooley’s anemia?
Answer. β-thalassemia is known as Cooley’s anemia; moreover as this β-thalassemia is the more common among all types, it is also called classic or major thalassemia.
Question 33. What are the general clinical features of thalassemia?
Answer.
- Persistent anemia, jaundice and hepatosplenomegaly
- Fever, chills, malaise, generalized weakness and lethargy
- Bone marrow hyperplasia often produces painless enlargement of mandible and maxilla; which often results in a typical chipmunk facies
- Cholelithiasis and leg ulcers also develop frequently
- Most of the patients have a mongoloid facies with prominent forehead, depressed nasal bridge, prominent cheekbones, protrusion of the maxillary anterior teeth and slanting eyes, etc
- Severe form of the disease arising during infancy may terminate in death as early in the adolescence. High output cardiac failure is often the cause of death in such patients.
Question 34. Describe the oral manifestations of thalassemia.
Answer.
- Bimaxillary protrusion of jaws with spacing in the anterior maxillary teeth.
- Sometimes severe malocclusion due to enlargement of the jaws with marked open bite.
- Prominent malar bones, mongoloid facies and retracted upper lips
- Depressed nasal bridge and prominent cheek
- Delayed pneumatization of maxillary sinuses.
- Pallor of the oral mucosa and dry mouth
- Discoloration of teeth due to iron deposition.
Question 35. How the skull radiograph appears in thalassemia?
Answer. The skull bone radiographically exhibits thin, poorly defied, inner and outer cortex of bone and the trabaculae between them are coarse, elongated, and bristle-like; this often produces a typical hair-on-end or a crew-cut appearance on the surface of the skull.
Question 36. What is salt-and-pepper radiographic effect in thalassemia?
Answer. In thalassemia sometimes the jaw bone radiograph exhibits coarsening of some bony trabaculae and blurring or disappearance of others and thus produces a typical radiographic appearance called salt-and-pepper effect.
Question 37. Describe the laboratory fidings in thalassemia.
Answer.
- Presence of target cells and safety pin cells in peripheral blood
- Presence of abnormal nucleated RBCs in blood
- WBC count can rise up to 10,000 to 25,000/mm3 of blood
- The serum bilirubin and fetal hemoglobin levels elevated
- Elevated fecal and urinary urobilinogen due to severe hemolysis
- Bone marrow is hyperplastic and produces large numbers of immature, primitive looking, stem forms of RBCs
- Excessive accumulations of alpha chains within the RBCs are called inclusion bodies (fessas bodies).
Question 38. Name the treatments of thalassemia.
Answer.
- Multiple blood transfusions
- Splenectomy
- Administration of desferrioxamine (chelating agent) to reduce iron overload in tissues.
Question 39. What is sickle-cell anemia?
Answer. Sickle-cell anemia is an inherited defect in the synthesis of hemoglobin molecule; in this disease, the erythrocytes are abnormal (sickle or crescent-shaped RBCs) and are easily destroyed.
Question 40. Describe the clinical features of sickle-cell anemia.
Answer.
- Delayed physical growth, weakness and jaundice with a yellow sclera
- Pallor, loss of appetite, dehydration and muscle rigidity
- Loss of consciousness is a common feature in severe cases (sickle-cell crisis)
- Extreme susceptibility to infections, renal failure and CNS disturbances are common
- There can be death due to widespread ischemia, hypoxia and hypothermia.
Question 41. What are the oral manifestations of sickle-cell anemia?
Answer.
- Oral mucosal polar with occasional yellowish color due to hemolytic jaundice
- Asymptomatic pulpal necrosis, anesthesia and paresthesia of the mandibular nerve
- Extraction of tooth may lead to the development of osteomyelitis especially in mandible
- Thrombosis of blood vessels and infraction in the jawbone often produce painful cries.
Question 42. Mention the important radiographic changes in sicke-cell anemia.
Answer.
- Skull radiographs reveal multiple, small icicle like spicules across the calvarium, which produces a hair-on-end appearance
- Occasionally infracts develop in the jaw bones, which radiographically mimic osteomyelitis
- Intraoral periapical radiographs reveal step-ladder-like trabaculae between roots of contagious posterior teeth.
Question 43. What is erythroblastosis fetalis?
