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RBC DISORDERS.ppt

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This document provides information on laboratory diagnosis of red blood cell disorders. It discusses various red blood cell indices and their reference ranges. It then covers various types of anemias including iron deficiency anemia, megaloblastic anemia, sickle cell anemia, erythroblastosis fetalis, thalassemia, and aplastic anemia. The diagnostic workup and features of each disorder are summarized. Red cell morphology changes seen in different conditions are also outlined.

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LAB DIAGNOSIS OF RBC DISORDERS - 1 1 SHERIN JAMES
CONTENTS Introduction RBC DISORDERS • Anemia Iron deficiency anemia Pernicious anemia Sickle cell anemia Erythroblastosis fetalis Thalassemia Aplastic anemia • Polycythemia vera References 2
3 INTRODUCTION
4
5
6
ERYTHROCYTES Total RBC count Haemoglobin Concentration male -- 14-18gm/dl female -- 12-16gm/dl 7
8 HEMOGLOBIN
COMPLETE BLOOD COUNT COMPONENTS Hemoglobin, Hematocrit, RBC count. RBC indices, RBC distribution width. WBC count, Differentials & Platelet count. Evaluation of peripheral blood smear morphology.
RED BLOOD CELL INDICES. Mean corpuscular volume (MCV): Mean corpuscular hemoglobin concentration(MCHC): Mean corpuscular Hb (MCH): Increased MCHC - Artefacts
12 ADULT REFERENCE RANGES-RED BLOOD CELLS Measurement (units) Men Women Hemoglobin (gm/dL) 13.6-17.2 12.0-15.0 Hematocrit (%) 39-49 33-43 Red cell count (106/μL) 4.3-5.9 3.5-5.0 Reticulocyte count (%) 0.5-1.5 Mean cell volume (μm3) MCV 82-96 Mean corpuscular hemoglobin (pg) MCH 27-33 Mean corpuscular hemoglobin concentration (gm/dL) - MCHC 33-37 RBC distribution width RDW 11.5-14.5
MISCELLANEOUS CHANGES 1. Spherocytosis : spheroidal red cells.  hereditary spherocytosis  auto immune hemolytic anaemia  ABO haemolytic disease of newborn. 2. Schistocytosis : fragmentation of erythrocytes.  Thalassemia  megaloblastic anaemia  hereditary elliptocytosis  iron def. anaemia  severe burns. 17
3. Irregularly contracted red cells :  drug & chemical induced hemolytic anaemia  unstable haemoglobinopathies. 4. Sickle cells / drepanocytes :  sickle cell disease. 18
5. Leptocytosis : presence of unusually thin red cells. severe iron def. anaemia thalassemia. 6. Crenated red cells : erythrocytes which develop numerous projections from the surface. Coarsely crenated red cells are called Acanthocytosis.  splenectomy pts  chronic liver disease. 19
8. Burr cells : cell fragments having one or more spines. Found in --- uraemia. 9. Stomatocytosis : stomatocytes have central area having a slit like or mouth like appearance. • hereditary stomatocytosis • alcoholism. 10. Ovalocytosis / elliptocytosis: oval or elliptical shape of red cells. • hereditary ovalocytosis • Elliptocytosis 20
21
Red cell disorders Anemias ↓red cell mass Polycythemia ↑ red cell mass
Anemias ↓red cell mass Blood Loss Hemolytic ↓Erythropiesis B12, Folate, Iron ↓
Anemias ↓red cell mass ↓Erythropoiesis Megaloblastic B12,Folate Iron deficiency Anemia of Chronic disease Aplastic anemia Pure red cell aplasia Others
Anemias ↓red cell mass Blood Loss Acute Chronic
ANEMIA 30 • Anemia refers to reduction in 1. Red blood cell count 2. Hemoglobin content 3. Packed cell volume •Hb conc. in blood below the lower limit of the normal range for the age & sex of the individual. • lowered ability of the blood to carry oxygen.
31 MORPHOLOGICALLY – Average Size Of The Cells And The Hb Concentration (Wintrobe’s): - Macrocytic: megaloblastic - Normochromic, normocytic: Acute post hemorrhagic, hemolytic, aplastic - Hypochromic, Microcytic: Iron Deficiency, Thalassemia, Chronic Post Hemorrhagic
1. Loss of blood: • Acute posthemorragic anemia • Chronic posthemorrhagic anemia 2. Excessive destruction of RBC: a) Extra corpuscular causes: Antibodies, Infection(malaria), • Splenic sequestration and destruction • Drugs, chemicals and physical agents b) Intracorpuscular haemolytic disease 1) Hereditary • Disorder of glycolysis, abnormalities in RBC membrane. • Abnormalities in synthesis of globin 2) Acquired – lead poisonong 3. Impaired blood production resulting from deficiency of substances essential for erythropoiesis a. Iron deficiency b. Deficiency of vitamin B12 , folic acid and Protein deficiency 4. Inadequate production of mature erythrocytes a) Deficiency of erythroblasts b) Pure red cell aplasia c) Infiltration of bone marrow- Leukemia, lymphoma, Multiple myeloma d) Endocrine abnormality- myxedema e) Chronic renal disease f) Chronic inflammatory disease g) Cirrohisis of liver ETIOLOGIC CLASSIFICATION OF ANEMIA:
33
WE COVER - Iron Def. Anemia Megaloblastic Anaemia Sideroblastic Anemia Aplastic Anemia Sickle Cell Anemia Thalassemia 35
36
37 Haemoglobin Concentration gm/dl Adult male 14-18 Adult female 12-16 Children 17-20 New born 10-12
1. IRON DEFICIENCY ANAEMIA Develops when the supply of iron is inadequate for the requirement of hb synthesis. Increased blood loss Increased requirements Inadequate dietary intake Decreased intestinal absorption 38
CLINICAL FINDINGS  Chronic fatigue  Pallor of conjunctiva and palmer creases.  Palpitations  Shortness of breath, numbness  Craving for Ice (pica)  Pallor of the conjunctiva, lips, and oral mucosa;  Brittle nails with spooning, cracking,  Splitting of nail beds, koilonychia  Palmar creases  Bone pain  Chronic iron deficiency: Esophageal webs: causes dysphagia to solid foods. Achlorydia.
