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    Red Blood Cell Disorders

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    Munawar Adhar
    This document discusses red blood cell disorders and anemias. It covers the main types of anemias including those caused by increased red blood cell destruction (hemolytic anemia), decreased red blood cell production, and blood loss. Hemolytic anemias can be intrinsic defects in red blood cells themselves or due to extrinsic factors that cause premature red blood cell destruction. Anemias of decreased production include aplastic anemia, iron deficiency anemia, and megaloblastic anemias caused by deficiencies in folate or vitamin B12. The document provides details on the causes, signs and symptoms, laboratory findings, treatments and prognoses for these different anemia types.

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    Red Blood Cell Disorders

    Uploaded by

    Munawar Adhar
    938 views56 pages
    This document discusses red blood cell disorders and anemias. It covers the main types of anemias including those caused by increased red blood cell destruction (hemolytic anemia), decreased red blood cell production, and blood loss. Hemolytic anemias can be intrinsic defects in red blood cells themselves or due to extrinsic factors that cause premature red blood cell destruction. Anemias of decreased production include aplastic anemia, iron deficiency anemia, and megaloblastic anemias caused by deficiencies in folate or vitamin B12. The document provides details on the causes, signs and symptoms, laboratory findings, treatments and prognoses for these different anemia types.

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    This document discusses red blood cell disorders and anemias. It covers the main types of anemias including those caused by increased red blood cell destruction (hemolytic anemia), decreased red blood cell production, and blood loss. Hemolytic anemias can be intrinsic defects in red blood cells themselves or due to extrinsic factors that cause premature red blood cell destruction. Anemias of decreased production include aplastic anemia, iron deficiency anemia, and megaloblastic anemias caused by deficiencies in folate or vitamin B12. The document provides details on the causes, signs and symptoms, laboratory findings, treatments and prognoses for these different anemia types.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    938 views56 pages

    Red Blood Cell Disorders

    Uploaded by

    Munawar Adhar
    This document discusses red blood cell disorders and anemias. It covers the main types of anemias including those caused by increased red blood cell destruction (hemolytic anemia), decreased red blood cell production, and blood loss. Hemolytic anemias can be intrinsic defects in red blood cells themselves or due to extrinsic factors that cause premature red blood cell destruction. Anemias of decreased production include aplastic anemia, iron deficiency anemia, and megaloblastic anemias caused by deficiencies in folate or vitamin B12. The document provides details on the causes, signs and symptoms, laboratory findings, treatments and prognoses for these different anemia types.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    of 56

    RED BLOOD CELL (RBC) DISORDERS

    ANEMIA too few RBC or an abnormality low hematocrit or too little hemoglobin Symptoms of anemia: - Fatigue - Chest pain - Weakness - Shock - Syncope - Congestive heart - Decreased appetite failure

    Physical signs of anemia: Pallor of the skin, palmar creases, nail beds, mucous membranes (conjunctiva) Severe anemia heart rate may increase

    RBC disorders are organized into 3 section:

    Increased RBC Destruction (Hemolytic anemia)

    Membrane disorders Enzyme deficiencies Abnormalities of hemoglobin synthesis Antibody mediated destruction Mechanical trauma Aplastic anemia Iron deficiency anemia Sideroblastic anemia Anemia of chronic disease Megaloblastic anemia

    Decreased RBC Production


    Blood Loss

    INCREASED RBC DESTRUCTION (HEMOLYTIC ANEMIA)


    Hemolysis premature destruction of RBCs. RBCs normally live about 80-120 days. Hemolytic anemia increased destruction of red blood cells in the peripheral blood without evidence of ineffective erythropoiesis

    Two types of hemolysis: - inherited intrinsic defect in the RBCs (extravascular) - acquired intravascular abnormalities

    Intrinsic defect in the RBCs (Extravascular) : - RBCs are phagocytized by macrophages in the spleen and liver (macrophage Fc receptors bind Ig attached to RBCs) - Causes include: * hemoglobinopathies (Thalassemia, etc) * RBC membrane abnormalities (hereditary spherocytosis, hereditary elliptocytosis, etc) * abnormality of enzymes (G6PD deficiency)

    Intravascular abnormalities (extrinsic to RBCs): - RBCs lyse in the circulation releasing hemoglobin into plasma which is immediately bound by haptoglobin. Hemoglobin-haptoglobin is cleared from the plasma by hepatic reticuloendothelial cells.
    - Causes include: * mechanical trauma * antibody mediated damage * other toxic or physical insults.