Answer. Erythroblastosis fetalis is a congenital hemolytic anemia of newborn which occurs due to Rh factor incompatibility between mother and fetus.
Question 44. Describe the pathogenesis of erythroblastosis fetalis.
Answer. If the mother is Rh negative and father is Rh positive, then the fetus is more likely to be Rh positive as it is the dominant quality.
In this backdrop if the Rh positive fetal blood mixes up with the Rh-negative mother’s blood via the placenta then there will formation of anti-Rh factor in mother’s blood.
If that anti-Rh factor comes back to the fetus by any chance, then there will be partial agglutination of the fetal blood leading to a severe type of anemia called erythroblastosis fetalis.
Question 45. What are the oral changes in erythroblastosis fetalis?
Answer. Black, brown or bluish discoloration of deciduous teeth in the child due to deposition of blood pigments. Enamel hypoplasia, which may produce a ring-like defect on the tooth crowns (Rh-hump).
Question 46. What is polycythemia vera?
Answer. Polycythemia vera is a chronic disease characterized by excessive proliferation of RBCs, usually with an increased hemoglobin level and total blood volume.
Question 47. Among which people polycythemia vera is more common?
Answer. It is particularly more common among Jewish people.
Question 48. Describe the clinical features of polycythemia vera.
Answer.
- Dyspnea, fullness of head and face, pruritus, fatigue and visual disturbances
- Plethoric appearance of face, slurring of speech, splenomegaly, coronary thrombosis and paresthesia of the cranial nerves, etc.
- Peptic ulcers, epigastric pain along with intestinal, nasal or cerebral hemorrhages
- A characteristic fiding of the disease is the ruddy cyanosis or a reddish-purple hue of the face, extremities and lips due to the presence of deoxygenated blood in the cutaneous vessels.
Question 49. Describe the oral manifestations of polycythemia Vera.
Answer.
- Deep purplish-red discoloration of the oral mucosa; especially in the tongue, gingiva, cheek and lip
- Engorged and swollen gingiva with extreme bleeding tendency
- Submucosal petechiae, hematoma and ecchymoses.
Question 50.Describe the laboratory fidings in polycythemia Vera.
Answer.
- RBC count as high as 8 to 12 million/mm3 of blood
- Increase in the total blood volume and blood viscosity
- Hemoglobin level may be high (18 to 20 g/100 mL)
- Greater hematocrit level with thrombocytosis and leukocytosis.
Question 51. Describe the treatment of polycythemia vera.
Answer.
- Repeated phlebotomy (venesection) to lower the Hb%, hematocrits and RBC mass.
- 500 mL of blood is removed every 2 to 3 days till the hematocrit reaches a desired level
- Chemotherapy with chlorambucil, melphalan or cytosine arabinoside to reduce the number of RBCs.
Question 52.Define leukemia.
Answer. Leukemia is a malignant disease of the blood-forming organs, characterized by increased proliferation of WBCs in the bone marrow at the cost of other hemopoietic cells (WBC count rise, while RBC and platelet count fall).
Question 53. Describe the impact of leukemia on other hemopoietic cells (cells other than WBCs).
Answer. Although leukemia is a disease of the WBCs; it often severely affects the other major cell types of blood e.g. RBCs and platelets.
- Leukemia causes anemia—due to decreased production of RBCs cells in the bone marrow.
- Leukemia also causes increased tendency for hemorrhage—due to reduced number of platelets in the blood.
Question 54. Why there is increased susceptibility to infection in patients with leukemia, in spite of huge WBC production?
Answer. There is increased WBC production in patients with leukemia; inspite of that these patients are highly susceptible to infection as the abnormal malignant WBCs are immature and functionally incapable of defending the body against infections.
Question 55. In which age group, leukemia is more common?
Answer. It is the common malignancy in children and young adults.
Question 56. Name the etiological factors of leukemia?
Answer.
- Chromosomal abnormality—presence of an abnormal chromosome, e.g. Philadelphia chromosome
- Exposure to high doses of radiation therapy
- Exposure to certain chemicals, e.g. benzene, phenyl butazone and chloramphenicol, etc
- Chemotherapy
- Pre-existing polycythemia Vera
- Genetic disorder (Down’s syndrome)
- Primary immune deficiency
- Infection with human leukocyte virus (HTLV-1—human T-cell leukemia virus-1)
- Hereditary or familial susceptibility.
