Unit 3 - Formative 8 D3

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Crew Cut Appearance

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Beta Thalassemia Major;

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Beta Thalassemia Major

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B chain is absent -> severe anemia requiring blood transfusion (due to secondary hemochromatosis)
Marrow expansion (crew ut on skull x-ray) -> skeletal deformities; chipmunk facies

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Beta Thalassemia Minor

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B chain is underproduced, usually asymptomatic, diagnosis onfirmed by increased HbA2

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Hydrops Fetalis

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Deletion o fall 4 alpha globin genes (alpha-thalassemia); hemoglobin Barts (Excess gamma globin chains form tetramers)

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Paroxysmal Nocturnal Hemoglobinuria

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Mutation in PIGA -> GPI linked complement regulator proteins deficient -> nocturnal red cell lysis due to increased complement activity secondary to decrease in pH during sleep

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Classic paroxysmal nocturnal hemoglobinuria presentation

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episodic red/brown urine (due to hemosiderinuria) = iron deficiency ; prothrombotic state (leading cause of death in those w/ PNH)

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Immunohemolytic Anemia

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Antibodies lead to premature destruction of RBC

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Direct Antiglobulin Test (direct Coombs test)

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For immunohemolytic annemia;

Pts red cells mixed w/ serum containing antibodies that target human immunoglobulin or complement; if ig/complemtn are present = agglutination

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Indirect Antiglobulin Test (indirect Coombs test)

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Pt serum mixed w/ rbc w/ known antigens; if ab present for given antigen agglutation occurs

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Warm Autoimmune Hemolytic Anemia

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IgG Ab that do NOT fix complement and are active at 37C

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Cold Autoimmune Hemolytic Anemia

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IgM antibodies that agglutinate red blood cells at low temperatures (below 30C); happens in periphery (fingers, toes, nose)

IgM binding fixes complement

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Tx for autoimmune hemolytic anemia?

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Steroids; splenectomy; immunosuppresive therapy

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Paroxysmal Cold Hemoglobinuria (Cold Hemolysin Hemolytic Anemia)

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IgG autoantibodies bind the P blood group ag in the cooler regions of the body; complemt mediated hemolysis occurs when circulation reaches warmer regions; possibly fatal intravascular hemolysis

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Drug Induced Immune Hemolytic Anemia; Drug Absorption Mechanism

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The drug binds to the red blood cell membrane and the antibody attaches to the drug without interacting with the erythrocyte
An example is penicillin

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Drug Induced Immune Hemolytic Anemia; Immune Complex Mechanism

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The antibody reacts with new antigenic sites created by the combination of the drug and the membrane
An example is quinidine

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Drug Induced Immune Hemolytic Anemia; Autoimmune Mechanism

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Antibodies bind to the red cell membrane in a manner indistinguishable from autoimmune hemolytic anemia
An example is alpha methyl dopa

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Hemolytic Anemias Resulting from Trauma to RBC

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Cardiac Valve Protheses (turbulent flow = shear forces)
Vessel narrowing due to fibrin deposition = shear stresses/cell damage

Peripheral blood smear findings = schistocytes (helmet cells, triangle cells)

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How to differentiate between megaloblastic anemia caused by folate or B12 deficiency?

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Increased homocysteine but with ELEVATED methylamalonic acid in B12 deficiency (normal in folate deficiency)

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Megaloblastic Anemia

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Impaired DNA synthesis secondary to inadequate B12 or folate levels (thymidine) -> maturation of nuclesy delayed relative to maturation of cytoplasm

Morphologic finding: hypersegmented neutrophils)

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Pernicious Anemia

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Type of megaloblastic anemia secondary to autoimmune gastritis; vitamin B12 deficiency due to impairment of intrinsic factor production)

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Transferrin

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Iron binding protein which transports iron in the serum; delivers iron to cells

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Ferritin

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Protein iron complex in liver, spleen, bone marrow, skeletal muscle

Used as a marker for body iron stores

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Hepcidin

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Peptide that regulates iron absorption; inhibits transfer of iron from enterocytes to the plasma; if the body has adequate iron stores elevated hepcidin levels inhibit iron absorption into the blood (if iron stores low, hepcidin levels decrease and iron absorption unimpaired)

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Ascorbic Acid

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Enhances absorption of inorganic iron

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Tannates (tea)

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Impairs absorption of inorganic iron

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Most common causes of iron deficiency

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Adult men/postmenopausal women: GI blood loss until proven otherwise

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Anemia of Chronic Disease (ACD)

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Inflammation -> increase hepcidin -> decreases absorbed iron

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Iron Deficiency Anemia (Microcytic, Hypochromic)

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Decreased iron due to chronic bleeding, malnutrition/absorption disorders or increased demand (pregnancy) -> decreased heme synthesis

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Plummer Vision Syndrome

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Esophageal webs; microcytic hypochromic anemia; atrophic glossitis

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Pica

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Desire to consume items such as clay or flour due to iron deficiency

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Fanconi Anemia

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Inherited defect in DNA repair; aplastic anemia

Upper extremity deformities w/ radius, wrist and thumb abnormalities most prevalent

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Aplastic Anemia Pathogenesis

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Pancytopenia w/ severe anemia, neutropenia and thromboytopenia; normal cell morphology but hypocellular bone marrow w/ fatty infiltration

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Aplastic Anemia Causes

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Failure or desturction of myeloid stem cells due to radiation/drugs, viral agents, fanconi's anemia, idiopathic

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Aplastic Anemia bone marrow histology

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Diminished hematopoietic cells; fat cells, fibrous stroma, lymphocytes/plasma cells (marrow becomes mostly fatty)

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Pure Red Cell Aplasia

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Marrow disorder resulting in suppression of erythroid progenitors; often secondary to thymoma

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Myelophthisic Anemia

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Space occupying lesion destroy marrow architecture/depress productive capacity; most common cause is metastatic cancer

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Chronic Renal Failure w/ Secondary Anemia

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Decreased synthesis of erythropoietin

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Hepatocellular Liver disease w/ secondary anemia

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Decreased marrow function; erythroid precursors primarily effected

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POlycythemia

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Elevated hematocrit associated w/ increased hemoglobin levels

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Relative polycythemia

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Decreased plasma volume associated w/ dehydration

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Stress Polycythemia

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Gaisbock Syndrome; idiopathic

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Primary Absolute Polycythemia

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Increased hematocrit due to myeloid neoplasm (polycythemia vera); RBC precursors proliferate in an erythropoietin independent fashion

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Secondary Appropriate Polycythemia

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Increased erythropoietein stimulate red cell progenitors (may be compensatory or pathologic)

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Reticulocyte Production Index (RPI)

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MEasure of the degree of reticulocytosis

RPI <2 = inadequate bone marrow response
RPI > 2 = appropriate bone marrow response for the degree of anemia

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Laboratory Tests which indicate hemolysis

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1. Decreased haptoglobin
2. Elevated bilirubin
3. Elevated LDH
4. Presence of urine hemoglobin or hemosiderin
5. Plasma hemoglobin

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Why would an RPI be greater than 2?

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Acute blood loss; hemolysis

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Causes of microcytic anemia

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Iron deficiency anemia; anemia of chronic disease; thalassemia; sideroblastic anemia

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Sideroblastic Anemia

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Bone marrow produces ringed sideroblasts rather than healthy bone marrow