44 ORAL MANIFESTATIONS • Angular cheilitis, • Glossitis with different degrees of atrophy of fungiform and filliform papillae • Pale oral mucosa • Oral candidiasis • Recurrent aphthous stomatitis • Erythematous mucositis • Burning mouth
45 PLUMMER-VINSON SYNDROME Classic triad : Upper esophageal webs or strictures + Dysphagia + Iron deficiency anemia Etiopathogenesis : • Unknown - iron deficiency (Fe dep oxidative enzymes) • Malnutrition, • Genetic predisposition and Autoimmune processes. Treatment: Iron supplementation
INVESTIGATIONS 1. BLOOD PICTURE & RED CELL INDICES i. Hb : fall in Hb conc. ii. Red cells : • Microcytic Hypochromic Red Cell. • Anisocytosis & Poikilocytosis Seen. • Target Cells, Elliptical Forms & Polychromatic Cells Often Present. iii. Reticulocyte count : normal or reduced iv. Absolute values : reduced MCV, MCH, MCHC, Increased RDW v. Leucocytes : normal vi. Platelets : increased 46
RBC DISORDERS.ppt
RBC DISORDERS.ppt
2) BONE MARROW FINDINGS i.Marrow cellularity : incerased due to erythroid hyperplasia. ii.Erythropoiesis : micronormoblastic (small polychromatic normoblasts). iii.Other cells : Myeloid, lymphoid & megakaryocytic cells are normal in no & morphology. iv.Marrow iron : deficient reticulo-endothelial iron stores. 49
3) BIOCHEMICAL FINDINGS i.Serum iron level – low 50µg/dl. (normal – 80-180µg/dl) ii.Total iron binding capacity (TIBC) – high (normal –250-450µg/dl) iii.Serum ferritin – low (normal – 150-2000ng/dl) iv.Red cell proptoporphyrin – low ( normal 20-40µg/dl) 50
51
TREATMENT • Ferrous sulphate orally. • Hct Should increase 0.5% to 1%/day. • Lack of response indicates: -Noncompliance. -Continued blood loss. -Iron is not being absorbed.
2. MEGALOBLASTIC ANAEMIA  Cells have small nuclei & large mature cytoplasm – Numerous large erythroid precursor series (THYMIDINE)  Impaired DNA synthesis - characterized by distinctive abnormality in the haemopoietic precursors in the bone marrow in which the maturation of the nucleus is delayed relative to that of the cytoplasm. 53
2A. PERNICIOUS ANAEMIA • 1st described by Addison in 1855. • Source: µorg:, Dietary animal prdcts • 2.4µg/day RDA (3 mg – stored) • Associated with gastric atrophy & lack of intrinsic factor (IF) production. • Neuropsychiatric, digestive symptoms • Hunter’s glossitis, beefy, glossodynia, lingual paresthesia 54
1. BLOOD PICTURE & RED CELL INDICES : i. Hb : fall in conc. ii. Red cells : o Macrocytotic Red Cell. o Anisocytosis, poikilocytosis & presence of Macro-ovalocytes. o Basophilic Stippling, Howell Jolly Bodies also seen iii. Retciulocyte count : low to normal iv. Absolute values : ↑MCV, MCH & normal or reduced MCHC v. Leucocytes : reduced. Hyperseg neutrophils vi. Platelets : reduced. INVESTIGATIONS 55
56 Howell-Jolly bodies
57 Cabot’s rings
BONE MARROW FINDINGS - i.Marrow cellularity : hypercellular with decreased myeloid – erythroid ratio. ii.Erythropoiesis : megaloblastic erythropoiesis. Megaloblasts are abnormal, large, nucleated erythroid precursors. iii.Marrow iron : increase in number & size of iron granules. BIOCHEMICAL FINDINGS - i.Rise in serum unconjugated bilirubin. ii.Serum iron & ferritin – normal iii. Increased MMA, Homocysteine 58
SPECIAL TESTS TESTS FOR VIT. B12 DEF. 1)Serum Vit B12 Assay – 2 methods : Microbiologic assay & radioassay. 2) Schilling Test (radioisotope absorption test) 3) Antibody test for IF (I, II) TREATMENT: Parental and oral - cyanocobalamin 59
2B. FOLATE DEF. ANEMIA •RDA 400µgm. (Diet) •Normal serum folate is 6-12 ng/ml. •Hyperpigm of skin, mucosa •Below 4ng/ml is diagnostic of folate deficiency + normal levels of MMA 1.Measurement of forminoglutamic acid (FIGLU) urinary excretion after oral administration of histidine. Its excretion is increased if folate def. is present. Because folic acid is required for conversion of FIGLU to glutamic acid. 2.Serum Folate Assay : by microbiological & radioassay. TREATMENT 1-5 mg FA daily, Orally. 60
3. APLASTIC ANAEMIA Paul Ehrlich, introduced the concept of aplastic anemia in 1888. 1n 1904 it was termed as aplastic anemia by Chauffard. Pancytopenia +Hypocellular bone marrow Normal hematopoietic tissue in the bone marrow has been replaced by fatty marrow. Acquired/ Genetic Mucosal pallor, gingival bleeding, hyperplasia, mucosal ulceration. 61
INVESTIGATIONS 1. Anaemia – Hb level <10gm. Blood picture shows normocytic normochromic red cells. Reticulocyte count is reduced or zero. 2. Leucopenia – Neutophil < 500/Cu.mm with relative lymphocytosis. 3. Thrombocytopenia – platelet count <20,000/µL, Prolonged bleeding time 4. Bone marrow aspiration – reduction of myeloid cells, megakaryocytes & erythroid cells. 5. BM: empty marrow, fat spaces, 62
APLASTIC ANEMIAS
4. HAEMOLYTIC ANAEMIAS  Results from an increased destruction of RBC.  Qualitative defect -- Sickle cell Anaemia 64
65 Destruction of fetal blood - r/n between maternal and fetal blood factors.  Inheritance by the fetus of a blood factor from the father that acts as a foreign antigen to the mother.  Rh factor, named after the rhesus monkey, was discovered by Landsteiner and wiener in 1940 as a factor in human RBC that would react with rabbit antiserum produced by administration of RBC from the rhesus monkey. ERYTHROBLASTOSIS FETALIS
66 PATHOGENESIS Transplacental transfer of this antigen, transplacental leaks of RBC From the fetus to the mother Immunization of the mother, formation of antibodies which When transferred back to the fetus by the same route Produce fetal hemolysis • If both parents are homozygously Rh positive→ infant will be Rh positive → no maternal immunization. • If mother is homozygously positive but father is Rh negative → no maternal immunization. • If father is Rh- positive and mother is Rh- negative → fetus inherits parental factor, which may act as an antigen to the mother and immunize her with resultant Ab formation.