Question 57. What are the different leukemias seen?
Answer. Leukemias are broadly classifid into two types:
- Acute leukemias
- Chronic leukemias.
Question 58. Name the different types of acute leukemias.
Answer. Acute leukemias are of two types:
- Acute lymphocytic leukemia (ALL)
- Acute myeloblastic leukemia (AML).
Question 59. Name the different subtypes of acute lymphocytic leukemia (ALL).
Answer.
- L1—common childhood leukemia
- L2— adult ALL
- L3—rare subtype, blast cells resemble Burkitt’s lymphoma
Question 60. What are the different subtypes of acute myeloblastic leukemias (AML)?
Answer. Acute myeloblastic leukemias are broadly divided into three types, which also have some subtypes:
- Granulocytic
- Monocytic
- Eythroid.
Question 61. What are the different subtypes in the granulocytic variant of AML?
Answer. The granulocytic variant of AML has three subtypes:
- M1— myeloblastic leukemia without maturation
- M2—myeloblastic leukemia with maturation
- M3—hypergranular promyelocytic leukemia.
Question 62. Name the different subtypes in the monocytic variant of AML.
Answer.
- M4—myelomonocytic leukemia
- M5—monocytic leukemia.
Question 63. What is the name of erythroid type of AML?
Answer. It is called erythroleukemia (M6).
Question 64. What are the different types of chronic leukemias seen?
Answer. Chronic leukemias can be of two types:
- Chronic myelogenous Leukemia (CML)
- Chronic lymphocytic Leukemia (CLL).
Question 65. What is the common age of occurrence of acute leukemia?
Answer.
- Acute leukemia of children—between 2 to 4 years of age
- Acute leukemia of adult—65 years or above.
Question 66. What is the common age of occurrence of chronic leukemia?
Answer. Between the ages of 25 and 60 years.
Question 67. In which sex leukemia is common?
Answer. Leukemias occur predominantly among males.
Question 68. Describe the general clinical features of leukemia.
- Fatigue, weakness, dyspnea and repeated epistaxis
- Ecchymoses, headache, vomiting and generalized weakness
- Hepatosplenomegaly, severe anemia, lymphadenopathy and cerebral hemorrhage
- Severe infections like pneumonia and septicemia.
Question 69. Describe the oral manifestations of leukemia.
Answer.
- Gingival hyperplasia with ulceration and severe bleeding tendency
- Diffuse hyperplastic gingivitis with cyanotic bluish discoloration of the gingiva
- Tumor-like swellings in the gingiva due to deposition of leukemic cells
- Oral mucosal pallor, ecchymosis, hemorrhage and hematoma formation
- Mucositis with frequent mucosal ulcerations
- Recurrent secondary infections e.g. candidiasis, histoplasmosis, aspergillosis and HSV infections, etc
- Rapid loosening and spontaneous exfoliations of teeth due to destruction of periodontal ligament and alveolar bone
- Delayed healing of socket after extraction and risk of development of osteomyelitis of the jaw
- Mental nerve neuropathy or the numb chin syndrome.
Question 70. Name the common investigations done in leukemia.
Answer.
- Complete peripheral blood count: WBC count may be greater than 20,00,000/mm3
- Bone marrow aspiration: Abnormal increase in bone marrow cells with high proportion of leukocyte series
- Lumber puncture: To see the presence of leukemic cells in central nervous system
- X-rays: Chest, skeleta, CT scan and MRI
- Lymphangiogram: To determine the leukemic cells within lymph nodes.
Question 71. Name the common imaging techniques used in leukemia.
Answer.
- Chest X-ray: To detect the mediastinal involvement
- Skeletal X-ray: To detect skeletal lesions
- MRI and CT scans: To detect lesions and sites of infection, especially in the head and neck areas.
Question 72. How leukemia can be treated?
Answer.
- Antileukemic chemotherapy
- Radiotherapy
- Repeated blood transfusions
- Administration of antibiotics.
Question 73. What is aleukemic leukemia?
Answer. This is a rare type of leukemia characterized by a normal or low number (less than 100/ mm3) of white blood cells in the blood, despite leukemic changes in bone marrow.
Question 74. How the aleukemic leukemia develops?
Answer. In case of conventional leukemia, the bone marrow produces abnormal, malignant WBCs in high numbers, which are present and easily detected in the peripheral blood.
