Hema. Mod.A Lec3

Question 1

What’s Anisopoiklocytosis ?

Answer 1

Different sizes and shapes of RBCs , present in many types of anemia like :

1-iron deficiency anemia
2- hemolytic anemia
3- sickle cell anemia
4- thalassemia

Question 2

Hct value

Answer 2

Low = it is anemia

High = polycythemia ( means cells more than plasma) ( occurs in cases of dehydration )

Question 3

Definition Of Anemia

Answer 3

Reduction in the hemoglobin concentrations of the blood below the normal range for age and sex.

Question 4

Signs of anemia depend on:

Answer 4

1-onset of anemia ( rapid or slow )
2- severity ( less than 9 )
3- age ( old or young )
HB dissociation curve : increased 2,3 DPG

Question 5

Anemia Symptoms (6)

Answer 5

Fatigue
Irritability
Weakness and lethargy
Decreased appetite
Frontal headache
Unusual food craving ( pica )

Question 6

General Signs Of Anemia (4)

Answer 6

Pallor of the mucous membrane
 Tachycardia
 Systolic flow murmur
 Cardiac failure

Question 7

Specific Signs Of Anemia:

Answer 7

 Koilonychia (spoon nail): iron deficiency anemia
 Jaundice: Hemolytic anemia or megaloblastic Anemia
 Leg ulcer: Sickle cell anemia
 Bone deformities: Thalassemia major

Question 8

What are the sites to see pallor?

Answer 8

1. Nails
2. Conjunctiva
3. Palm
4. Tongue

Question 9

Do all cases of reduced hemoglobin have anemia? Yes or No

Answer 9

In some cases plasma volume is increased ( like : anemia or
hypervolemia ) leading to dilution of of RBCs resulting in
false anemia

Question 10

hypervolemia occurs in

Answer 10

(splenomegaly , pregnancy)

Question 11

In apparent polycythemia

Answer 11

decreased plasma volume than

polycythemia making blood more viscous

Question 12

What are the causes of anemia?

Answer 12

1-IDA : the most common type ( due to decreased iron )
2-megaloblastic A : due to low VIT-B12 and folate
3-hemolytic A : due to infection , drugs , hypersplenism
,tumors
4-Aplastic anemia : due to bone marrow diseases
5- sickle cell anemia :due to inherited gene mutation

Question 13

Features of extravascular hemolysis :

Answer 13

1- Occurs in RES ( spleen , BM , liver )

2- Presence of urobilinogen and stercobilinogen

Question 14

Features of intravascular hemolysis :

Answer 14

1- Occurs inside vessels
2- Presence of methaemalbumin
3- Hemosiderinuria
4- Hemoglobinuria
5- Absent haptoglobin and hemopexin

Question 15

Schumms test

Answer 15

Detects precence of methaemalbumin

Question 16

G6PD deficiency:

Answer 16

G6PD= glucose 6 phosphate dehydrodenase deficiency
 Produce NADPH ( antioxidant =prevent
oxidation of RBCs)
 x-linked disease (male only is affected)
 is example of intra vascular hemolysis

due to destruction of RBCs

Question 17

Types Of G6PD deficiency:

Answer 17

1- Mediterranean type : more serious because hemolysis occurs all the time

2- African type : hemolysis occurs only when exposed to any oxidants

Question 18

Clinical Picture Of G6PD deficiency (3)

Answer 18

 Acute hemolytic anemia in response to oxidant stress
 Neonatal jaundice
 A congenital non‐spherocytic haemolytic anemia

Question 19

Diagnosis Of G6PD Deficiency:

Answer 19

same as diagnosis of hemolytic anemia)

1-Screening tests
 Hemoglibinuria
 Jaundice
 Reticulocytosis
 Low RBCs
 LDH : high

2-Direct enzyme assay .

Question 20

Normal Reticulocytic count

Answer 20

0.5 – 2 %

Question 21

Causes of increase reticulocytes count: -

Answer 21

 Hemolytic anemia
 Chronic hemolysis
 During the response to treatment with a specific hematinic ( agents that increase blood cells or HB formation )

Question 22

 Causes of low reticulocytes count in anemic patients: - (5)

Answer 22

 Bone marrow failure
 A deficiency of a hematinic
 Lack of EPO ( in renal disease )
 Ineffective erythropoiesis
 Chronic inflammatory or malignancy

Question 23

Q. If there is bleeding.

What is the response of the body with regards to EPO and Retics?