67
68 CLINICAL FEATURES • Some infants are stillborn. • Anemia with pallor, Jaundice • Compensatory erythropoiesis • Fetal hydrops ORAL MANIFESTATION • Deposition of blood pigments in the enamel and dentin (green/brown/blue) • Ground sections- positive test for bilirubin • Intrinsic stains • Enamel hypoplasia • Rh hump
69 Laboratory findings • RBC count decreased, large number of normoblasts or nucleated red cells • Icterus index high • Positive direct coombs test on cord blood Treatment • At present, Rh-negative mothers are being given anti-D gamma globulin to prevent immunization
SICKLE CELLANEMIA MC Hereditary chronic hemolytic anemia Mendalian dominant, nongender linked Exclusively - Mediterranean origin. A concordance exists between the prevalence of Malaria and HbS HbA is genetically altered to produce HbS, Substitution of valine for glutamine at the sixth position of the β globin chain Homozygous, Heterozygous 70
 RBCs - normal biconcave discoid shape distorted, generally presenting a sickle-like shape.  Reduces both their plasticity and lifetime from normal 120 days average down to 14 days.  This results in the underlying anemia and hypertrophic bone marrow.  In heterozygote- 40% of hemoglobin is HbS, 60% HbA  In homozygote- nearly all hemoglobin is HbS Deoxygenatio n of the heme moiety of HbS Hydrophobic interactions between adjacent Hbs molecule Aggregate into larger polymer- viscous gel Long needle like fibres within cell Stiff, distorted, Classic sickle shape Obstructs microcircu lation – ischemia, infacrtn, organ damage Hypoxia → promotes sickling
COMPLICATIONS • Pain • Lethargy • Lifelong anemia (low RBC) • Organ failure • Stroke CLINICALLY; • Before The Age Of 30 Years • Cerebrovascular Accidents/ Strokes, • Aplastic Crises Leading To Severe Anemia, • Chronic Leg Ulcers, • Hematuria, • Aseptic Osteonecrosis, • Retinitis Leading To Blindness • Splenic Sequestration, Renal Failure • Acute Chest Syndrome - Fever, Cough, Sputum Prodctn, Dyspnea, Or Hypoxia.
CHEEK BONES MAXILLAR OVERGROWTH BM EXPANSION CHRONIC PAIN – TMJ GINGIVAL EDEMA/ENLRGMNT OSTEOMYELITIS OSTEOPOROSIS STEP LADDER – INTRTRBECLR SPACING BONE WITHIN BONE SKULL X-RAY- CREW-CUT 73
INVESTIGATIONS  RBC- may reach a level of 1,000,000 cells per cubic mm.  Moderate – severe anaemia (Hb conc is 6-9g/dl).  PCV – 17-29%  ^ WBC (N), Platelet  High reticulocyte count- Anemia  Increased marrow response.  Elevated lactic dehydrogenase and decreased levels of hepatoglobin- confirms hemolysis  ^ unconju Billirubin BLOOD SMEAR:  Blood film shows sickle cells & target cells.  Presence of Howell – jolley bodies. 74
DIAG TEST: Hb analysis – PROTEIN ELECTROPHORESIS/ LIQUID CHROMATOGRAPHY
5. THALASSAEMIA COOLEYS / ERYTHROBLASTIC ANEMIA • Thomas B Cooley in 1925. • Diverse group of hereditary disorders in which there is reduced rate of synthesis of one or more of the globin polypeptide chain. • Depending on presence of genetic defect in alpha or beta globin chain genes o Alpha – thalassaemias o Beta – thalassaemias 76
77 ALPHA AND BETA THALASSEMIAS
CLINICALAND GENETIC CLASSIFICATION
RBC DISORDERS.ppt
β-THALASSEMIAS β0 mutations - no β-globin chains are produced β+ mutations - reduced (but detectable) β- globin synthesis Cortical thinning of bones facial bones. expansion of hematopoietic ally active marrow ^ maxillry antrum Mongoloid  "crew-cut" appearance - skull X-rays  Splenomegaly ( up to 1500 gm)  Hemosiderosis and secondary Hemochromatosis RODENT FACIES - develops mongoloid features due to prominence of the cheeks, protrusion of the maxillary anterior teeth, depression of the bridge of the nose.