However in aleukemic leukemia, although bone marrow generates abnormal WBCs but those are not detectable in peripheral blood (as these cells actually accumulate within the spleen, liver, lymph nodes, testes, brain, etc). Hence in aleukemic leukemia, peripheral blood count shows normal or less than normal number of WBCs.
Question 75. What is agranulocytosis?
Answer. Agranulocytosis is a serious acute leukopenia, characterized by a signifiant decrease in the number of granular leukocytes (chiefl the neutrophils).
Question 76. Which people are commonly affected by agranulocytosis?
Answer. The disease commonly occurs among adult females and interestingly this disease frequently affects the health professionals.
Question 77. Name the common causes of agranulocytosis.
Answer.
- Toxic effects of some drugs, e.g. sulfonamides, chloramphenicol, anti-histaminics and chlorambucil, etc.
- Due to the hypersensitivity to drugs like aminopyrine
- Long-term administration of analgesics (phenylbutazone), antithyriods, diuretics, cytotoxic drugs and anti-coagulants, etc
- Ionizing radiation, tuberculosis, typhoid fever and malaria.
Question 78. Describe the clinical features of agranulocytosis.
Answer.
- Fever, sore throat, weakness, bone pain, jaundice and skin pallor
- Persistent bacterial infections with regional lymphadenitis
- Severe dysphagia, weak and rapid pulse, etc. are the other important features of the disease
- Urinary tract infections with vaginal and rectal ulcerations are common
- Early onset of pneumonia, sepsis and shock, etc, often with fatal outcome.
Question 78a. What are the oral manifestations of agranulocytosis?
Answer.
- Development of necrotizing ragged ulcerations (agranulocytic angina) in the gingiva, soft palate, tonsils, lips, pharynx and cheek, etc
- Oral ulcers are often deep, hemorrhagic and are often covered with a yellow or grey membrane; and characteristically there is absence of any red halo due to the lack of inflmmation
- Gingival bleeding, halitosis, excessive salivations and dysphagia often cause difficulty in taking food
- Excessive tendency for developing secondary infections, which develop rapidly and soon become overwhelming. These lesions often resemble acute necrotizing ulcerative gingivitis (ANUG)
- Opportunistic fungal infections are also common in the mouth.
Question 79. What is the commonest oral change in agranulocytosis?
Answer. Occurrence of necrotizing ragged ulcerations in the mouth, with absence of any inflmmatory reaction in the area.
Question 80. What are the important laboratory fidings in agranulocytosis?
Answer.
- WBC count reveals severe leukopenia (500 to 3000/mm3) with extreme reduction in neutrophil count (0 to 2%).
- Bone marrow examination reveals an absence of granulocytes, a maturational arrest of young developing cells or an increased number of myeloid precursors (signifying peripheral granulocyte destruction).
Question 81. What is cyclic neutropenia?
Answer. Cyclic neutropenia is an idiopathic disease characterized by episodic defects in neutrophil maturation in the bone marrow, resulting in periodic fall in circulating neutrophils at regular intervals of 3 to 4 weeks.
Question 82. What is the pathologic signifiance of cyclic neutropenia?
Answer. Cylic neutropenia is an episodic problem and is often characterized by severe infections (especially RTI) whenever the neutrophil count falls below the critical level (less than 500/mm3 of blood); interestingly the symptoms subside as the neutrophil count rises toward normal.
Question 83. Describe the oral manifestations of cyclic neutropenia.
Answer.
- Recurrent aphthous-like ulceration in the month for short duration
- Development of rapidly progressive periodontitis at an early age with severe gingival recessions, rapid alveolar bone loss and tooth mobility, etc.
Question 84. What is purpura?
Answer. Purpura is defied as the extravasation of small amount of blood into the skin or mucous membrane, causing petechiae, ecchymoses or spontaneous bruising, etc.
Question 85. How purpura occurs?
Answer. The disease purpura results from platelet disorder or occasionally due to vascular defects and is characterized by prolonged bleeding time (BT) but normal clotting functions.
Question 86. What are the different types purpuras?
Answer. The disease is of two types:
- Idiopathic thrombocytopenic purpura (ITP)
- Non-thrombocytopenic purpura.