    Clinical features of hemolytic anemia


    May be asymptomatic Moderate: weakness, pallor, icterus Severe: fever, weakness, abdominal pain, dyspnea Physical examination: - pallor of mucous membrane, mild icterus, spleen enlargement - congenital: tower skull, growth retardation, cardiomegaly

    Laboratory findings of hemolytic anemia


    Normochromic, normocytic, normoblast (+) Reticulocytosis, mild leucocytosis with shift to the left, platelet count may be elevated Bone marrow: erythropoietic hyperplasia Indirect bilirubin increases, bilirubinuria (-) Hemoglobin electrophoresis hemoglobinopathy Coombs test: direct and indirect, Ham test, osmotic fragility test, isotop Cr51

    Polychromasi

    Normoblast

    Erythropoietic hyperplasia

    Target cell in Thalassemia

    Differential diagnosis of hemolytic anemia Iron deficiency anemia Pernicious anemia Post massive bleeding Erythroleucemia Aplastic anemia Myelofibrosis

    Complications of hemolytic anemia


    Hemolytic crisis: an acute drop of the hematocrite (Hct) and hemoglobin (Hgb) is accompanied by reticulocytosis. Aplastic crisis: an acute drop of the Hct and Hgb is accompanied by a fall in reticulocytes. Megaloblastic crisis: particularly in hemoglobinopathies Cholelithiasis Post transfusion hepatitis

    Treatment of hemolytic anemia


    Depend on the underlying disease Corticosteroid : prednison 1mg/kg bodyweight Blood transfusion: normal RBCs, washed red cell in hypoxia Splenectomy, indicated on: Hypersplenism Mechanical disturbance Congenital spherocytosis not absolute

    Prognosis of hemolytic anemia

    Determined by the underlying disease

    ANEMIAS OF DECREASED PRODUCTION


    Aplastic anemia Iron deficiency anemia Sideroblastic anemia Anemia of chronic disease Megaloblastic anemia

    Aplastic anemia
    Aplastic anemia is the failure of pluripotential stem cells to produce red cells, white cells, and megakaryocytes. Etiology: Idiopathic about 65% of all cases Secondary aplastic anemia Congenital: Fanconi anemia, dyskeratosis congenita, Schwachman-Diamond syndrome

    Secondary aplastic anemia


    Radiation Viruses: parvovirus, hepatitis, EBV Drugs: marrow suppresive chemotherapeutic agents-alkylating agents, chloramphenicol, phenylbutazone, gold salts, hydantoin Chemical/Toxins: benzene, arsenic Immune disorders: SLE

    Clinical features of aplastic anemia


    Onset may be insidious Erythropoietic hypoplasia pallor, weakness, fatigue Neutropenia fever, chills, pharyngitis or other infections Thrombocytopenia petechiae, bruising, and bleeding

    Physical examination of aplastic anemia


    Evidence of infection : fever Evidence of bleeding: petechiae, oral purpuric lesions (wet purpura), retinal hemorrhages No lymphadenopathy No hepatosplenomegaly No gum hypertrophy

    Laboratory findings of aplastic anemia


    Peripheral pancytopenia, normochromic normocytic anemia. Absolute neutrophil count is the most important prognostic feature < 500/mm3 increased risk of infections Marrow aspirate: numerous spicules with empty fatty spaces and relatively few hematopoietic cells.

    Marrow: Normal cellularity

    Marrow: Hypocellularity

    Severe aplastic anemia


    ( International Aplastic Anemia Study Group) Marrow cellularity < 25% or < 50% with < 30% hematopoietic cells, with at least two of the following: neutrophil count < 500/mm3 platelet count <20,000/mm3 absolute reticulocyte count < 40,000/uml

    Differential diagnosis of aplastic anemia


    Peripheral pancytopenia: Myelodysplastic syndromes Hypersplenism Paroxysmal Nocturnal Hemoglobinuria Pernicious anemia Malignant lymphoma Multiple myeloma Hypothyroidism

    Treatment of aplastic anemia


    Less severe disease (neutrophils > 1000 mm3), do not require red cells or platelet transfusion supportive care Secondary aplastic anemia: therapy the etiology Marrow transplantation: for patients with severe disease Immunosuppressive therapy: ALG and ATG 15 to 40 mg/kg daily for 4 to 10 days

    Glucocorticoids: methylprednisolone 500 to 1000 mg daily for 3 to 14 days Cyclosporine: orally 3 to 7 mg/kg per day for at least 4 to 6 months Anabolic steroids: nandrolone decanoate 400 mg intramuscularly per week Growth factor, cytokine?

    Course and prognosis of aplastic anemia


    Marrow transplantation is curative for 75 to 85% of untransfused patients and for 55 to 60% of those with multiple transfusions. At diagnosis, the prognosis is largely related to the absolute neutrophil and platelet count.

