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Introduction to Hematology and Anemia
 

Introduction to Hematology and Anemia

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    Introduction to Hematology and Anemia Introduction to Hematology and Anemia Presentation Transcript

    • Introduction to Hematology and Anemia Dr. Kalpana Malla MD Pediatrics Manipal Teaching HospitalDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
    • Blood• Blood volume is about 8% of body weight• 45 % is formed elements• 55% plasma
    • PLASMA 90 % Water 10 % Solutes - Plasma proteins – Albumins(58 %) - maintain osmotic (oncotic) pressure Globulins (38 %) - antibodies synthesized by plasma cells Clotting factors – fibrinogen – 4 %
    • FORMED ELEMENTS• Three types: Erythrocytes – red blood cells Leukocytes – white blood cells Thrombocytes – platelets – cell fragments
    • Development of hemopoietic system: 3 anatomic stages: Mesoblastic: in extraembryyonic structures - yolk sac (10- 14 days of gestation till 10-12 wks) Hepatic: liver 6-8wks gestation - 20-24wks-primary site of blood cell production (continues till remainder of gestation) Myeloid: bone marrow (10-12 weeks) Exception: lymphocytes –bone marrow+ other organs
    • Developmental changes:• 2nd to 3rd trimester: circulating erythrocytes and granulocytes increase• 2nd trimester - Haematocrit levels rise 30-40 % & at term rise is 50-63 %• Platelet concentration remains constant from 18th wks till term• Life-span of RBCs ~60- 90 days in newborns vs 120 days
    • • Fetal bone marrow space develops - 8th wk of gestation• Neutrophils first observed ~ 5 wks of gestation• 14th wk to term: most common cell found in bone marrow is neutrophil• Red marrow:• Newborns- in all cavities of bones• Older children & adults - in upper shaft of femur, humerus, pelvis, spine, skull and bones of thorax• Erythropoiesis: - In-utero controlled by erythroid growth factors produced by monocyte-macrophages of fetal liver - After birth controlled by erythropoietin from kidneys
    • The Red Cell• Average life span = 120 days (60-90 days NB)• Cleared by RES (spleen, liver primarily)• Homeostasis daily loss = daily production• Otherwise anemia
    • Hemoglobin:• Is complex protein - Made up of heme which contains an atom of iron and 4 polypeptide globin chains – reversible transport of Oxygen without expenditure of metabolic energy – Oxygen binds to iron in heme (also CO) – 23 % of CO2 is bound to globin portion• If there is a problem with any part of the molecule it may not be functional
    • Types of Hb:• Embryonic Hb: Gower-1: ζ2 ε2 Gower-2: α2 ε2 Portland: ζ2 2• Fetal Hb: Hb F: α2 2• Adult Hb: Hb A : α2 β2 Hb A1: α2 δ2
    • Developmental changes in Hb:• 4-8 wks gestation: Gower Hb predominates; disappears by 3rd month• > 8th wk of gestation- Hb F predominant Hb• ~ 24 wks gestation 90 % of total Hb• At birth declines to 70 %• 6-12 months postnatal life < 2 %
    • Developmental changes in Hb:• 16-20 wks gestation- some Hb A detectable• 24th wk gestation: 5-10 % , At term ~ 30 %Hb A present• Hb A2 - < 1 % - At birth - 2-3.4 % At 12 months (normal level)• Throughout life ratio of Hb A: Hb A2 is ~ 30:1
    • Hgb Norms• Normal values vary by age and gender – High at Birth 20g/dl since HbF has high affinity for oxygen , by 3 months HbA replaces HbF – Falls to lower-than-adult values by 3-6 months – Rises gradually to adult value by the early teenage years – On average, Adult male Hb 2g/dl > female counterpart due to effect of androgen .
