Outline Definition Normal RBC destruction Classification Hemolytic anemia Diagnosis and treatment
Haematopoiesis Blood cellular components formation derived from haematopoietic stem cells.
“WITHOUT BLOOD” Decreasing number of RBC , haemoglobin or haematocrit below normal range for age and sex Normal HB range between (13.5 – 17.5) for men, (12-15.5) for women Infants have higher HB level
Normal red cell distruction
Classification:
Tests show increased RBC destructrion Serum unconjugated bilirubin Urobilinogen in urine Stercobilinogen in feces Raised LDH level Serum haptoglobin Hemoglobinuria , hemoglobinemia , hemosedrinuria in intravascular hemolysis.
Intravascular h aemolysis
Intravascular haemolysis Mismatched blood transfusion. G6PD with oxidant stress Red cell fragmentation syndrome Cold autoimmune haemolytic anemia Paroxysmal nocturnal haemoglobinuria
Hereditory Spherocytosis Abnormality of RBC’s membrane AD Jaundice (extravascular haemolysis ) Spleenomegaly Reticulocytosis (5-20%0
Investigation Osmotic fragility Negative direct antiglobulin CBC
Hereditary Elliptocytosis AD, defect in cytoskeletal membrane 90% not anemic ,10% have same clinical manifestation as Spherocytosis
Treatment of spherocytosis and elliptocytosis Surgery Vitamins Blood transfusion
G6PD Deficiency Anemia
G6PD deficiency anemia Due to point mutation or deletion of enzyme G6PD causing less activity of the enzyme than normal. Oxidant stress examples: D rugs Infections F avism
Diagnosis of G6PD Blood test for G6PD enzyme level Blood film during crises showing blister and bite cells
irregularly contracted cells and blister cells
Clinical manifestations Asymptomatic between crises During crises patient experience Yellowish skin Shortness of breath dark urine Haemoglobinemia Haemosiderinuria
Thalassemia Inherited blood disorder. Thalassemia is an AR genetic disorder. Inadequate Hb production
slideplayer.com ThalassemiaGerald A. Soff
C lassifications Beta thalassemia Alpha thalassemia
Beta thalassemia Major Severe form of beta thalassemia Presence of two abnormal genes Caused by mutation of HBB gene on chromosome 11
pathophysiology
Clinical manifestation: anemia Iron overload Pallor Jaundice Skull deformity, mongoloid face Cardiac dysfunction or failure
Treatment: symptomatic relieve Blood tranfusion Iron chelating drugs like D eferoxamine Speleenectomy Bone marrow transplantation
Betathalassemia minor Presence of one abnormal gene Slight reduced Hb level RBC count. Blood film shows anisocytosis , hypochromia , target and tear cells
Diagnosis of beta thalassemia minor Increased HbA2 4 to 7 percent TIBC is normal
Prevention Premarital screening Prenatal diagnosis Pre implantation genetic diagnosis
Sickle cell anemia Inherited Structural abnormality S ubstitution of glutamic acid by valine
Clinical manifestation Episodes of pain (crisis) Painful swelling of hand and fit Frequent infection Delayed growth due to lowering O2 Vision problems
Complications Stroke Acute chest syndrome Pulmonary hypertension Blindness Leg ulcer
Investigation and diagnosis Blood film: normochromic, microcytic, retoculocytosis 10-20% Sickling test HbS 80-95% in severe cases
Iron deficiency anemia Iron deficiency is cause defect in hemoglobin synthesis , result in the RBC is smaller than normal (microcytic , hypochromic)
Cause of iron deficiency anemia Inadequate iron intake Mal absorption of iron Increase iron requirement Increase iron loss Chronic blood loss Subtotal or complete gastrectomy
Sings and syntom General fatigue weakness Pale skin shortness of breath dizziness tongue swelling cold hands and feet tachycardia brittle nails headaches
Diagnosis of iron deficiency anemia Blood film Microcytic and hypochromic CBC
treatment Diet: meat , vegetable ,nuts Iron supplement
What is megaloblastic anemia? Hematological disorder Deficency of folic acid and vit.B12
Vitamin B12 Imprtant for synthesis DNA
Folic acid Important in synthesis DNA
Cause of M egaloblastic anemia B12 deficiency Folic acid deficiency
B . Cause of vit.B12 deficiency Inadequate dietary intake Mal absorption 1.gastric cause 2.intestinal cause
A. Cause of folic acid deficiency Alcoholism Malnutrition Excessive cooking Drug: phenytoin, barbiturate Increase requirement : pregnancy ,lactation
Clinical manifestations: Fatigue Weakness Body ache Vertigo
2. CBC RBC count reduce Heamoglobin reduce Mcv increase MCH increase MCHC normal or reduce
3. Bone marrow finding: Hypercellular marrow. Erythropoisis , with megaloblastic maturation. Giant metamyelocytes .
4.Biochemical finding: Vitamin B12 reduce Folate acid reduce Unconjugate bilirubin increase Serum iron and fertin may be normal or elevated
treatment 1.treatment of B12 deficiency DIET
2. medication: 1000 ug B12 IM once a week for 8 weeks
2.Treatment of folic acid deficiency A. diet : vegetable, nuts, meat
B. medication 5mg/day for 2 – 4 months
REFERENCE Hoff brand essential heamatology 7 th edition Essential of clinical medicine (PROF.DR.MAGDY ISHAK) 2018 22edtion DAVIDSON principle and practic of medicine( BRIAN.R WALKER)2018 22edition DACIDSON ESSENTIAL OF MEDICINE (J.ALASTAIR INNES)2018 2 edition First aid usmle 2018 WWW.HEALTHCARE.COM WWW.MYOCLINIC.COM Google image