Hemolytic Anemias Done by :Aseel Twaijer & Laith Sorour mot common pathway in hemolytic anemia

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Text of Hemolytic Anemias Done by :Aseel Twaijer & Laith Sorour mot common pathway in hemolytic anemia

  • Hemolytic Anemias

    mohammad Typewritten Text Done by :Aseel Twaijer & Laith Sorour

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  • • Hemolytic anemias share the following features:

    - A shortened red cell life < 120 days

    - Elevated erythropoietin levels (compensatory increase in erythropoiesis)

    - Accumulation of hemoglobin degradation products (red cell hemolysis)

    me Highlight In order to say it is hemolytic anemia it should have these 3 features

    me Highlight instead of normal RBC life which is 120 days it can be from 10-90 days

    me Highlight protein comes from kidney stimulates hemoglobin production(or RBCs)

    me Highlight in order to compensate the loss of RBCs (Anemia)*Unless the patient have KIDNEY failure\disease he wont compensate or the erythropoiesis will be LOW

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  • Extravascular hemolysis (within phagocytes): m.C Cause: RBCs deformability sequestration/phagocytosis

    Splenomegaly (hyperplasia of phagocytes) Anemia Jaundice (unconjugated bilirubin) Tx: splenectomy

    me Sticky Note Hemolytic Anemia is divided according to Hemolysis site (extra or intra vascular)

    me Highlight Hemolysis occur outside the blood vessel\In the phagocytes in the reticular system(spleen\liver\bone marrow)

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    me Highlight mot common pathway in hemolytic anemia

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    me Highlight there are some deformaties(factors) that make the RBC more fragile\deformable\rigid and weak*we will talk about them later* so when they enter the spleen the rigid RBCs will be trapped and sequestration(phagocytosis)

    me Highlight because the RBCs are rigid and trapped in the spleen

    me Highlight Enlargment of spleen

    me Highlight There are two types of jaundice 1-Unconjugated(hemolytic\pre-Hepatic) 2-Conjugated(Obstructive or hepatic)

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    me Highlight because it is enlarged we remove part of it

    me Highlight Treatment*

  • Intravascular hemolysis:

    Less common

    Cause:

    mechanical injury (cardiac valves, thrombotic narrowing of the Microcirculation, repetitive physical trauma ) complement fixation

    intracellular parasites

    exogenous toxic factors (clostridial sepsis/release ofenzymes that digest the red cell membrane)

    Anemia hemoglobinemia hemoglobinuria hemosiderinuria jaundice (unconjugated bilirubin)

    Lysed RBCs---- free hemoglobin------haptoglobin------phagocytes

    If haptoglobin ------free hemoglobin/ methemoglobin------ Red-brown urine

    No splenomegaly

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    me Highlight Prosthetic valves(either mechanical or biological) can cause turbulent flow which will lead to Hemolysis of RBCs because of mechanical sharing with valves

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    me Highlight Occur a lot in athletes

    me Highlight Putting antigen on RBCs which will lead to attacking it

    me Highlight like Malaria and helminths(worms)

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    me Highlight Hemoglobin deposition in Blood because of hemolysis of RBCS release its hemoglobi in blood

    me Highlight Blood deposition in Urine

    me Highlight Iron deposition in Urine

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    me Highlight Because there is Sequestration(phagocytosis) in spleen*so spleenoctomy as treatment is INeffective

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    me Highlight Most important finding

    me Highlight Oxidized form of Hemoglobin

    me Highlight Enzyme that binds to Hemoglobin to phagocyte it laterbut since the number free hemoglobin is higher,haptoglobin will decrease and the rest of hemoglobin will enter the urine

  • MORPHOLOGY:

    erythroid precursors (normoblasts) in BM

    prominent reticulocytosis in the p. blood

    Erythropoietin

    Hemosiderosis (accumulation of the iron containing pigment hemosiderin, particularly in the spleen, liver, and bone marrow)