Answer 23

EPO and Retics will be increased

Question 24

Effective Erythropoiesis:

Answer 24

It is the balance between the number of cells produced and their life span and function.

Question 25

what differentiates between effective and ineffective erythropoiesis ?

Answer 25

Reticulocyte index (higher than 2 is effective)

Question 26

Ineffective Erythropoiesis:

Answer 26

10-15% of developing erythroblasts die within bone marrow without producing mature cells Reticulocyte Index <2 HIGH LDH HIGH INDIRECT BILIRUBIN

Question 27

Tests of hemolysis in hemolytic anemias (6)

Answer 27

1. Indirect bilirubin (unconjugated) 2. Lactic dehydrogenase enzyme( increased in hemolytic anemia and ineffective erythropoiesis ) 3. Urobilinogen : in EVH 4. Urinary hemosiderin : in IVH 5. Haptoglobin : absent in IVH 6. Schumm’s test : for detection of methemalbumin and severity of IVH

Question 28

Hemoglobin abnormalities

Answer 28

1.Synthesis of abnormal hemoglobin (sickle). 2.Reduced rate of synthesis of normal α, or β globin chains (the α and β thalassaemias). 3.Combined 1,2 (sickle thalassemia) : if father is carrier of thalassemia and mother is carrier of sickle

Question 29

Inheritance of sickle cell disease

Answer 29

Both mother and father must be carrier

Question 30

Etiology of sickle cell disease

Answer 30

Caused by mutation which change A into T leading to conversion of glutamate into valine , so BETA globin chains are changed

Question 31

Pathogenesis of sickle cell disease

Answer 31

``` HbS is insoluble  Hypoxia ( deoxygenated HbS) cause polymerization of it leading to sickling of cells result in occlusion of blood vessels Causing infarction ```

Question 32

Clinical Picture Of Sickle Cell Disease

Answer 32

1. Severe hemolytic anemia 2. Other organ damage  Hand or feet swelling , painful , redness  Brain stroke  Auto-splenectomy عنده تضخم اصلا وعندما تسوء الحالة تحس ان الطحال متشال  Pulmonary hypertension  Infections  Ulcers of the lower legs  Gall bladder stones ( due to increased bilirubin )

Question 33

What are the types of crises present in SCD?

Answer 33

1) Vaso‐occlusive crises  Painful , swelling , painful , redness of hand or feet of baby ( hand foot syndrome )  Visceral  acute sickle chest syndrome ( due to sickling of small blood vessels in lung leading to pulmonary infarction )  Splenic sequestration ( painful enlarged spleen ) انحباس الدم كله في الطحال 2) Aplastic crises Due to infection with parvovirus ( B19 virus - which cause infection in hemolytic anemia causing pancytopenia because it infect BM ) and are characterized by a sudden fall in hemoglobin and reticulocytes . B19 virus : cause swelling joints , arthritis 3) Haemolytic crises Characterized by an increased rate of hemolysis and fall in hemoglobin but rise in reticulocytes

Question 34

What are the factors that precipitate the crises ?

Answer 34

* Infection * Acidosis * Dehydration * Deoxygenation * High altitude * Operations * Obstetric delivery * Stasis of the circulation * Exposure to cold * Violent exercise

Question 35

Diagnosis of SCD

Answer 35

1) Complete blood count (what is the type of anemia?) 2) Blood film ( sickling of RBCs ) 3) Hb electrophoresis: (diagnostic test )  HB-A : absent  S- band : HBS present  HBF : low  HB-A2 : low 4) Sickling test Add substance to stimulate hypoxia ( if sickling occurs , so it is sickle cell disease) 5) Solubility test In sickle cell disease: hemoglobin is insoluble ( Turbidity of tube ) 6) Both normoblast and retics are present in peripheral blood

Question 36

Diagnosis of SCT

Answer 36

 HbA is present  HbA2 : present  HbS : not exceed 45%  HBF : low Hematuria is the most Common symptom. Why? •HbS from 25–45% of the total hemoglobin. •Care must be taken with anesthesia, pregnancy and at high altitudes.

Question 37

How to manage a case of sickle cell disease?

Answer 37

 Prophylactic –avoid precipitating factors.  Regular folic acid.  Pneumococcal, Hemophilus and meningococcal vaccination.  Crises –treat by rest, warmth, rehydration by oral fluids and/or intravenous normal saline and antibiotics.  Analgesia.  Blood transfusion if there is very severe anemia with symptoms.  Exchange transfusion.  neurological damage, visceral sequestration crisis or repeated painful crises.