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Hematology (lecture 2) Dr Heersh HMH Raof Saeed

Hematology (lecture 2) Dr Heersh HMH Raof Saeed. Hemolytic anemias : ( Hemolysis is defined as the premature destruction of red blood cells (RBCs )). Charachterized by: A nemia results when the rate of destruction exceeds the capacity of the m arrow to produce RBCs( The bone marro

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Hematology (lecture 2) Dr Heersh HMH Raof Saeed

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  1. Hematology (lecture 2)DrHeersh HMH RaofSaeed

  2. Hemolytic anemias:(Hemolysis is defined as the premature destruction of red blood cells (RBCs)) • Charachterized by: • A nemiaresults when the rate of destruction exceeds the capacity of the m arrow to produce RBCs(The bone marro 8 times increased percentage of reticulocytes in the blood.

  3. Hemolytic anemias: • Etiologic classification of H. A. A -Intracorpuscular defects; 1. R. C. membrane disorder as spherocytosis & ovalocytosis (elliptocytosis). 2. R. C. enzyme deficiencies: G6PD, pyrovate kinase deficiency. 3. Ineffective erythropoiesis :as Hemoglobinopathiesas sickle cell disease & Hb C, D. E disease. thalassemia 4. Paroxysmal nocturnal hemoglobinuria. B –Extra corpuscular defects: acquired: 1. Immune hemolytic anemia: -- Isoimmune, as Rh & ABO hemolytic disease. -- Autoimmune. 2.Nonimmune hemolytic anemia: As occurs in severe burns, prosthetic heart valves, bacterial sepsis, malaria, venom & metallic poisoning as lead & copper

  4. Hemolytic anemias: • Clinical manifestation: 1. Anemia: 2. Jaundice 3. Dark urine 4. Various degrees of splenomegaly. 5. Leg ulcers & gall stones are rare complications of some H.A. as S.C.A., thalassemia major & congenital spherocytosis

  5. Hemolytic anemias: • Laboratory: 2. Evidences of increased erythropoiesis: -- Reticulocytosis, -- Increased normoblasts in the B. smear -- Bone marrow expansion, may produce, frontal bossing, mongoloid facies, bone pain & increased liability for fracture. 1. Evidences of increased Hb breakdown: -- Hemoglobinemia (intravascular hemolysis) -- Raised serum bilirubin (mainly indirect bilirubin). -- Hemoglobinuria, released Hb ≥haptoglobin. -- Raised urine urobilinogen. -- Hemosiderinuria,

  6. Hemolytic anemias: • Laboratory: (cont.) 3. Morphological abnormalities of the R. C.: -- Spherocytes : spherocytosis& acquired H. A. -- Elliptocytosis congenital ovalocytosis & rarely in the other forms of H. A. -- Sickle cells in S. C. A. -- Fragmented RC in HUS -- Target cells, in thalassemia, HbC & S.C.A. -- Siderocytes :after splenectomy. (siderocytes are reticulocytes which contain iron granules, which are confirmed by “Prussian blue reaction”) 4. Evidences of shortened RC survival: 51Cr 5. Evidence of increased hemolysis; osmotic fragility test

  7. Hemolytic anemias:(Congenital spherocytosis) • Definition:An A.D. inherited disorder characterized by R.C. stromal protein (spectrin) deficiency which makes the R.Cs. become spherical, rigid & more prone to lyse.

  8. Hemolytic anemias:(Congenital spherocytosis) • Clinical picture: • Various degrees of • anemia, • jaundice • splenomegaly • hyperbilirubinemia (neonatal period) • Aplastic crisis • Hemolytic crisis is less common (leg ulcer and gall stone)

  9. Hemolytic anemias:(Congenital spherocytosis) • INV. • Peripheral film shows microspherocytes • MCH is normal but MCHC is increased. • Increased reticulocyte count (except during aplastic crisis). • Increased S. indirect bilirubin. • Increased osmotic fragility, which becomes more exaggerated after R. C. incubation for 24 hour. • Negative coombs test • Hbelectrophoresis shows normal Hb A. A , Hereditary spherocytosis. B, Hereditary elliptocytosis.

