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Rbc disorders 1
 

Rbc disorders 1

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    Rbc disorders 1 Rbc disorders 1 Presentation Transcript

    • RBC Disorders Dr.CSBR.Prasad, M.D.,
    • Clinical presentations
    • Fatigue
    • Pallor / Anemia
    • Spoon nails [Koilonychia]
    • Jaundice
    • Prolonged Neonatal jaundice
    • Hepatosplenomegaly
    • Hepatosplenomegaly
    • Delayed mile stones +organomegaly
    • PigmentedGall stones in young Persons
    • Ruddy facies ofpolycytehmia
    • Some terms• Anisocytosis• Poikilocytosis – Schistocytes – Stomatocytes – Elliptocytes – Sickle cells – Spherocytes• Polychromasia• Normocyte• Microcyte• Macrocyte• Rouleaux formation• RBC Agglutinates
    • Normocyte
    • Microcyte
    • Macrocyte
    • Spherocyte
    • Anisopoikilocytosis
    • Polychromatic cells
    • Hypochromia
    • RBC agglutinates
    • Rouleaux formation
    • The nucleated RBC in the center contains basophilic stippling of the cytoplasm. This suggests atoxic injury to the bone marrow. Such stippling may also appear with severe megaloblasticanemia.
    • Sickle cell anemia
    • RBC Indices• HGB• RBC count• HCT• MCV• MCH• MCHC• RDW
    • Here is data from a CBC in a person with iron deficiency anemia. Note the lowhemoglobin (HGB). Microcytosis is indicated by the low MCV (mean corpuscularvolume). Hypochromia correlates here with the low MCH (mean corpuscularhemoglobin).
    • The CBC here shows a markedly increased MCV, typical for megaloblastic anemia. TheMCV can be mildly increased in persons recovering from blood loss or hemolyticanemia, because the newly released RBCs, the reticulocytes, are increased in size overnormal RBCs, which decrease in size slightly with aging.
    • This CBC demonstrates findings suggestive of spherocytosis, a condition in which theRBCs are small and round (rather than the normal biconcave appearance) withincreased hemoglobin content. This is indicated here by the increase MCHC (meancorpuscular hemoglobin concentration). There is a rare condition known as hereditaryspherocytosis. Also, RBCs in the condition of autoimmune hemolytic anemia can alsoappear similarly.
    • Anemias
    • Symptoms of Anemia • Fatigue • Shortness of breath • Dizziness • Pale or yellowish skin • Swollen tongue that may appear dark red • Weight loss • Diarrhea • Numbness or tingling in your hands and feet • Muscle weakness • Irritability • Unsteady movements • Mental confusion or forgetfulness
    • Anemia Definition:Decrease in hemoglobin concentrationbelow the normal value for the age, sex and for the given individual
    • Classification of anemiasThere are many classifications of anemia• Based on underlying mechanisms• Morphologic characteristics
    • Anemias due to BLOOD LOSS Mechanism Specific Examples BLOOD LOSS Acute blood loss Trauma Chronic blood loss Gastrointestinal tract lesions, gynecologic disturbances
    • INCREASED RED CELL DESTRUCTION (HEMOLYSIS)Inherited genetic defects Red cell membrane disorders Hereditary spherocytosis, hereditary elliptocytosis Enzyme deficiencies Hexose monophosphate shunt enzyme G6PD deficiency, glutathione synthetasedeficiencies deficiency Glycolytic enzyme deficiencies Pyruvate kinase deficiency, hexokinase deficiency Hemoglobin abnormalities Deficient globin synthesis Thalassemia syndromes Structurally abnormal globins Sickle cell disease, unstable hemoglobins(hemoglobinopathies)Acquired genetic defects Deficiency of phosphatidylinositol-linked Paroxysmal nocturnal hemoglobinuriaglycoproteinsAntibody-mediated destruction Hemolytic disease of the newborn (Rh disease),Mechanical trauma transfusion reactions, drug-induced, autoimmune disorders Microangiopathic hemolytic anemias Hemolytic uremic syndrome, disseminated intravascular coagulation, thrombotic thrombocytopenia purpura Cardiac traumatic hemolysis Defective cardiac valves Repetitive physical trauma Bongo drumming, marathon running, karate choppingInfections of red cells Malaria, babesiosisToxic or chemical injury Clostridial sepsis, snake venom, lead poisoningMembrane lipid abnormalities Abetalipoproteinemia, severe hepatocellular liver diseaseSequestration Hypersplenism
    • DECREASED RED CELL PRODUCTIONInherited genetic defects Defects leading to stem cell Fanconi anemia, telomerase defectsdepletion Defects affecting erythroblast Thalassemia syndromesmaturationNutritional deficiencies Deficiencies affecting DNA B12 and folate deficienciessynthesis Deficiencies affecting hemoglobin Iron deficiency anemiasynthesisErythropoietin deficiency Renal failure, anemia of chronic diseaseImmune-mediated injury of Aplastic anemia, pure red cellprogenitors aplasiaInflammation-mediated iron Anemia of chronic diseasesequestrationPrimary hematopoietic neoplasms Acute leukemia, myelodysplasia, myeloproliferative disorders ( Chapter 13 )Space-occupying marrow lesions Metastatic neoplasms, granulomatous diseaseInfections of red cell progenitors Parvovirus B19 infectionUnknown mechanisms Endocrine disorders, hepatocellular liver disase
    • END
    • Dr.CSBR.Prasad, M.D.,Associate Professor of Pathology,Sri Devaraj Urs Medical College, Kolar-563101, Karnataka, INDIA. csbrprasad@reiffmail.com