Red Blood Cell Disorders PDF

411 Oral Medicine, Diagnosis & Periodontology I Note : students are responsible for any material given in the lectures and sections even if not mentioned in the handouts Textbook: Burkets Oral Medicine 12th edition  Red Cell Disorders Dr : Amira Abdelwhab Lecturer of O...

411 Oral Medicine, Diagnosis & Periodontology I Note : students are responsible for any material given in the lectures and sections even if not mentioned in the handouts Textbook: Burkets Oral Medicine 12th edition  Red Cell Disorders Dr : Amira Abdelwhab Lecturer of Oral Medicine, Diagnosis and Periodontology  RBC Life span: 120 days Count: 4.5 – 5 million / cubic mm, males > females Hemoglobin: 13-16 gm/ dl in males 13-14 gm/ dl in females  Adult hemoglobin, Hb A consists of two alpha and two beta globin chains as α2β2. Fetal hemoglobin, HbF, consists of two alpha and two gamma chains as α2 δ2. N.B: In normal person most of the hemoglobin is HbA. At birth about 80% of hemoglobin is HbF. Adult life HbF is not more than 1%  RBC disorders polycythemia Anemia Normocytic Microcytic Macrocytic Absolute Relative Normochromic Hypochromic normochromic Primary secondary Polythythemia  Definition Abnormal increase in the erythrocyte count in the peripheral blood, usually accompanied by an increase in hemoglobin and hematocrit. value Polycythemia is divided into: Absolute erythrocytosis (a true increase in red- cell mass) Relative erythrocytosis (the red cell mass is normal, but the plasma volume is reduced. Relative polycythemia Causes : Loss of intravascular fluid Rapid diuresis secondary to treatment for congestive heart failure. In relative polycythemia, the hemoglobin rarely rises more than 25%, and there are no appreciable oral changes.  Absolute polycythemia Primary (idiopathic) Secondary “Polycythemia “Erythrocytosis” Rubra Vera”  Primary (idiopathic) Polycythemia “Polycythemia Rubra Vera” Etiology: unknown Age & gender: elderly patients M> F. Characterized by : number of RBCs (may reach 18 million/mm3). hemoglobin (18-24 g/dl). WBC count platelets count with platelets dysfunction blood viscosity serum iron and ferritin. Secondary Polycythemia “Erythrocytosis” 2ry polycythemia  Clinical features The main clinical problems are due to: Increased blood volume. Increased blood viscosity → thrombosis. Bone marrow over activity  Extra-oral features Purplish red Distended Headache face and dark neck extremeties veins Bone pain  Oral features Purplish-red oral mucosa Varicositis in the ventral aspect of tongue Bleeding from gingiva???????? Petechia and ecchymosis.  Dental management: Special attention to local hemostasis. Anemia Definition Defined as reduction in the oxygen carrying capacity of the blood. It is usually related to decrease in the number of circulating red blood cells and reduction of the quantity of hemoglobin.  Classification of Anemia The size of RBCs into: Normocytic, Microcytic and Macrocytic. The hemoglobin concentration into: Hypochromic and Normochromic Anemia. Etiology : decrease red blood cell production increase blood loss,  Anemia Iron deficiency anemia  Iron deficiency anemia is Microcytic Hypochromic anemia. It is the most common type of anemia particularly in women at child bearing age  Etiology: Inadequate iron intake Consumption of tea with meal (tannic acid interfere with iron absorption Impaired iron absorption Gastrectomy (due to HCl) Chronic blood loss. eg. heavy menstruation. Increased iron requirement e.g. pregnancy & growing children  Clinical features 1- Due to iron depletion Sparse hair Brittle nails Koilonychia: spoon shaped and brittle nails Burkets p: 440 2) Due to anemia and tissue hypoxia Symptoms Signs Fatigue Pallor of skin, nail beds & Headache palpebral conjunctiva Palpitation Tachycardia Dyspnea on exertion Cardiac murmurs  Oral Features 1. Pallor of oral mucosa. 2. Angular cheilitis 3. Glossitis 4. Sore mouth: sore atrophic oral mucosa. 5. Slow wound healing. Due to decreased iron storage. The epithelial integrity depends on  Diagnosis Case history????????? Clinical examination?????????? Laboratory investigations ↓RBCs count.( 3,000,000 - 4,000,000/mm3) ↓ Hb concentration ( less than 11 g/dl of blood ) Many immature cells are seen in peripheral blood RBC may be irregular in size (anisocytosis) or shape (poikilocytosis). ↓ MCV, MCH, MCHC ↓ Serum iron level Transferrin saturation.  Treatment Eliminate the cause Ferrous sulphate tablets (600 mg daily contain 120 mg ferrous iron) best absorbed when the patient is fasting.  