4. “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
18. G6PD deficiency anemia
• Due to point mutation or deletion of enzyme G6PD causing less
activity of the enzyme than normal.
• Oxidant stress examples:
1. Drugs
2. Infections
3. Favism
19. Diagnosis of G6PD
• Blood test for G6PD enzyme level
• Blood film during crises showing blister and bite cells
36. Sickle cell anemia
• Inherited Structural abnormality
• Substitution of glutamic acid by valine
37. Clinical manifestation
• Episodes of pain (crisis)
• Painful swelling of hand and fit
• Frequent infection
• Delayed growth due to lowering O2
• Vision problems
39. Investigation and diagnosis
• Blood film: normochromic, microcytic,
• retoculocytosis 10-20%
• Sickling test
• HbS 80-95% in severe cases
40. Iron deficiency anemia
• Iron deficiency is cause defect in hemoglobin synthesis , result in the
RBC is smaller than normal (microcytic , hypochromic)
41. Cause of iron deficiency anemia
1. Inadequate iron intake
2. Mal absorption of iron
3. Increase iron requirement
4. Increase iron loss
5. Chronic blood loss
6. Subtotal or complete gastrectomy
42. Sings and syntom
A. General fatigue
B. weakness
C. Pale skin
D. shortness of breath
E. dizziness
F. tongue swelling
G. cold hands and feet
H. tachycardia
I. brittle nails
J. headaches
43. Diagnosis of iron deficiency anemia
Blood film
Microcytic and hypochromic
CBC
54. 2. CBC
A. RBC count reduce
B. Heamoglobin reduce
C. Mcv increase
D. MCH increase
E. MCHC normal or reduce
55. 3. Bone marrow finding:
A. Hypercellular marrow.
B. Erythropoisis, with megaloblastic maturation.
C. Giant metamyelocytes.
56. 4.Biochemical finding:
A. Vitamin B12 reduce
B. Folate acid reduce
C. Unconjugate bilirubin increase
D. Serum iron and fertin may be normal or elevated
61. REFERENCE
• Hoff brand essential heamatology 7th 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
Editor's Notes
In developing embryos, blood formation occurs in aggregates of blood cells in the yolk sac, called blood islands. As development progresses, blood formation occurs in the spleen, liver and lymph nodes. When bone marrow develops, it eventually assumes the task of forming most of the blood cells for the entire organism.[1] However, maturation, activation, and some proliferation of lymphoid cells occurs in the spleen, thymus, and lymph nodes. In children, haematopoiesis occurs in the marrow of the long bones such as the femur and tibia. In adults, it occurs mainly in the pelvis, cranium, vertebrae, and sternum.[8]
Macrophage phagocytes rbcs then destruct it to haem and globin, the globin part reused again, and haem changes to protoporphyrin and iron, iron release into circulation, and protoporphyrin change to bilirubin in liver, bilirubin relaeased to bile then released ethier by stool or urine
Only mention two or three examples of types, and in RBC low and high means RBC count with microcytic, if its high you must say its bcz compensatory action of bone marrow, bcz in thalassemia minor our BM try to produce more RBCs so lead to high RBC
First class we will discuss Haemolytic anemia
Results from increasing rate of RBC destruction
Life span of RBCs less than 3 days
Intrinsic defect: hereditory
hemoglobinopathies:like thalassemia ,sickle cell anemia
Enzymopathies : like G6PD deficiency anemia
Membrane cystoskeletal defect: spherocytosis,eleptocystosis
Acquired
Paroxysmal nocturnal hemoglobinuria: rare life threatening disease characterized by RBC destruction by complement system due to defective presence of a protein called DAF on the RBC(complement decay acclererating factor) its function is to inhibit complement system from RBC decstruction
Extrinsic defect: hereditary
Familial uremic hemolytic syndrome: rare renal failure, anemia, thrombocytopenia, is two types typical whichs due to ingestion of specific strain of bacteria release shi
Ga toxin and cause enterohemorrhage means in intestine, atypical due to genetic mutation
Acquired:
Mechanical destructiuon like red cell fragmentation syndrome: The red cell fragmentation syndrome can occur due the blood vessels or vascular malformations
Toxin agent domestic chemical substances
Drugs associated
Infection b y malaria
Autoimmune warm and cold
Reduced serum haptoglobin(is a protein that binds to free haemoglobin released from erythrocyte ,hemoglobin haptoglobin complex removed by spleen, bcz in
Defect in protein due to gene mutation involved in interaction between the membrane cystoskeleton and the lipid bilayer of the RBC (Vertically), the cell membrane of RBC become fragile and unstable at early life’s RBC they are Biconcave but when they circulate become spherocyte,the cell membrane produce small fragment and in the macrophage the small fragements deattached and released as SPherocytes
The RBC become more hypertonic
Means it’s not autoimmune haemolytic anemia
Normochromic microcytic
There is no cure for HS, but it can be treated. The severity of your symptoms will determine which course of treatment you receive. Options include:
Surgery: In moderate or severe disease, removing the spleen can prevent common complications that result from hereditary spherocytosis. Your red blood cells will still have their spherical shape, but they’ll live longer. Removing the spleen can also prevent gallstones.