    • Hgb and MCV Variability Age Hgb mean MCV mean birth 16.5 108 2 wk 16.5 105 2 mo 11.5 96 6mo-2yr 12.5 77 2-6 yr 12.7 80 10-12 yr 13.5 85 12-17 14 85 Adult 14-16 90Contemporary Pediatrics, Vol 18, No. 9
    • GRANULOCYTES• Neutrophils – phagocytes• Eosinophils – red granules, associated with allergic response and parasitic worms• Basophils – deep blue granules - Release heparin and histamine
    • Neutrophil
    • Eosinophil
    • Basophil
    • AGRANULOCYTES• Granules too small to be visible• Monocytes – become macrophages• Lymphocytes – B cells and T cells = immune functions
    • Monocyte
    • Lymphocyte
    • Lab Investigation• Table: Laboratory Tests in Anemia Diagnosis• i. Complete blood count (CBC)• A. Red blood cell count• 1. Hemoglobin• 2. Hematocrit• B. Red blood cell indices• 1. Mean cell volume (MCV)• 2. Mean cell hemoglobin (MCH)• 3. Mean cell hemoglobin concentration (MCHC]• 4. Red cell distribution width (RDW)C.
    • RBC indices• Part of the (CBC) -• Mean cell volume(MCV) – Quantifies average red blood cell size• Mean cell hemoglobin (MCH) –Hb amount per red blood cell• Mean cell hemoglobin concentration (MCHC) - The amount of hemoglobin relative to the size of the cell (hemoglobin concentration) per red blood cell
    • Contd -Red Blood Cell Indices Index Normal Value• MCV = 90 ± 8 hematocrit /red cell count (80 – 100) femtoliter• (MCH) = 30 ± 3 pg Hb /red cell count (27 - 31)picograms/cell• (MCHC)= 33 ± 2 Hb/hematocrit or MCH/MCV (32 – 36) gm/dl
    • Anemias - based on cell size (MCV) and amount of Hgb (MCH)• MCV < lower limit of normal: microcytic anemia• MCV normal range: normocytic anemia• MCV > upper limit of normal: macrocytic anemia• MCH < lower limit of normal: hypochromic anemia• MCH within normal range: normochromic anemia
    • Mentzer index• Calculated number to help differentiate between iron deficiency vs. thalassemia if having microcytic anemia• MCV/RBC• >13 iron deficiency• <13 thal trait
    • Red Cell Volume Distribution Width (RDW)• Reflects the variability in cell size• Aids in further differentiating between specific etiologies of microcytic, normocytic, and macrocytic• RDW = (Standard deviation of MCV ÷ mean MCV) ×
    • Contd• C. White blood cell count• 1. Cell differential• 2. Nuclear segmentation of neutrophils• D. Platelet count
    • Blood smear• Assess the size, color, and shape of red cells– Look for abnormalities – macrocyte, leptocyte, target cell, Tear drop, Elliptocytosis,burr cell,acanthocyte, Schistocytes,Spherocytosis,Sickle cells,Poikilocytes – Anisocytosis, Polychromasia
    • MACROCYTE Larger than normal >8.5 µm diameter
    • LEPTOCYTE Hypochromic cell with a normal diameter and decreased MCV Thalassemia.
    • TARGET CELL Hypochromic with central "target" of hemoglobin. Liver disease, thalassemi a, hemoglobin D, postsplenectomy
    • TEAR DROP CELLDrop-shaped erythrocyte, often microcytic. Myelofibrosis and infiltration of marrow with tumor. Thalassemia
    • ELLIPTOCYTE Oval to cigar shaped. Hereditary elliptocytosis, certain anemias (particularly vitamin B-12 and folate deficiency)
    • ECHINOCYTE (BURR CELL) Evenly distributed . spicules on surface of RBCs, usually 10- 30. Uremia, peptic ulcer, gastric carcinoma, pyruvic kinase deficiency
    • ACANTHOCYTE Five to 10 spicules of various lengths and at irregular interval on surface of RBCs.
    • STOMATOCYTE • Slitlike area of central pallor in erythrocyte. Liver disease, acute alcoholism, malignan cies, hereditary stomatocytosis, and artifact
    • SCHISTOCYTE Fragmented helmet- or triangular-shaped RBCs. Microangiopathic anemia, artificial heart valves, uremia, malignant hypertension
    • Microcytic and Hypochromic Smaller than normal ( <7 µm diameter Less hemoglobin in cell. Enlarged area of central pallor.