CLINICAL FEATURES INEFFECTIVE ERYTHROPOIESIS AND HEMOLYSIS – Expanded marrow fills intramedullary space & invades bony cortex - Impaired Bone Growth, Skeletal Deformities – Extramedullary hematopoiesis & hyperplasia of mononuclear cells - Splenomegaly, Hepatomegaly, Lymphadenopathy – Ineffective erythropoietic precursors consume nutrients - Growth Retardation and Cachexia – Severe hemosiderosis – Occurs within the first 2 years of life. – Yellowish pallor of the skin – Fever, chills, malaise, – Generalized weakness – Splenomegaly and hepatomegaly
RBC DISORDERS.ppt
83 1. Silent Carrier State • single α-globin gene is deleted • insufficient to result in anemia, completely asymptomatic 2. α-Thalassemia Trait • deletion of two α-globin genes • clinical picture identical to β-thalassemia minor • small red cells (microcytosis), minimal or no anemia, and no abnormal physical signs 3. Hemoglobin H Disease • only one normal α-globin gene • moderately severe anemia 4. Hydrops Fetalis • Evident By- Third Trimester Of Pregnancy • Excess Γ-globin Chains Form Tetramers (Hemoglobin Barts) Has High Affinity For Oxygen & No Oxygen To Tissues • Fetus - Severe Pallor, Generalized Edema, And Massive Hepatosplenomegaly (Similar To In Erythroblastosis Fetalis) α-THALASSEMIAS
LAB FINDINGS Hypochromia, Microcytosis, MCV, MCH, MCHC – reduced, Anisocytosis And Poikilocytosis Target Cells, Fragmented RBC (Schistocytes) Basophilic Stippling Serum bilirubin – elevated. Supravital staining- Methyl blue demonstrate inclusion bodies. Reticulocytosis Normoblasts (Nucleated RBC), Safety pin cells in the circulating RBC Hbf - Markedly Increased (Major Red Cell Hemoglobin) Bone marrow- cellular hyperplasia with large number of immature, primitive and stem form of RBCs. Best Confirmatory Test -Hemoglobin Electrophoresis Bone Marrow-mild Erythroid Hyperplasia
85 Basophilic stippling
• β-thalassemia minor and α-thalassemia trait – Peripheral blood smears show small (microcytic) and pale (hypochromic) RBCs but regular in shape – Target cells
87 THALASSEMIA MAJOR 87 THALASSEMIA MINOR
88 FESSAS BODIES: Intracellular inclusion bodies, leads to increased erythrocyte haemolysis and severe ineffective haematopoiesis.
6. POLYCYTHEMIA Increase in circulating red blood cells (RBCs) - increased hemoglobin level. • 2 types- • Relative • Absolute • Primary/PRV/Erythraemia: True idiopathic ^ – neoplastic (UNKNOWN) • Secondary/Erythrocytosis: Enhanced RBC production – Hypoxia - ^EPO PRV/ Vaquez’s Disease: • Chronic stem cell disorder with an insidious onset characterized as a panhyperplastic, malignant and neoplastic marrow disorder. •Mutation – JAK2(single progenitor marrow cell) •^ Accumltn of blood, Flushed skin,Erythromelagia, Spleenomegaly, Myelofibrosis
93 ORAL MANIFESTATIONS • Erythema (red-purple color) of mucosa, G •Submucosal petechiae • Erythematous & edematous gingiva • Spontaneous gingival bleeding
94 LAB FINDINGS : Raised HB concn. - >20gm/dl Erythrocytosis normochromic normocytic- >10,000.000/cubic mm HCT ^ 60% (specific gravity, viscosity ^) Mild – moderate leucocytosis with basophilia Thrombocytosis with defective platelet function - 400,000-800,000/dl Bone marrow examn – erythroid hyperplasia & panhyperplasia, hypercellular.
95
96 Nuclear dust
97 Heinz bodies
TEST WHAT IT MEASURES NORMAL VALUES Hemoglobin Amount of this oxygen-carrying protein within RBCs. Men: 12.7 to 13.7 g/dl Women: 11.5 to 12.2 g/dl Hematocrit Proportion of total blood volume made up of RBCs. Men: 42 to 50% Women: 36 to 45% Mean corpuscular vol (MCV) Average vol. of individual RBCs 86 to 98 femtoliters Mean corpuscular Hb conc (MCHC) Average conc. of Hb within RBCs 33.4 to 35.5 g/dl WBC count Number of WBCs in a specified vol. of blood 4,500 to 10,500 per microliter Differential WBC count % of the diff. types of WBCs •Segmented neutrophils: 34 to 75% •Band neutrophils: 0 to 8% •Lymphocytes: 12 to 50% •Monocytes: 2 to 9% •Eosinophils: 0 to 5% •Basophils: 0 to 3% Platelet count No. of platelets in a specified vol. of blood 140,000 to 450,000 per microliter COMPLETE BLOOD COUNT (CBC)
REFERENCES  Essential Pathology – Harsh Mohan, 4th ed.  Pathologic Basis of Disease – Robbins & Cotran, 8th ed.  Text book of oral pathology - Shafer’s. 8th ed.  Text book of oral pathology – Neville -  Text book of physiology – Sembulingham  Burket’s oral medicine, 11th edition.  Contemporary OMFP, Sapp. 2nd edition 100
101  Kuesap J, Chaijaroenkul W, Rungsihirunrat K, Pongjantharasatien K, Na-Bangchang K. Coexistence of malaria and thalassemia in malaria endemic areas of Thailand. The Korean journal of parasitology. 2015 Jun;53(3):265.  Diagnosis of Hemophilia & Other Bleeding Disorders. A Laboratory Manual. Steve Kitchen et al. 2nd Ed.  Understanding the Complete Blood Count With Differential. Beverly George-Gay. Journal of Peri Anesthesia Nursing, Vol 18, No 2 (April), 2003: pp 96-117.  WHO. Iron Deficiency Anemia. Assessment, Prevention, & Control. A Guide for Program Managers. 2001.