Not everyone with this condition needs to have their spleen removed. Some mild cases can be treated without surgery. Your doctor might think less invasive measures are better suited for you. For example, surgery is not recommended for children who are younger than 5 years.
Vitamins: Folic acid, a B vitamin, is usually recommended for everyone with HS. It helps you make new red blood cells. A daily dose of oral folic acid is the main treatment option for young children and people with mild cases of HS.
Transfusion: You may need red blood cell transfusions if you have severe anemia.
Before we discuss this types, we mention that RBC have a pathway called hexose monophosphate pathway, in this pathway generate NADPH whichs important for maintain reduced glutathione,which is important to prevent damage from free radicals
Any response to oxidant stress leading to intravascular haemolysis,because G6PD is not worked properly
Drugs like premaquine sulphonamied,nitrofurantoins
Infections like viral hepatitis , pneumonia
Favism : ingestion of fava beans
Yellowish skin due to execcive RBC destruction
Dark urine due to intravascular haemolysis
Commonly found in ethnic groups, whose origins are near the medesterian sea and equatorial or near equatorial region of Asia;middle east
Commonest group of AR in India
Carier frequencies vary from 3% to 17% in different populations,with over 30 million people carring the defective gene
About more than 9000 thalasemic children are born every year
We classified like that bcz normal hemoglobin molecule contains 2 beta and 2 alpha chain.
Defect in each chain lead to thalassemia
Beta: characterized by genetic deficiency in the synbthesis of beta globin chain
Alpha: change in the genes for alpha globin production
Present before age of 2 years
Transfusion dependent
When theres excessive decrease in the beta globin change leading to decrease adult haemoglobin and as compensatory mechanism the body produce Haemopoisis in the spleen and liver which produce fetal haemoglobin ,then leading to improper delivery of oxygen to the tissue and hepatospleenomegaly,in the other hand the exess alpha chain in RBC precursor produce defect in RBC metabolism and membrane leading to destroying RBC in the extravascular organ like bone marrow
Iron overload : Due to excessive rbc destruction
Jaundice due to excessive unconjugated bilirubin
Skull deformity due to erythroid hyperplasia and intramedullary expansion and cortical bone thining
Heart failure due to iron overload and deposition in epicardium
To prevent iron overload and decrease risk of heart failure we use iron chelating drugs like deferoxamine
Spelenectomy to decrease RBC destruction and decrease transfusion requirement, and to prevent complications caused by splenomegaly
Why RBC above normal bcz bone marrow try compensate of abnormal Hb
Generally develops in first years of life , intial signs may not be noticed until during routingly haematological analysis
Survive adult to life
Electrophresis: showing HbA with 5-40% HbH
HbH appear in blood film as golf ball like appearance
Premaretal screening to identify coules at risk of bearing affected children, by estimating (looking HbA2 and HbF level, prenatal screening allows the couples the choice of termination
Tests one or two cells from each embryo for a specific genetic abnormality , usese for IVF embryos
form of assisted reproductive technology that combines preimplantation genetic diagnosis with in vitro fertilization may help parents who have thalassemia or who are carriers of a defective hemoglobin gene give birth to healthy babies
Is a type of anemia results from genetic change of Hb due to exchange of Glutamic acid by valine amino acid
RBCs become rigid and sticky and sickle shape
RBCs is insoluble and form crystal when expose to low oxygen
There are not enough healthy RBC to carry adequate oxygen
Major symptoms of sickle cells anemia:
Pain develop when sickle block blood flow through tiny blood vessel to ur chest, abdomen and joints
Painful swelling due to blocking blood flow
Sickle cell can damage organs that fight against infection
Acute chest syndrome characterized by fever, and difficulty in breathing, caused by lung infection
Blindness due to chronic blood vessel obstruction
Sickling test :
Iron deficiency anemia is the most common type of anemia, and it occurs when your body doesn’t have enough of the mineral iron. Your body needs iron to make hemoglobin. When there isn’t enough iron in your blood stream, the rest of your body can’t get the amount of oxygen it needs.
Daily requir ment is 1 mg for male
And 2 mg for female
2. Malabsorbtion due to hypochloryhydria and diarrhea
3. As in childeren and pregnancy
4 as injury
5 menstral and menoposal bleeding
VITAMIN B12 IS IMPORTANT FOR SYSNTHESIS DNA AND DEFICIENCY OF VIT B12 LEAD TO DELAY NUCLEAR MATURATION AND CELL DIVISION .
VIT B12 SYNTHESIS IN HUMAN LARGE BAWEL BUT CAN NOT ABSORBED , HUMAN DEPEND ON DIETARY SORCE . AFTER ABSORBED FROM LOWER ILIUM STORE IN LIVER . THE STORED B12 MAY LAST FOR 3 YEARS DAILY REQUIRMENT IS 1 -2 MG PER DAY
FOLIC ACID IS Is imprtant for sysnthesis dna and rna and to repair and aiding in rapid cell devision and growth and to produce healthy rbc
Folic acid is absorbed in jejenum daily requement is 400 mg per day
Folic acid is stored in liver