Question 87. What is idiopathic thrombocytopenic purpura (ITP)
Answer. ITP refers to thrombocytopenia caused by an unknown, possibly autoimmune disease.
This disorder is characterized by premature destruction of platelets due to the formation of antibodies against them. The platelets are then destroyed by phagocytosis in the spleen or in the liver.
Question 88. How the lifespan of platelet cells change in purpura (ITP)?
Answer. Normally platelet cells survive 8 to 10 days within the circulation but in ITP the platelet survival time is as brief as 1 to 3 days or less.
Question 89. What is non-thrombocytopenic purpura?
Answer. Non-thrombocytopenic purpura occurs due to the rupture of smaller blood vessels with resultant bleeding into the tissue.
Question 90. What are the different forms of non-thrombocytopenic purpura?
Answer. Major forms of this disease are as follow:
- Familial hemorrhagic telangiectasia
- Anaphylactoid purpura
- Toxic purpura.
Question 91. Name the drugs which might cause purpura.
Answer.
- Chloramphenicol
- Phenyl butazone
- Indomethacin
- Thiazide
- Diuretics
- Quinine
- Quinidine.
Question 92. In which age and gender purpura is more common?
Answer. Purpura commonly occurs among adults below 40 years of age and females are more frequently affected than males.
Question 93. Name the major clinical symptoms in purpura.
Answer.
- Sudden, spontaneous occurrences of petechiae, ecchymosis or hematomas in the skin and mucous membrane
- Excessive bruising tendency, epistaxis, hematuria, melena and hematemesis
- Prolonged bleeding after surgery or injury
- Purpuric spots on the skin
- Bleeding into the diaphragm may result in pulmonary complications
- Intracranial hemorrhage may produce paresthesia of the cranial nerves and hemiplegia
- Bleeding into the muscles and joints with diffiulty in movements.
Question 94. What is petechiae?
Answer. Petechiae is small pinpoint hemorrhage under the skin or mucosa, which often occurs in purpura.
Question 95. What is ecchymoses?
Answer. Ecchymosis is the escape of blood into tissues producing a large bruise.
Question 96. What is a hematoma?
Answer. Hematoma is a blood-filed large, dark appearing tumor in the skin and mucous membrane.
Question 97. Describe the oral manifestations of purpura.
Answer.
- Profuse gingival bleeding and development of petechiae and ecchymoses in oral mucosa
- Persistent uncontrolled hemorrhage after oral surgical procedures
- Submucosal hematomas often develop in the oral cavity following trauma
- Bleeding into the facial muscles may cause diffiulty in opening and closing the mouth
- Bleeding into the temporomandibular joint results in pain and trismus.
Question 98. When does spontaneous bleeding start in purpura?
Answer. Normal platelet count in our blood is 2.5 to 4 lac/mm3; when the platelet count goes down below 100,000/mm³ of blood the condition is called purpura. At this level of platelet count, spontaneous bleeding does not occur, rather it can occur only if the platelet count goes down below 20,000 mm3 of blood.
Question 99. Besides reduction in the number of platelet cells, what are the other laboratory fidings seen in purpura?
Answer.
- Prolonged bleeding time with normal coagulation time
- Increased capillary fragility as demonstrated by tourniquet test
- Positive platelet antibody screening
- Bone marrow aspirates contain normal or increased number of megakaryocytes
- Examination of urine reveals proteinuria or hematuria.
Question 99a. What does Rumple-Leede test measure?
Answer. It helps to measure capillary fragility.
Question 100. Is spleen palpable in case of pupura?
Answer. The spleen is not palpable in case of purpura and if it is palpable, leukemia should be suspected rather than purpura.
Question 101. What are the treatments done in purpura?
Answer.
- Steroid and immunosuppressive drug therapy
- Repeated blood transfusion
- Splenectomy.
Question 101a. Why blood doesn’t clot as long as it is within the vessel?
Answer.
- Due to presence of heparin and
- As thrombin has a positive feedback on plasminogen.
Question 102. What is hemophilia?
Answer. Hemophilia is a potentially fatal inherited bleeding disorder characterized by profound hemorrhage due to the genetic deficiency of clotting factors.
Question 102a. What type of disorder hemophilia is?
Answer. It is a sex-linked disorder, means a disease or abnormality that is determined by the sex hormones; hemophilia exhibits a genetic defect on an X chromosome.