THANK YOU..!! 102

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RBC DISORDERS.ppt

  • 1. LAB DIAGNOSIS OF RBC DISORDERS - 1 1 SHERIN JAMES
  • 2. CONTENTS Introduction RBC DISORDERS • Anemia Iron deficiency anemia Pernicious anemia Sickle cell anemia Erythroblastosis fetalis Thalassemia Aplastic anemia • Polycythemia vera References 2
  • 4. 4
  • 5. 5
  • 6. 6
  • 7. ERYTHROCYTES Total RBC count Haemoglobin Concentration male -- 14-18gm/dl female -- 12-16gm/dl 7
  • 9. COMPLETE BLOOD COUNT COMPONENTS Hemoglobin, Hematocrit, RBC count. RBC indices, RBC distribution width. WBC count, Differentials & Platelet count. Evaluation of peripheral blood smear morphology.
  • 10. RED BLOOD CELL INDICES. Mean corpuscular volume (MCV): Mean corpuscular hemoglobin concentration(MCHC): Mean corpuscular Hb (MCH): Increased MCHC - Artefacts
  • 11. 12 ADULT REFERENCE RANGES-RED BLOOD CELLS Measurement (units) Men Women Hemoglobin (gm/dL) 13.6-17.2 12.0-15.0 Hematocrit (%) 39-49 33-43 Red cell count (106/μL) 4.3-5.9 3.5-5.0 Reticulocyte count (%) 0.5-1.5 Mean cell volume (μm3) MCV 82-96 Mean corpuscular hemoglobin (pg) MCH 27-33 Mean corpuscular hemoglobin concentration (gm/dL) - MCHC 33-37 RBC distribution width RDW 11.5-14.5
  • 12. MISCELLANEOUS CHANGES 1. Spherocytosis : spheroidal red cells.  hereditary spherocytosis  auto immune hemolytic anaemia  ABO haemolytic disease of newborn. 2. Schistocytosis : fragmentation of erythrocytes.  Thalassemia  megaloblastic anaemia  hereditary elliptocytosis  iron def. anaemia  severe burns. 17
  • 13. 3. Irregularly contracted red cells :  drug & chemical induced hemolytic anaemia  unstable haemoglobinopathies. 4. Sickle cells / drepanocytes :  sickle cell disease. 18
  • 14. 5. Leptocytosis : presence of unusually thin red cells. severe iron def. anaemia thalassemia. 6. Crenated red cells : erythrocytes which develop numerous projections from the surface. Coarsely crenated red cells are called Acanthocytosis.  splenectomy pts  chronic liver disease. 19
  • 15. 8. Burr cells : cell fragments having one or more spines. Found in --- uraemia. 9. Stomatocytosis : stomatocytes have central area having a slit like or mouth like appearance. • hereditary stomatocytosis • alcoholism. 10. Ovalocytosis / elliptocytosis: oval or elliptical shape of red cells. • hereditary ovalocytosis • Elliptocytosis 20
  • 16. 21
  • 17. Red cell disorders Anemias ↓red cell mass Polycythemia ↑ red cell mass
  • 18. Anemias ↓red cell mass Blood Loss Hemolytic ↓Erythropiesis B12, Folate, Iron ↓
  • 19. Anemias ↓red cell mass ↓Erythropoiesis Megaloblastic B12,Folate Iron deficiency Anemia of Chronic disease Aplastic anemia Pure red cell aplasia Others
  • 20. Anemias ↓red cell mass Blood Loss Acute Chronic
  • 21. ANEMIA 30 • Anemia refers to reduction in 1. Red blood cell count 2. Hemoglobin content 3. Packed cell volume •Hb conc. in blood below the lower limit of the normal range for the age & sex of the individual. • lowered ability of the blood to carry oxygen.
  • 22. 31 MORPHOLOGICALLY – Average Size Of The Cells And The Hb Concentration (Wintrobe’s): - Macrocytic: megaloblastic - Normochromic, normocytic: Acute post hemorrhagic, hemolytic, aplastic - Hypochromic, Microcytic: Iron Deficiency, Thalassemia, Chronic Post Hemorrhagic
  • 23. 1. Loss of blood: • Acute posthemorragic anemia • Chronic posthemorrhagic anemia 2. Excessive destruction of RBC: a) Extra corpuscular causes: Antibodies, Infection(malaria), • Splenic sequestration and destruction • Drugs, chemicals and physical agents b) Intracorpuscular haemolytic disease 1) Hereditary • Disorder of glycolysis, abnormalities in RBC membrane. • Abnormalities in synthesis of globin 2) Acquired – lead poisonong 3. Impaired blood production resulting from deficiency of substances essential for erythropoiesis a. Iron deficiency b. Deficiency of vitamin B12 , folic acid and Protein deficiency 4. Inadequate production of mature erythrocytes a) Deficiency of erythroblasts b) Pure red cell aplasia c) Infiltration of bone marrow- Leukemia, lymphoma, Multiple myeloma d) Endocrine abnormality- myxedema e) Chronic renal disease f) Chronic inflammatory disease g) Cirrohisis of liver ETIOLOGIC CLASSIFICATION OF ANEMIA:
  • 24. 33
  • 25. WE COVER - Iron Def. Anemia Megaloblastic Anaemia Sideroblastic Anemia Aplastic Anemia Sickle Cell Anemia Thalassemia 35
  • 26. 36
  • 27. 37 Haemoglobin Concentration gm/dl Adult male 14-18 Adult female 12-16 Children 17-20 New born 10-12
  • 28. 1. IRON DEFICIENCY ANAEMIA Develops when the supply of iron is inadequate for the requirement of hb synthesis. Increased blood loss Increased requirements Inadequate dietary intake Decreased intestinal absorption 38
  • 29. CLINICAL FINDINGS  Chronic fatigue  Pallor of conjunctiva and palmer creases.  Palpitations  Shortness of breath, numbness  Craving for Ice (pica)  Pallor of the conjunctiva, lips, and oral mucosa;  Brittle nails with spooning, cracking,  Splitting of nail beds, koilonychia  Palmar creases  Bone pain  Chronic iron deficiency: Esophageal webs: causes dysphagia to solid foods. Achlorydia.