  10. Hemolytic anemias:(Congenital spherocytosis) • Treatment: • Splenectomy • anemia & the accompanying symptoms. • The crisis also disappears • prevents gall stone formation (although spherocytosis & increased osmotic fragility however persist.) • blood transfusion • folic acid supplement are essential.

  11. Hemolytic anemias:Hereditary elliptocytois • rare AD, Mild hereditary elliptocytosis produces no symptom s; more severe varieties can result in neonatal poikilocytosis (shape variation) and hemolysis • In the rare instances when 2 abnormal alleles are inherited (HPP) severe hemolytic anemia • abnormalities of α - and β-spectrin

  12. Hemolytic anemias:Hereditary elliptocytois • Anemia, jaundice &splenomegaly are the main manifestations • Reticulocyte count is increased • osmotic fragility test & autohemolysis are normal. • Splenectomy cures the symptomatic case • Prognosis is good as longevity is not affected

  13. Hemolytic anemias: • Other rare R.C. shape defect which may cause H.A. • Hereditary acanthocytosis; an AR rare disorder in which there is marked irregularity of the RC surface. It’s seen in a syndrome called ”a- betalipoprotienemia): • Steatorrhoea • nervous system degeneration • retinitis pigmentosa • Symptoms are present since infancy & the condition is fatal during childhood. • S. level of cholesterol is low -- Hereditary stomatocytosis; AR or AD in which RC with a slit- like central pallor predominates in the peripheral film. Some patients are symptomatic with anemia, jaundice & splenomegaly. Splenectomy may be beneficial.

  14. Hemolytic anemiasRed cell enzyme defects

  15. This is an acquired type of H A which is caused by genetic deficiency of G6PD. • is responsible for 2 clinical syndromes, • episodic hemolytic anemia, • chronic nonspherocytic hemolytic anemia. • most com m on m anifestation : • neonatal jaundice • episodic acute hem olytic anem ia,

  16. Hemolytic anemias: G6PD deficiency • Hemolysis in the susceptible patients occurs after the administration of one of the following: • Fava beans (ingestion or inhalation of its pollen), (Favism) • Aspirin • Sulphonamide as bactrim, & some food coloring agents which also contains sulfa. • Furadantin & furazolidone • Nalidixic acid (nigram) • Paracetamol • Antimalarials especially primaquine. • Vit.K • Phenacetin. • Chloramphenicol. • Naphthalene. • Ciprofloxacin.

  17. Hemolytic anemias: G6PD deficiency • H.A. follows administration of the mentioned agents by 1-3 days • jaundice, nausea, vomiting, epigastric pain & dark colored urine (hemoglobinurea). • Types of epesodic H.A.: • (G6PD A−) (chronic hemolysis) • G6PD B− (G6PD Mediterranean).enz. Activity hemizygous males is <5% of norm al. • (G6PD Canton) : common in chines

  18. Hemolytic anemias: G6PD deficiency • Laboratory investigations: • Rapid drop in Hb & R.C. counts • Raised reticulocyte count • Hemoglobinemia & hemoglobinurea • Absent haptoglobin • G6PD enzyme activity • Treatment: • Blood transfusion • Sodium bicarbonate;

  19. Hemolytic anemias: • 2- Pyrovate kinase deficiency; • An A.R. disorder which may present in the neonatal period as jaundice &anemia. • During infancy & childhood anemia, jaundice & splenomegaly are present. • Osmotic fragility is normal. • Reticulocytosis is present, with erythroid hyperplasia in the bone marrow. • Diagnosis is confirmed by demonstration of reduced P.K. enzyme activity in the R.C.

  20. Hemolytic anemias: • Treatment: The severity of the disease decreases after childhood • Exchange transfusion may be required in neonates. • Blood transfusion on need • Folic acid supplement • Splenectomy

  21. THANKS

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