    • Elongated cell with pointed ends.Sickle cell Hemoglobin S and certain types of hemoglobin CSpherocyte Loss of central pallor, stains more densely, often microcytic. Hereditary spherocytosis and certain acquired hemolytic anemias.poikilocytosis & variation in shape and variation in size anisocytosis
    • Contd• II. Reticulocyte count• III. Iron supply studies• A. Serum iron• B. Total iron-binding capacity• C. Serum ferritin, marrow iron stain
    • Contd• IV. Marrow examination• A. Aspirate• 1. E/G ratio• 2. Cell morphology• 3. Iron stain• B. Biopsy• 1. Cellularity• 2. Morphology E/G ratio, ratio of erythroid to granulocytic precursors.
    • Reticulocyte Count• Reticulocyte production index• RPI= Retic ct x Hb(obsv)/ Hb(normal) x0.5• Indicates whether the BM is appropriately responding to anemia• RPI >3 : inc prod = blood loss/hemolysis• RPI <2 : dec prod / ineffective prod
    • CLASSIFICATIONINADEQUATE RESPONSE (RPI <2)• Hypochromic, microcytic• Normocytic normochromic• MacrocyticADEQUATE RESPONSE (RPI >3)• Hemolytic anemia• Blood loss
    • ANEMIA
    • What is Anemia?• Anemia is defined as a reduction of the red blood cell (RBC) volume or hemoglobin concentration below reference level for the age and sex of the individual• Hb < - 2SD or 95th centile for age and sex
    • Anemia BasicsAll anemias are either due to….1. Ineffective RBC production or2. Accelerated destruction of the RBC
    • Classification• By RBC morphology and By Etiological factors responsible for anemia
    • Microcytic hypochromic anemia1. Iron deficiency anemia – nutritional, posthemohragic2. Ineffective Erythropoiesis - Abnormal hemoglobinopathies, Thalassemia syndrome, - Lead poisoning, Cu deficiency, - Pyridoxine responsive -chronic ds - infection, inflammations , renal ds
    • MICROCYTICTAILS P:• T - Thalassemia A - Anemia of chronic disease• I - Iron deficiency anemia• L - Lead toxicity associated anemia• S - Sideroblastic anemia• P – Pyridoxine deficiency
    • Macrocytic anemia• Megaloblastic Erythropoiesisa) Nutritional - Folate deficiency, B12 deficiencyb) Toxic – Treatment with antifolate compound – methotrexate,, and drugs that inhibit DNA replication – zidovudine, phenytoinc) Congenital disorders of DNA synthesis like Orotic aciduria etc.d) Malabsorption - liver ds - normal newborns, reticulocytosis
    • Macrocytic anemia• Non - Megaloblastic Erythropoiesisa) Chronic hemolytic anemiab) Liver dsc) Hypothyroidismd) Diamond blackfan syndrome
    • Normocytic, Normochromic anemia1. Impaired cell production (low reticulocyte count) - aplastic anemia - pure red cell aplasia - physiological anemia of infancy - infections - Systemic diseases like endocrinal, renal and hepatic diseases - bone marrow replacement – leukemia, tumors, starage ds, myelofibrosis, osteopetrosis2 Hemolytic anemia ( reticulocyte count high)
    • DIMORPHIC ANEMIA• When two causes of anemia act simultaneously, e.g : macrocytic hypochromic due to hookworm infestation leading to deficiency of both iron and vitamin B12 or folic acid• following a blood transfusion
    • ETIOLOGICAL CLASSIFICATION OF ANEMIA• Blood loss Acute Chronic• Decreased iron assimilation - Nutritional deficiency - Hypoplastic or aplastic anemia - Bone marrow infiltration like leukemia & other malignancies, - Myelodysplastic syndrome - Dyserythropoietic anemia
    • ETIOLOGICAL CLASSIFICATION OF ANEMIA• Increased physiologic requirement- Extracorpscular - alloimmune & isoimmune hemolytic anemia, microangiopathic anemias, infections, hypersplenism, - Intracorpsular defect – Red cell membranopathy i.e. congenital spherocytosis, elliptocytosis – Hemoglobinopathy like HbS, C,D,E etc. Thalassemia syndrome – RBC enzymopathies like G6PD deficiency, PK deficiency etc.