  • 30. 44 ORAL MANIFESTATIONS • Angular cheilitis, • Glossitis with different degrees of atrophy of fungiform and filliform papillae • Pale oral mucosa • Oral candidiasis • Recurrent aphthous stomatitis • Erythematous mucositis • Burning mouth
  • 31. 45 PLUMMER-VINSON SYNDROME Classic triad : Upper esophageal webs or strictures + Dysphagia + Iron deficiency anemia Etiopathogenesis : • Unknown - iron deficiency (Fe dep oxidative enzymes) • Malnutrition, • Genetic predisposition and Autoimmune processes. Treatment: Iron supplementation
  • 32. INVESTIGATIONS 1. BLOOD PICTURE & RED CELL INDICES i. Hb : fall in Hb conc. ii. Red cells : • Microcytic Hypochromic Red Cell. • Anisocytosis & Poikilocytosis Seen. • Target Cells, Elliptical Forms & Polychromatic Cells Often Present. iii. Reticulocyte count : normal or reduced iv. Absolute values : reduced MCV, MCH, MCHC, Increased RDW v. Leucocytes : normal vi. Platelets : increased 46
  • 35. 2) BONE MARROW FINDINGS i.Marrow cellularity : incerased due to erythroid hyperplasia. ii.Erythropoiesis : micronormoblastic (small polychromatic normoblasts). iii.Other cells : Myeloid, lymphoid & megakaryocytic cells are normal in no & morphology. iv.Marrow iron : deficient reticulo-endothelial iron stores. 49
  • 36. 3) BIOCHEMICAL FINDINGS i.Serum iron level – low 50µg/dl. (normal – 80-180µg/dl) ii.Total iron binding capacity (TIBC) – high (normal –250-450µg/dl) iii.Serum ferritin – low (normal – 150-2000ng/dl) iv.Red cell proptoporphyrin – low ( normal 20-40µg/dl) 50
  • 37. 51
  • 38. TREATMENT • Ferrous sulphate orally. • Hct Should increase 0.5% to 1%/day. • Lack of response indicates: -Noncompliance. -Continued blood loss. -Iron is not being absorbed.
  • 39. 2. MEGALOBLASTIC ANAEMIA  Cells have small nuclei & large mature cytoplasm – Numerous large erythroid precursor series (THYMIDINE)  Impaired DNA synthesis - characterized by distinctive abnormality in the haemopoietic precursors in the bone marrow in which the maturation of the nucleus is delayed relative to that of the cytoplasm. 53
  • 40. 2A. PERNICIOUS ANAEMIA • 1st described by Addison in 1855. • Source: µorg:, Dietary animal prdcts • 2.4µg/day RDA (3 mg – stored) • Associated with gastric atrophy & lack of intrinsic factor (IF) production. • Neuropsychiatric, digestive symptoms • Hunter’s glossitis, beefy, glossodynia, lingual paresthesia 54
  • 41. 1. BLOOD PICTURE & RED CELL INDICES : i. Hb : fall in conc. ii. Red cells : o Macrocytotic Red Cell. o Anisocytosis, poikilocytosis & presence of Macro-ovalocytes. o Basophilic Stippling, Howell Jolly Bodies also seen iii. Retciulocyte count : low to normal iv. Absolute values : ↑MCV, MCH & normal or reduced MCHC v. Leucocytes : reduced. Hyperseg neutrophils vi. Platelets : reduced. INVESTIGATIONS 55
  • 44. BONE MARROW FINDINGS - i.Marrow cellularity : hypercellular with decreased myeloid – erythroid ratio. ii.Erythropoiesis : megaloblastic erythropoiesis. Megaloblasts are abnormal, large, nucleated erythroid precursors. iii.Marrow iron : increase in number & size of iron granules. BIOCHEMICAL FINDINGS - i.Rise in serum unconjugated bilirubin. ii.Serum iron & ferritin – normal iii. Increased MMA, Homocysteine 58
  • 45. SPECIAL TESTS TESTS FOR VIT. B12 DEF. 1)Serum Vit B12 Assay – 2 methods : Microbiologic assay & radioassay. 2) Schilling Test (radioisotope absorption test) 3) Antibody test for IF (I, II) TREATMENT: Parental and oral - cyanocobalamin 59
  • 46. 2B. FOLATE DEF. ANEMIA •RDA 400µgm. (Diet) •Normal serum folate is 6-12 ng/ml. •Hyperpigm of skin, mucosa •Below 4ng/ml is diagnostic of folate deficiency + normal levels of MMA 1.Measurement of forminoglutamic acid (FIGLU) urinary excretion after oral administration of histidine. Its excretion is increased if folate def. is present. Because folic acid is required for conversion of FIGLU to glutamic acid. 2.Serum Folate Assay : by microbiological & radioassay. TREATMENT 1-5 mg FA daily, Orally. 60
  • 47. 3. APLASTIC ANAEMIA Paul Ehrlich, introduced the concept of aplastic anemia in 1888. 1n 1904 it was termed as aplastic anemia by Chauffard. Pancytopenia +Hypocellular bone marrow Normal hematopoietic tissue in the bone marrow has been replaced by fatty marrow. Acquired/ Genetic Mucosal pallor, gingival bleeding, hyperplasia, mucosal ulceration. 61
  • 48. INVESTIGATIONS 1. Anaemia – Hb level <10gm. Blood picture shows normocytic normochromic red cells. Reticulocyte count is reduced or zero. 2. Leucopenia – Neutophil < 500/Cu.mm with relative lymphocytosis. 3. Thrombocytopenia – platelet count <20,000/µL, Prolonged bleeding time 4. Bone marrow aspiration – reduction of myeloid cells, megakaryocytes & erythroid cells. 5. BM: empty marrow, fat spaces, 62
  • 50. 4. HAEMOLYTIC ANAEMIAS  Results from an increased destruction of RBC.  Qualitative defect -- Sickle cell Anaemia 64
  • 51. 65 Destruction of fetal blood - r/n between maternal and fetal blood factors.  Inheritance by the fetus of a blood factor from the father that acts as a foreign antigen to the mother.  Rh factor, named after the rhesus monkey, was discovered by Landsteiner and wiener in 1940 as a factor in human RBC that would react with rabbit antiserum produced by administration of RBC from the rhesus monkey. ERYTHROBLASTOSIS FETALIS
  • 52. 66 PATHOGENESIS Transplacental transfer of this antigen, transplacental leaks of RBC From the fetus to the mother Immunization of the mother, formation of antibodies which When transferred back to the fetus by the same route Produce fetal hemolysis • If both parents are homozygously Rh positive→ infant will be Rh positive → no maternal immunization. • If mother is homozygously positive but father is Rh negative → no maternal immunization. • If father is Rh- positive and mother is Rh- negative → fetus inherits parental factor, which may act as an antigen to the mother and immunize her with resultant Ab formation.