    Iron deficiency anemia


    The most common of all anemias Iron deficiency content of iron in the body is less than normal Iron depletion: earliest stage of iron deficiency; storage iron is decreased, Hgb N Iron deficiency without anemia: decreased storage iron, low serum iron and transferrin Iron deficiency anemia: decreased storage iron, low serum iron, transferrin and Hgb

    Etiology of iron deficiency anemia


    Chronic blood loss (adult) Inadequate dietary iron intake (children) Malabsorption of iron Diversion of iron to fetal and infant erythropoiesis during pregnancy and lactation. Intravascular hemolysis with Hgb-uria Combination

    Clinical features of iron deficiency anemia Anemia develops slowly adaptation of homeostatic mechanism. Symptoms: fatigue, irritability, headache, dyspnea deffort, oedema, epigastric distress, diarrhea Physical examination: pallor, glossitis, stomatitis, angular cheilitis, koilonychia, cardiomegaly, splenomegaly (in a small proportion of patients)

    Laboratory findings of iron def. anemia


    Hypochromic and microcytic anemia Low MCV, MCH, MCHC Reticulocytes decreased Plasma iron concentration diminished Iron-binding capacity increased Low serum ferritin concentration Marrow aspirate: erythropoietic hyperplasia, negative sideroblast (Prussian blue)

    Normal erythrocyte

    Hypochromic microcytic

    Prussian blue

    Differential diagnosis of iron def.anemia


    Thalassemia and hemoglobinopathies Chronic inflammatory states Blockade of heme synthesis caused by chemicals (lead, pyrazinamide, isoniazid) Anemia of myxedema

    Therapy of iron def. anemia


    Source of blood loss should be eliminated High protein diet Iron therapy: oral: 150-200 mg elemental iron daily for 3 months parenteral: 2 ml (100 mg) iron dextran IM or IV once a week, indicated on:

    Malabsorption, intolerance to iron taken orally, uncooperativeness patient, third trimester in pregnancy

    Prognosis of iron deficiency anemia

    Excellent if : the cause of iron deficiency is a benign disorder, bleeding is controlled or can be compensated for by continual iron therapy

    Megaloblastic anemia
    Definition: Disorder caused by impaired DNA synthesis and characterized by the presence of megaloblastic cells Failure of DNA synthesis also affects myeloid and megakaryocytes Most commonly due to a deficiency of folate or cobalamine (vitamin B12)

    Folic acid deficiency

    Caused by: Dietary deficiency Impaired absorption due to small intestinal mucosa abnormality:
    nontropical sprue(celiac disease) tropical sprue other intestinal disorders

    Increased folate requirements: pregnancy

    Clinical features of folate deficiency


    Anemia develops slowly Weakness, palpitation, fatigue, shortness of breath, diarrhea, steatorrhoe, decreased appetite, weight loss, glossitis, paresthesia, irritability, muscle cramp, hemorrhagic diathese Physical examination: Pallor, slight jaundice, dry skin, oedema in extremities

    Laboratory findings of folate deficiency


    Macrocytic anemia, anisocytosis, poikilocytosis Leucopenia with hypersegmentation neutrophil Mild trombocytopenia Low serum folate Marrow aspirate: hypercellular, megaloblastic Bulky stool with an unsually foul odor Hypocalcemia, hypolipidemia, hypoprothrombinemia, low serum nitrogen, fosfor, Na, K, Cl, vitamin A

    Macrocytic erythrocyte

    Macrocytic erythrocyte

    Giant stab cell

    Neutrophyl: hypersegmentation

    Megakaryocyte: hypersegmentation

    Differential diagnosis of folate deficiency


    Pernicious anemia, pancreatic disorder, intestinal lymphosarcoma, amyloidosis, Whiple disease Schilling test to differentiate sprue and pernicious anemia

    Therapy and Prognosis of folate def.


    Folic acid : 15-30 mg/day orally May combine with vitamin B12 if there is neurologic abnormality Low fat, high calories and protein diet Good prognosis

    Pernicious anemia
    A condition in which the portion of gastric mucosa that contains the parietal cells is destroyed through an autoimmune mechanism intrinsic factor secretion decreases cobalamine deficiency Synonim: Addison anemia, Biermer anemia Familial

    Clinical features of pernicious anemia


    Family history of same disease Weakness, weight loss, anorexia, epigastric pain, obstipation, vertigo, paresthesia Physical examination: pallor, papilla atrophy, mild icterus, petechiae, hepatomegaly, splenomegaly(25%), neurologic abnormality (tendon reflex increased, positive Romberg tes)

    Laboratory findings of pernicious an.


    Macrocytic anemia, leucopenia with hypersegmentation, thrombocytopenia Marrow aspirate: hypercellular, megaloblastic, agranuler megakaryocyte, large metamyelocyte and giant stab cell Free gastric acid test: achlorhydria Shortened RBCs survival Schilling test

    Differential diagnosis of pernicious an.


    Acute leucemia Hemolytic anemia Aplastic anemia Liver disease Sprue Hypothyroidism

    Therapy of pernicious anemia


    Parenteral vitamin B12 : 30-100ugr every 34 weeks Patiens with neurologic abnormality: vitamin B12 100 ugr daily until improvement, then 100 ugr every week for 6 months.

    Prognosis of pernicious anemia


    Good if there is no cardiovascular complication or severe infection. Neurologic abnormalities may become normal or persist.

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