Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan
description
Transcript of Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan
![Page 1: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/1.jpg)
Course title: Hematology (1)Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan
Outcome :The student will know :-The types of hemolytic anemias-The diagnosis of hemolytic anemias -The types of hereditary hemolytic anemias.-The diagnosis of hereditary hemolytic anemias -Types of acquired hemolytic anemias-The diagnosis of acquired hemolytic anemias -The laboratory tests needed for diagnosis
![Page 2: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/2.jpg)
Hemolytic anemia
• Hemolytic anemia = decreased levels of red blood cells (anemia) because of their destruction (hemolysis)
• A red blood cell survives 120 days • The spleen is the main organ which removes old
RBCs from the blood.
![Page 3: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/3.jpg)
Causes of hemolytic anemias can be either:
1 - hereditary.2 - acquired.
![Page 4: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/4.jpg)
Hereditary Hemolytic anemia Defects of hemoglobin
Thalassaemia, Sickle cell anemia Defects of the red cell membrane
Hereditary spherocytosis, Hereditary elliptocytosis
Defective red cell metabolism (enzymes)
G6PD deficiency. P K deficiency
![Page 5: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/5.jpg)
Acquired- Immune mediated : Autoimmune, isoimmune, drugs- Microangiopathic: DIC, HUS- Hypersplenism- Miscellaneous: drugs, toxin, infection, burn, chemical
![Page 6: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/6.jpg)
Signs of hemolytic anemia: Physical
• Symptoms of anemia• Jaundice• Pallor• Splenomegaly / hepatosplenomegaly
![Page 7: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/7.jpg)
Laboratory features Anemia of increased
destruction Normochromic, normocytic anemia Short RBC survival Reticulocytes increased Increased indirect bilirubin Increased LDH
*Peripheral blood smear microscopy: fragments of the red blood cells and spherocytes Normoblasts can be present.Bone marrow smear microscopy: Erytrhroid hyperplasia
![Page 8: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/8.jpg)
Hereditary Hemolytic anemia
Defects of hemoglobin Thalassaemia, Sickle cell anemia
Defects of the red cell membrane
Hereditary spherocytosis, Hereditary elliptocytosis
Defective red cell metabolism G6PD deficiency. P K deficiency
![Page 9: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/9.jpg)
Sickle cell anaemia The abnormalities of the gene may
result from substitution of single amino acid (Substitution of glutamic acid by valine )
The Hb is stable when oxygenated state and become unstable and polymerized on deoxygenated state
![Page 10: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/10.jpg)
Polymerization will lead to precipitation of Hb. The cell become deformed (sickle shape) and very sticky leading to vascular occlusion and small infarction to the affected areas.
Short life span of cells leading to chronic anaemia,
Sickle cell anaemia
![Page 11: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/11.jpg)
Diagnosis 1-Sickling test2- Hemoglobin
electrophoresis:Increased hemoglobin S(90% Hgb S, 10% Hgb F, small fraction of Hgb
A2)
Sickle cell anaemia
![Page 12: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/12.jpg)
HEMOGLOBIN NORMAL ADULT RBC CONSISTS OF
3 FORMS OF Hb: - HbA - 2 α and 2 β globin chains - HbA2 – 2 α and 2 δ globin chains - HbF - 2 α and 2 γ globin chains
![Page 13: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/13.jpg)
![Page 14: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/14.jpg)
![Page 15: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/15.jpg)
Means decrease synthesis of one ofthe globin chain which form normal hemoglobin.
(HbA - 2 α and 2 β globin chains HbA2 – 2 α and 2 δ globin chains HbF - 2 α and 2 γ globin chains )
. The defect may be in alpha chain ( thalassaemia),Beta chain ( thalassaemia) or Delta chain ( thalassaemia)
Thalassaemia
![Page 16: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/16.jpg)
Beta ThalassaemiaDefective chain synthesis
Excess chain Precipitation
cell membrane damageCirculating Red cell
Anaemia
Bone marrow expansion
skeletal changes & hyper metabolism
Erythropoietin increased
Ineffective erythropoiesis
Bone marrow
blood transfusion
Iron overload
Complication and death
Hemolytic
Iron absorption
![Page 17: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/17.jpg)
Beta-Thalassemia major laboratory features
Severe anemia Blood film: microcytic hypochromic ,
target cells, basophylic stippling, reticulocytes increased and normoblasts .