  • 53. 67
  • 54. 68 CLINICAL FEATURES • Some infants are stillborn. • Anemia with pallor, Jaundice • Compensatory erythropoiesis • Fetal hydrops ORAL MANIFESTATION • Deposition of blood pigments in the enamel and dentin (green/brown/blue) • Ground sections- positive test for bilirubin • Intrinsic stains • Enamel hypoplasia • Rh hump
  • 55. 69 Laboratory findings • RBC count decreased, large number of normoblasts or nucleated red cells • Icterus index high • Positive direct coombs test on cord blood Treatment • At present, Rh-negative mothers are being given anti-D gamma globulin to prevent immunization
  • 56. SICKLE CELLANEMIA MC Hereditary chronic hemolytic anemia Mendalian dominant, nongender linked Exclusively - Mediterranean origin. A concordance exists between the prevalence of Malaria and HbS HbA is genetically altered to produce HbS, Substitution of valine for glutamine at the sixth position of the β globin chain Homozygous, Heterozygous 70
  • 57.  RBCs - normal biconcave discoid shape distorted, generally presenting a sickle-like shape.  Reduces both their plasticity and lifetime from normal 120 days average down to 14 days.  This results in the underlying anemia and hypertrophic bone marrow.  In heterozygote- 40% of hemoglobin is HbS, 60% HbA  In homozygote- nearly all hemoglobin is HbS Deoxygenatio n of the heme moiety of HbS Hydrophobic interactions between adjacent Hbs molecule Aggregate into larger polymer- viscous gel Long needle like fibres within cell Stiff, distorted, Classic sickle shape Obstructs microcircu lation – ischemia, infacrtn, organ damage Hypoxia → promotes sickling
  • 58. COMPLICATIONS • Pain • Lethargy • Lifelong anemia (low RBC) • Organ failure • Stroke CLINICALLY; • Before The Age Of 30 Years • Cerebrovascular Accidents/ Strokes, • Aplastic Crises Leading To Severe Anemia, • Chronic Leg Ulcers, • Hematuria, • Aseptic Osteonecrosis, • Retinitis Leading To Blindness • Splenic Sequestration, Renal Failure • Acute Chest Syndrome - Fever, Cough, Sputum Prodctn, Dyspnea, Or Hypoxia.
  • 59. CHEEK BONES MAXILLAR OVERGROWTH BM EXPANSION CHRONIC PAIN – TMJ GINGIVAL EDEMA/ENLRGMNT OSTEOMYELITIS OSTEOPOROSIS STEP LADDER – INTRTRBECLR SPACING BONE WITHIN BONE SKULL X-RAY- CREW-CUT 73
  • 60. INVESTIGATIONS  RBC- may reach a level of 1,000,000 cells per cubic mm.  Moderate – severe anaemia (Hb conc is 6-9g/dl).  PCV – 17-29%  ^ WBC (N), Platelet  High reticulocyte count- Anemia  Increased marrow response.  Elevated lactic dehydrogenase and decreased levels of hepatoglobin- confirms hemolysis  ^ unconju Billirubin BLOOD SMEAR:  Blood film shows sickle cells & target cells.  Presence of Howell – jolley bodies. 74
  • 61. DIAG TEST: Hb analysis – PROTEIN ELECTROPHORESIS/ LIQUID CHROMATOGRAPHY
  • 62. 5. THALASSAEMIA COOLEYS / ERYTHROBLASTIC ANEMIA • Thomas B Cooley in 1925. • Diverse group of hereditary disorders in which there is reduced rate of synthesis of one or more of the globin polypeptide chain. • Depending on presence of genetic defect in alpha or beta globin chain genes o Alpha – thalassaemias o Beta – thalassaemias 76
  • 63. 77 ALPHA AND BETA THALASSEMIAS
  • 66. β-THALASSEMIAS β0 mutations - no β-globin chains are produced β+ mutations - reduced (but detectable) β- globin synthesis Cortical thinning of bones facial bones. expansion of hematopoietic ally active marrow ^ maxillry antrum Mongoloid  "crew-cut" appearance - skull X-rays  Splenomegaly ( up to 1500 gm)  Hemosiderosis and secondary Hemochromatosis RODENT FACIES - develops mongoloid features due to prominence of the cheeks, protrusion of the maxillary anterior teeth, depression of the bridge of the nose.