    Extramedullary hematopoiesis (liver, spleen, and lymph nodes)----- Anemia

    pigment gallstones (cholelithiasis)----- biliary excretion of bilirubin

    me Highlight Findings Under the microscope in General Hemolytic Anemia

    me Highlight nucleated RBC

    me Sticky Note As compensatory mechanism Erythropoietin will be increased to stimulate the production of RBCs qnd the number of erythroid precursors will increase leading To BONE MARROW HYPERPLASIA

    me Highlight still not mature RBC *DEnucleated but different from RBC it have a network of rRNA

    me Highlight Compensatory

    me Highlight Accumulation of iron in tissuethere is primary and secondary In hemolytic anemia it is secondary

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    me Highlight If the bone marrow cant compensate it will stimulate the stem cell to differentiate to blood cells in liver\spleen and lymph nodes which can lead to their enlargment(Hepatomegaly\spleenomegaly)

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    me Highlight a sign of Chronic hemolysis

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  • me Sticky Note

    me Sticky Note Abnormal seeing a nucleus in RBC in peripheral blood *A sign of Bone marrow hyperplasia to compensate Anemia

  • me Sticky Note Reticulucyte percentage should be from 1-2%,more tha that will be reticulocytosis which is a sign of bone marrow hyperplasia to compensate the anemia

  • Hereditary Spherocytosis

    autosomal dominant

    defects in RBC membrane skeleton---spheroid RBCs---- less deformable ----splenic sequestration/destruction

    HS is caused by diverse mutations that lead to an

    insufficiency of membrane skeletal components.

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    me Sticky Note Most of Hemolytic anemias are inherited

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    me Highlight The membrane skeleton is rigid and cant be squeezed because the surface area is less than the size of RBC

    me Highlight because the surface area is less than cell size, we can see this in any defect that affect the membrane skeleton of RBC

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    me Highlight ***Extravascular Hemolysis

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    me Sticky Note Type of Extravascular hemolytic anemia

  • • Young HS red cells are normal in shape, but the destabilized lipid bilayer sheds membrane fragments as red cells age in the circulation.

    • the life span of the affected red cells is decreased on average to 10 to 20 days from the normal 120 days.

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    me Sticky Note any mutation in these proteins can lead to sphere shape*the biggest protein is Spectrin

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    me Sticky Note acidosis

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  • • MORPHOLOGY:

    Spherocytosis ----------- destinctive but not pathognomonic

    Other features of hemolytic anemias

    Moderate splenic enlargement (500-1000 gm)

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    me Highlight we can see it in other anemias

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    me Sticky Note normal spleen weight 100-150 gm

  • me Sticky Note compact\spheroid\no central pallar

  • • Clinical Features:

    family history

    RBCs---hypotonic salt solutions---osmotic lysis (influx of water) mean cell hemoglobin concentration (dehydration loss of K+ H2O)

    If compensated by erythropoiesis----------- asymptomatic

    If decompensated-----hemolytic anemia--- Splenomegaly Anemia Jaundice

    me Highlight since it is hereditary

    me Highlight low salt

    me Highlight sign of rigid membrane(spherocytosis)

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    me Sticky Note Extra information: even normal RBCs will be lysed in hypotonic solution,but the difference is the concentration where it will be lysed\\\\\example:if normal one lyses at 5g\L the sphere will be lysed at 6g\L since it has higher MCHC and more rigid(less deformable)

    me Highlight Bone marrow is functional

  • • aplastic crises ---- acute parvovirus infection.

    • Hemolytic crises----intercurrent events leading to increased splenic destruction of red cells (e.g., infectious mononucleosis)

    • Gallstones

    me Sticky Note Aplastic anemia: deficiency of all types of blood cell caused by failure of bone marrow development.***not hemolytic

    me Highlight Parvovirus attack normoblasts (erythroid precursors) which will lead to defficiency in RBCs because there wont be compensation

    me Highlight Epstein-Barr virus\EBV\HHV-4

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    me Sticky Note Leads to splenomegaly which will lead to increased sequestration on RBCs

    me Highlight Occur with any chronic hemolytic syndrome

  • • Splenectomy treats the anemia and its complications