Dental implications CBC with differential should be ordered for anemic patient. ? Avoid general anesthesia if Hb < 10mg/dl Avoid surgical procedures in severely anemic state  B. Plummer-Vinson Syndrome (Patterson-Kelley Syndrome) Smooth red painful tongue Atrophy of the mucosa of the mouth, pharynx and upper esophagus. Angular cheilitis and oral ulceration. Dysphagia due to muscular degeneration in the esophagus and stenosis of the esophageal mucosa. The depletion of iron stores in the body may be the direct cause of mucosal atrophy, since the integrity of the epithelium is dependent upon adequate serum iron levels. Patients with these syndromes should be followed up closely for any oral or pharyngeal changes that may be an early indicator of carcinoma  Anemia Pernicious Anemia (Addisonian Anemia) (Macrocytic Normochromic anemia)  It is an autoimmune disease in which there is progressive destruction of the gastric fundic glands, leading to atrophic gastritis, loss of production of intrinsic factor and vitamin B12 malabsorption. Etiology: Circulating autoantibodies against parietal cells of stomach defect in intrinsic factor secretion by parietal cells defect in vit. B12 absorption defect in RBCs maturation N.B.: Vit. B12 is essential for: Maturation of RBCs Metabolism of nerve tissues. Vit. B12 deficiency results in: ↓ Count of RBCs (anemia). Degeneration of spinal cord causing neurologic manifestation Clinical Features Insidious onset Clinical features  Clinical Features ❖Insidious onset with progressive signs and symptoms of anemia ❖Severe pallor (lemon yellow color) due to: Tissue hypoxia. Jaundice: as a result of increased serum bilirubin due to premature breakdown of newly formed RBC ❖Neurological symptoms: symmetrical numbness of the extremities & muscular weakness. ❖Gastrointestinal symptoms: constipation or diarrhea, vague epigastric pain & increased risk of stomach cancer Burkets p : 442  Oral features Glossitis, glossodynia & sore atrophic tongue Pallor of the mucous membrane of the entire oral cavity. Aphthous like ulceration may occur particularly on the tongue. Intolerate to wearing denture due to changes in oral mucosa. Disturbance of taste sensation.  Diagnosis Clinical Laboratory History examination investigations  Diagnosis Pernicious Anemia could be suspected if the patient complains of triad of symptoms: Sore, painful atrophic tongue Generalized weakness Numbness in the extremities Hematological finding ↓ RBCs count. ↓ Hb concentration. ↑ MCV, MCH. Normal MCHC ↓ Serum vit. B12 level (usually < 100 pg/ml). Definitive diagnosis can be reached by 1. Schilling test: 2. Detection of serum antibody against Parietal cells. Gastric intrinsic factors Treatment: Patients are treated (for life) with I.M. injection of hydroxy cobalamine (1000 µg), given as total dose of 5000-6000 µg over 3 weeks and then1000 ug every three months. N.B.The hematological changes of pernicious anemia may be improved by oral folic acid therapy but will not stop the progression of neurologic symptoms. It may aggravate neuropathy. For this reason anemic patients should never take folic acid therapy without being first sure that their anemia is not pernicious.  Anemia Folate Deficiency Anemia ❖Macrocytic normochromic anemia due to deficiency of folic acid. ❖Body reserve of folate in the liver can be depleted within 4 months. ❖The main causes of Folate deficiency are: 1. Poor intake. 2. Malabsorption: inflammatory bowel disease. 3. Increased demand: infancy & pregnancy 4. Drugs: alcohol, barbiturates& phenytoin The initial clinical presentation of folate and Vit.B12 deficiency may be indistinguishable from each other but, folate deficiency does not lead to neurological manifestation Folate deficiency anemia is characterized by: Serum folate level low and serum Vit.B12 normal Schilling test is normal. Treatment: Folic acid 5 mg/daily  Anemia Aplastic Anemia (Normocytic Normochronic Anemia)  Aplastic anemia is a rare bone marrow aplasia, causing refractory Normocytic Normochromic anemia, leukopenia and thrombocytopenia (pancytopenia)  Etiology Congenital e.g. Fanconi's anemia ( skeletal, renal and CNS abnormalities). Primary Acquired: immune suppression of bone marrow stem cells by T suppressor cells. Drugs: cytotoxic (methotrexate) and noncytotoxic (chloramphenicol). Ionizing radiation. Secondary Viral infection: vital hepatitis and measles.  Clinical Manifestations General features of anemia ??? General features of thrombocytopenia ??? General features of leukopenia???? Oral manifestations include: Pallor of the oral mucosa, Minimal but persistent gingival bleeding, Petechiae and ecchymosis Oral ulcers: Burkets : p437  Diagnosis (1) Case history????????????? (2) Clinical examination???????????? (3) Laboratory investigation???????????? * Pancytopenia: Decrease in the count of: - RBCs. - Platelets. - WBCs. * Normal: MCV, MCH & MCHC. Treatment Remove the cause if possible. Bone marrow transplant Hemoglobinopathies  Definition defects in the globin portion of hemoglobin molecule. The defect makes RBCs that contain the abnormal hemoglobin more susceptible to hemolysis. Examples: Sickle cell disease. Thalassemia  Each hemoglobin molecule consists of two alpha and two beta globin chains. Defects of the globin portion of hemoglobin patient become susceptible to hemolytic anemia.  