Marrow: marked erythroid hyperplasia, Shortened red cell survival
Haemoglobin electrophoresis : Fetal hemoglobin > 90%, HbA
absent, HbA2 low/normal/high
![Page 18: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/18.jpg)
HEREDITARY SPHEROCYTOSIS Defective or absent spectrin
molecule Leads to loss of RBC membrane,
leading to spherocytosis Decreased deformability of cell Increased osmotic fragility Extravascular hemolysis in spleen
![Page 19: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/19.jpg)
Hereditary spherocytosis (HS) Laboratory features - hemolytic anemia - blood smear spherocytes - increased osmotic fragility time
![Page 20: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/20.jpg)
G6PD DEFICIENCYFunction of G6PD
G6PD
GSSG 2 GSH
NADPH NADP
2 H2O H2O2
6-PG G6P
Hgb
Sulf-Hgb
Heinz bodies
Hemolysis
InfectionsDrugs
![Page 21: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/21.jpg)
Glucose 6-Phosphate Dehydrogenase
Functions
Regenerates NADPH, allowing regeneration of glutathione
Protects against oxidative stress Lack of G6PD leads to hemolysis during
oxidative stress Infection Medications Fava beans
Oxidative stress leads to Heinz body formation, extravascular hemolysis
![Page 22: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/22.jpg)
G6PD DEFICIENCY DIAGNOSIS : QUANTITATIVE ASSAY
DETECTING LOW ENZYME
TREATMENT – SUPPORTIVE AND PREVENTATIVE
![Page 23: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/23.jpg)
Acquired hemolytic anaemia
Due to Antibodies directed against RBC membrane = autoimmune hemolytic anemia destruction of RBC in an enlarged spleen
![Page 24: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/24.jpg)
Introduction Increased RBC Destruction – Short RBC life span <120 days. Normocytic normochromic,
reticulocytosis. Anemia, Jaundice, marrow
hyperplasia Splenomegaly, increased bilirubin
![Page 25: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/25.jpg)
Types of acquired HA AutoImmune Haemolytic Anemias(+ve Direct CoombꞋs) Alloimmune haemolytic anemias Drug-induced immune haemolytic
anemias
![Page 26: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/26.jpg)
Assesment of HA Clinical features: - pallor - jaundice - splenomegaly
![Page 27: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/27.jpg)
Laboratory features:1. Laboratory features - normocytic, normochromic anemia - reticulocytosis - antiglobulin Coombs’ test is positive
2. Blood smear - anisopoikilocytosis, spherocytes - normoblasts - schistocytes
3. Bone marrow smear - erythroid hyperplasia
![Page 28: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/28.jpg)
DIRECT ANTIGLOBULIN TEST (DAT)Coomb′s test
![Page 29: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/29.jpg)
Procedure of DAT1. Take 2-3 drops of blood to be tested in a clean labeled tube.2. Wash the red cells 3-4 times in a large volume of saline to
remove free globulin molecules. Remove all supernatant after each wash. Completely decant the final supernatant wash.
3. Add 2 drops of polyspecific AHG serum in 1 drop of sensitized washed red cells or in 1 drop of 3-5 % suspension of sensitized cells immediately.
4. Mix, Centrifuge at 1000 rpm for 1 minutes immediately.5. Gently shake the tube to dislodge the cell button and see
for agglutination, use optical aid if needed. Record the result.
6. Add 1 drop of IgG coated red cells to a negative test. Mix, centrifuge at 1000 rpm for 1 min. Immediately look for agglutination. If a negative result (no agglutination) is obtained the test result is invalid and whole test should be repeated. If agglutination is obtained, the result is valid.
![Page 30: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/30.jpg)
Indirect antiglobulin (coomb′s ) test
![Page 31: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/31.jpg)
Procedure:1. Place 2-3 drops of the test serum in a tube.
Serum should be fresh for detecting complement components and complement binding antibodies, otherwise, fresh AB serum should be added to it.
2. Add 1 drop of 3-5% suspension of washed O Rh (D) positive red cells to the serum in the tube.
3. Mix and incubate at 37°C for 30-40 minutes.4. Centrifuge at 1000 rpm for 1 minutes.5. Examine for hemolysis and/or agglutination. Use
optical aid if necessary. Agglutination at this stage indicates the presence of saline (complete) antibodies.
6. If no agglutination is seen, wash cells 3-4 times in large volume of saline. Decant supernatant in each wash as completely as possible.
![Page 32: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/32.jpg)
Procedure:7. Add 2 drops of AHG serum to the cells.8. Mix and centrifuge at 1000 rpm for 1
minutes immediately.9. Gently shake the tube to dislodge the
button and examine for agglutination, using optical aid. Record the result.
10. Add 1 drop of IgG coated red cells to any test that is negative. Mix and centrifuge at 1000 rpm for 1 minutes. Look for agglutination. If there is no agglutination, the test result is invalid and the whole test is repeated. If agglutination is obtained the result is valid.
11. Auto control should be kept with IAT.
![Page 33: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/33.jpg)
![Page 34: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/34.jpg)
![Page 35: Course title: Hematology (1) Course code: MLHE-201 Supervisor: Prof. Dr Magda Sultan](https://reader035.fdocuments.us/reader035/viewer/2022062323/568163a7550346895dd4b46b/html5/thumbnails/35.jpg)
Training questions :What are the tests of hemolysis ? How to diagnose Autoimmune hemolytic
anaemia ?
Reference book : Essential Hematology . Dacie .