    • Differential of Anemia Hgb, indices, retic count and smear Inadequate response (RPI<2) Adequate response (RPI>3) r/o blood loss/hemolytic disHypochromic, microcytic Normochromic,normocytic Macrocytic hemoglobinopathy iron def chronic dis B12/folate def enzymopathy thalssemia Ca/BM failure Liver disease membranopathy chronic disease Transient erythroblastopenia Down Syndrome extrinsic factors of childhood (DIC,HUS,TTP) lead poisoning Renal disease Drugs (etoh) Immune Hemolytic anemia
    • Follow-up• Re-check CBC 4-6 weeks (to confirm response)• Continue iron 3-4 months (to replace stores)• Generally, should not need treatment for more than 5 months unless there are ongoing losses• If no improvement on adequate iron therapy, consider evaluating the child for lead poisoning or thalassemia
    • PHYSIOLOGIC ADJUSTMENTS• increased cardiac output• increased oxygen extraction (increased arteriovenous oxygen difference)• shunting of blood flow toward vital organs and tissues• the concentration of 2,3-diphosphoglycerate (2,3-DPG) increases within the RBC• The resultant “shift to the right” of the oxygen dissociation curve, reducing the affinity of hemoglobin for oxygen, results in more complete transfer of oxygen to the tissues
    • CLINICAL FATURES• weakness• tachypnea• shortness of breath on exertion• tachycardia• cardiac dilatation• congestive heart failure• ultimately result from increasingly severe anemia, regardless of its cause.
    • D/D of microcytic anemia: TIBC BM Iron Comment Serum IronIron deficiency D I 0 Responsive to iron therapy Chronic D D ++ Unresponsive to iron inflammation therapy Thalassemia I N ++++ Reticulocytosis and major indirect bilirubinemia
    • Serum iron TIBC BM Comment Iron Elevation of A of fetalThalassemia minor N N ++ hemoglobin, target cells, and poikilocytosisLead poisoning N N ++ Basophilic stippling of RBCsSideroblastic I N ++++ Ring sideroblasts in marrow
    • PYROPOIKILOCYTE • RBCs w/c are extremely sensitive to heat
    • HEMOLYTIC ANEMIA- INTRACORPUSCULAR Hereditary spherocytosis Hereditary elliptocytosis Hemoglobinopathies Thalassemias Congenital dyserythropoietic anemias Hereditary RBC enzymatic deficiencies Paroxysmal nocturnal hemoglobinuria Severe iron deficiency
    • HEMOLYTIC ANEMIA- EXTRACORPUSCULARPhysical agents: Burns, cold exposureTraumatic: Prosthetic heart valves, graft rejectionChemicals: Drugs and venomsInfectious agents: Malaria, toxoplasmosis, leishmaniasisHepatic and renal diseaseCollagen vascular diseaseMalignanciesTransfusion of incompatible bloodHemolytic disease of the newbornCold hemagglutinin d/sAutoimmune hemolytic anemiaThrombotic thrombocytopenic purpura (TTP)Hemolytic uremic syndrome (HUS)DIC
    • Alterations of Hbs by disease:• Gower Hb in few newborns: Trisomy 13/15• Hb Portland: stillborns with homozygous α-thalassemia• Elevated HbF (>2 %): β-thalassemia trait homozygous thalassemia Hb SS, Hb SC preterm infants treated with human recombinant EPO others: hemolytic anemias leukemia aplastic anemia
    • • Hb A2 > 3.4 %: β-thalassemia trait megaloblastic anemia• Decreased Hb A2 :IDA α-thalassemia
    • Thank youDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]