Anemia Sickle Cell Disease  Definition Sickle cell disease is hereditary disorder of hemolytic anemia, characterized by an abnormality in hemoglobin where glutamic acid on beta chain is replaced by valine.  Definition Disease may be present in the form of: - Sickle cell trait. - Sickle cell anemia.  Pathogenesis Infection. Dehydration. Cold. sickling of RBC Acidosis. Hypoxia (GA & high altitude).  Destruction of sickle cells (hemolysis) with features of anemia. Sickling can lead to Increased viscosity and clumping of cells in the microcirculation. This leads to thrombosis, ischemia and infarction  Clinical features Extra-oral features Anemia ?????????????. Underdeveloped child Jaundice: ↑ level of bilirubin ??????? Vasooccluison??????????? Chronic leg ulcers????????? Cerebral vessel: stroke. Spleenomegaly???????? Crisis:  Clinical features Extra-oral features Painful crisis: abdominal & bone pain due to occlusion of blood vessel by sickled RBC. Aplastic crisis: Red blood cell production stops and the patient becomes acutely ill due to infection. Splenic sequestration crisis: In early childhood, the spleen is enlarged with entrapment of sickle cells. This can occur acutely and can cause infant death. In adults splenomegaly is rare due to repeated infarction and fibrosis. Splenic function is lost and patients are susceptible to infection.  Clinical features Oral manifestations The oral mucosa especially the soft palate is pale with yellowish tinge. Delayed eruption and enamel hypoplasia. Maxillary overgrowth  Radiologic changes The jaw bone shows osteoporosis as well as areas of osteosclerosis???????? Trabecular pattern of alveolar bone tends to run horizontally between the roots. (Ladder like effect). Lamina dura: dense and distinct. Skull x : “hair on end appearance”. The frontal bossing  Laboratory findings Normochromic normocytic anemia. Sickling occurs after sealing fresh blood in a small chamber of microscopic side with a reducing agent for 1 hour (sodium metabisulphite). Sickling solubility test: a mixture of Hb S in reducing solution (sodium dithionite) gives a turbid appearance due to precipitation of Hb S, while Hb A, gives clear solution. Hemoglobin electrophoresis: 80-90% is Hb S, the remainder is Hb F.  Management (No treatment other than symptomatic treatment). Avoid precipitating factors Acute attack requires supportive therapy (IV fluid, oxygen, antibiotic and narcotic analgesic). Folic acid given during pregnancy and those with severe hemolysis. Blood transfusion is indicated during aplastic crisis were Hb level is extremely reduced.  Dental implications Avoid extensive soft tissue surgery due to delayed healing. Avoid general anaesthesia as it may result in crisis. Treat infection immediately as it may result in aplastic crisis. Pain may be due to pulpitis or osteomyelitis secondary to vascular occlusion. Burkets p 446  Sickle cell trait (HbAS) Heterozygous cases in which 60% of hemoglobin is HbsS, the remainder is normal hemoglobin. The patient is asymptomatic and lives normal life. Sickle cell crisis can be caused by reduced oxygen tension (general anesthesia, high altitude or unpressurized aircraft).These patients have few problems in management, but if general anesthesia is necessary full oxygen must be maintained. Thalassemia  Definition Group of heredietary diseases characterized by a deficient synthesis of either: α or β chain Thalassemia (Greek thalassa = sea) are anemia originally found in people living on the shore of the Mediterranean but are now known to affect persons throughout the world.  Clinical features Colley’s anemia is the most serious type &characterized by: Slow growth & development of children. Ashen gray skin color due to combination pallor, jaundice & hemosiderosis Recurrent bacterial infection (increased availability of iron). Features of severe anemia. Hepatospleenomegaly Skeletal growth retardation.  Clinical features Iron overload; due to: Destruction of RBCs with release of iron. Repeated blood transfusion. Iron is deposited in heart, liver, pancreas and skin resulting in organ dysfunction.  Oral manifestation Oral mucosa appears pale with yellowish tinge Painful swelling of the parotid glands caused by iron deposition. Painful tongue as the patients is susceptible to folate deficiency Thalassemic face “Chipmunk face”: Dentin and enamel contain excessive amount of iron (5 times normal) with teeth discoloration. Poor wound healing.  Oral manifestation Thalassemic face “Chipmunk face”: Hyperplasia of the bone marrow results in : Bimaxillary protrusion. Retractred upper lip. Spacing of teeth and open bite  Radiographic features Similar to sickle cell anemia  Diagnosis 1) ↓ RBCs count and Hb concentration. 2)↓ MCH, MCHC, MCV. 3)Normal serum iron level (unlike iron deficiency anemia). 4)Electrophoresis: shows HbF and the remainder is HbA2. Dental management: 1)Avoid extensive soft tissue surgery due to delayed wound healing. 2)Surgery may successfully treat facial deformity THANK YOU