  • 67. CLINICAL FEATURES INEFFECTIVE ERYTHROPOIESIS AND HEMOLYSIS – Expanded marrow fills intramedullary space & invades bony cortex - Impaired Bone Growth, Skeletal Deformities – Extramedullary hematopoiesis & hyperplasia of mononuclear cells - Splenomegaly, Hepatomegaly, Lymphadenopathy – Ineffective erythropoietic precursors consume nutrients - Growth Retardation and Cachexia – Severe hemosiderosis – Occurs within the first 2 years of life. – Yellowish pallor of the skin – Fever, chills, malaise, – Generalized weakness – Splenomegaly and hepatomegaly
  • 69. 83 1. Silent Carrier State • single α-globin gene is deleted • insufficient to result in anemia, completely asymptomatic 2. α-Thalassemia Trait • deletion of two α-globin genes • clinical picture identical to β-thalassemia minor • small red cells (microcytosis), minimal or no anemia, and no abnormal physical signs 3. Hemoglobin H Disease • only one normal α-globin gene • moderately severe anemia 4. Hydrops Fetalis • Evident By- Third Trimester Of Pregnancy • Excess Γ-globin Chains Form Tetramers (Hemoglobin Barts) Has High Affinity For Oxygen & No Oxygen To Tissues • Fetus - Severe Pallor, Generalized Edema, And Massive Hepatosplenomegaly (Similar To In Erythroblastosis Fetalis) α-THALASSEMIAS
  • 70. LAB FINDINGS Hypochromia, Microcytosis, MCV, MCH, MCHC – reduced, Anisocytosis And Poikilocytosis Target Cells, Fragmented RBC (Schistocytes) Basophilic Stippling Serum bilirubin – elevated. Supravital staining- Methyl blue demonstrate inclusion bodies. Reticulocytosis Normoblasts (Nucleated RBC), Safety pin cells in the circulating RBC Hbf - Markedly Increased (Major Red Cell Hemoglobin) Bone marrow- cellular hyperplasia with large number of immature, primitive and stem form of RBCs. Best Confirmatory Test -Hemoglobin Electrophoresis Bone Marrow-mild Erythroid Hyperplasia
  • 72. • β-thalassemia minor and α-thalassemia trait – Peripheral blood smears show small (microcytic) and pale (hypochromic) RBCs but regular in shape – Target cells
  • 74. 88 FESSAS BODIES: Intracellular inclusion bodies, leads to increased erythrocyte haemolysis and severe ineffective haematopoiesis.
  • 75. 6. POLYCYTHEMIA Increase in circulating red blood cells (RBCs) - increased hemoglobin level. • 2 types- • Relative • Absolute • Primary/PRV/Erythraemia: True idiopathic ^ – neoplastic (UNKNOWN) • Secondary/Erythrocytosis: Enhanced RBC production – Hypoxia - ^EPO PRV/ Vaquez’s Disease: • Chronic stem cell disorder with an insidious onset characterized as a panhyperplastic, malignant and neoplastic marrow disorder. •Mutation – JAK2(single progenitor marrow cell) •^ Accumltn of blood, Flushed skin,Erythromelagia, Spleenomegaly, Myelofibrosis
  • 76. 93 ORAL MANIFESTATIONS • Erythema (red-purple color) of mucosa, G •Submucosal petechiae • Erythematous & edematous gingiva • Spontaneous gingival bleeding
  • 77. 94 LAB FINDINGS : Raised HB concn. - >20gm/dl Erythrocytosis normochromic normocytic- >10,000.000/cubic mm HCT ^ 60% (specific gravity, viscosity ^) Mild – moderate leucocytosis with basophilia Thrombocytosis with defective platelet function - 400,000-800,000/dl Bone marrow examn – erythroid hyperplasia & panhyperplasia, hypercellular.
  • 78. 95
  • 81. TEST WHAT IT MEASURES NORMAL VALUES Hemoglobin Amount of this oxygen-carrying protein within RBCs. Men: 12.7 to 13.7 g/dl Women: 11.5 to 12.2 g/dl Hematocrit Proportion of total blood volume made up of RBCs. Men: 42 to 50% Women: 36 to 45% Mean corpuscular vol (MCV) Average vol. of individual RBCs 86 to 98 femtoliters Mean corpuscular Hb conc (MCHC) Average conc. of Hb within RBCs 33.4 to 35.5 g/dl WBC count Number of WBCs in a specified vol. of blood 4,500 to 10,500 per microliter Differential WBC count % of the diff. types of WBCs •Segmented neutrophils: 34 to 75% •Band neutrophils: 0 to 8% •Lymphocytes: 12 to 50% •Monocytes: 2 to 9% •Eosinophils: 0 to 5% •Basophils: 0 to 3% Platelet count No. of platelets in a specified vol. of blood 140,000 to 450,000 per microliter COMPLETE BLOOD COUNT (CBC)
  • 82. REFERENCES  Essential Pathology – Harsh Mohan, 4th ed.  Pathologic Basis of Disease – Robbins & Cotran, 8th ed.  Text book of oral pathology - Shafer’s. 8th ed.  Text book of oral pathology – Neville -  Text book of physiology – Sembulingham  Burket’s oral medicine, 11th edition.  Contemporary OMFP, Sapp. 2nd edition 100
  • 83. 101  Kuesap J, Chaijaroenkul W, Rungsihirunrat K, Pongjantharasatien K, Na-Bangchang K. Coexistence of malaria and thalassemia in malaria endemic areas of Thailand. The Korean journal of parasitology. 2015 Jun;53(3):265.  Diagnosis of Hemophilia & Other Bleeding Disorders. A Laboratory Manual. Steve Kitchen et al. 2nd Ed.  Understanding the Complete Blood Count With Differential. Beverly George-Gay. Journal of Peri Anesthesia Nursing, Vol 18, No 2 (April), 2003: pp 96-117.  WHO. Iron Deficiency Anemia. Assessment, Prevention, & Control. A Guide for Program Managers. 2001.