Question 103. Which gender is affected by disease hemophilia?
Answer. The disease occurs in males but is transmitted by females (often transmitted from mother to son).
Question 104. What is the usual incidence rate of hemophilia?
Answer. The incidence rate of hemophilia is 1 in 8,000–10,000 populations.
Question 105. Name the possible etiological factors in hemophilia.
Answer.
- Heredity
- Sex-linked recessive trait
- Spontaneous genetic mutations in case of negative family history.
Question 106. Name the different types of hemophilia.
Answer. There are three major types of hemophilia:
- Hemophilia A (classic hemophilia)
- Hemophilia B (christmas disease)
- Von Willebrand’s disease.
Question 107. What is hemophilia A or classic hemophilia?
Answer. Hemophilia caused by a congenital defiiency of factor VIII is called hemophilia A (it constitutes about 80 percent of all hemophilias).
Question 108. What is hemophilia B or christmas disease?
Answer. Christmas disease is a clotting disorder similar to hemophilia A but caused by a congenital defiiency of factor IX.
Question 109. What is Von Willebrand’s disease or vascular hemophilia?
Answer. It is a form of hemophilia discovered by Erik von Willebrand; a genetic disorder that is inherited as an autosomal recessive trait and characterized by a defiiency of the coagulation factor and mucosal bleeding.
Question 110. Describe the general clinical features of hemophilia.
Answer.
- Easy bruising and prolonged bleeding, particularly after minor accidental, surgical or dental trauma
- Bleeding into muscles and joints cause pain, swelling and diffiulty in movement of the affected organ
- Spontaneous bleeding into the subcutaneous tissue or internal organs leads to recurrent soft tissue hematoma formation
- Interestingly no petechial spots are found on the skin or mucosal surfaces
- Severe, sometimes fatal epistaxis after injury to the nose
- Gastric hemorrhage may occur in case a gastric ulcer is present
- Recurrent hemarthrosis (bleeding into the joints) occurs commonly in elbow, knee and ankle joints, etc. and untreated or recurrent cases may result serious joint deformity and permanent crippling
- Patients often have spontaneous hematuria and intracranial hemorrhage.
Question 111. Describe the oral manifestations of hemophilia.
Answer.
- Severe hemorrhage from the gingival tissue after surgical incision, curettage or dental extraction
- Bleeding may even start after brushing the teeth with a hard toothbrush
- Severe bleeding if not controlled, may continue for weeks or until the patient dies
- Slight trauma (e.g. common bumps or fall in case of small babies) may lead to ecchymosis or hematoma formations in the tongue, lips or palate, etc
- Severe bleeding unexpectedly occurs from the tiny injection sites in the mouth
- Internal bleeding into the glottis with subsequent airway obstruction; may occur following pterygomandibular-block-anesthesia, which may cause death of the patient
- Recurrent subperiosteal hematoma with reactive newbone formation maycause tumorlike malformations of the jaw (such lesions are called pseudo-tumor of hemophilia)
- Hemophilic patients often carry a high caries index and severe periodontal disease.
Question 112. Describe the laboratory fidings in hemophilia.
Answer.
- Clotting time is prolonged or sometimes it may test normal
- Platelet count and bleeding time is normal
- Prothrombin time is normal
- Prothrombin consumption—decreased
- Activated partial thromboplastin time is prolonged
- Thromboplastin generation—increased
- Genetic counseling and carrier detection by biologic and immunologic assays
- Blood grouping and cross-matching
- Specific quantitative assays for factor VIII will determine the severity of the disease.
Question 113. Describe the treatment of hemophilia.
Answer.
- Immediate transfusion of factor VIII or IX concentrate is the primary treatment
- Although plasma and cryoprecipitate contain factor VIII, the concentrates have a known AHG content and carry less risk of blood volume overload
- As the pro-coagulant activity of AHG disappears rapidly, patients need transfusions every 12 hours until bleeding stops
- Transfusion of packed red blood cells or white blood cells are used only to replace blood volume when there has been severe loss
- Prophylactic transfusion of factor VIII to a level of 50 percent above normal is recommended in cases of minor injury or dental extractions.
Question 114. Which disease is resulted due to deficiency of all the three components of coagulation factor VIII?
Answer.Von Willebrand’s disease.
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