Professional Documents
Culture Documents
Mcq-of-Hematology BY ABDULRAHMAN PDF
Mcq-of-Hematology BY ABDULRAHMAN PDF
4. Regarding platelets:
A. They have a life span of approximately 10 days
B. Platelet production is controlled by specific cytokines
C. Platelets should be stored between 2 and 6 degrees C
D. Platelets can be stored for a maximum of 48 hours
E. A pool of platelets transfused can produce an increment of 50 000 / ml if platelet consumption is
not an issue
Ans:-AB
Platelets are produced from megakaryocytes, which are regulated by thrombopoietin, a specific cytokine.
They have a lifespan of 10-12 days thereafter being destroyed in the spleen.
Platelet concentrates should be stored at around 20 degrees C and the pH kept between 6.2 and 7.8 - these
conditions reduce the risk of a change in morphology of the platelets. They should also be continuously
agitated to encourage gas exchange.
One pool of platelets gives on average, an increment of 10 000 / ml.
5. All of the following statements regarding iron deficiency are true Except::-
A. Because absorption of dietary iron is assumed to be about 10%, a diet containing 80-100 mg of
iron daily is necessary for optimal nutrition
ABDULRAHMAN BASHIRE 2 CHILDREN HOSPITAL -- BENGHAZI
7. A 2 year old Pakistani boy has a haemoglobin of 8g/dl and an MCV of 65. The following tests are
essential:
A. Serum ferritin
B. Serum B12
C. Serum folate
D. Faecal occult blood
E. Haemoglobin electrophoresis
Ans:-ADE
Comments:
In progressive iron deficiency, a sequence of biochemical and haematologic events occurs. First, the tissue
iron stores represented by bone marrow haemosiderin disappear. The level of serum ferritin, an iron-storage
protein, provides a relatively accurate estimate of body iron stores in the absence of inflammatory disease.
Normal ranges are age dependent, and decreased levels accompany iron deficiency. Next, there is a decrease
in serum iron (also age dependent), the iron-binding capacity of the serum (serum transferrin) increases, and
the percent saturation (transferrin saturation) falls below normal (also varies with age). When the availability
of iron becomes rate limiting for haemoglobin synthesis, a moderate accumulation of heme precursors, free
erythrocyte protoporphyrins (FEP), results. As the deficiency progresses, the red blood cells (RBCs) become
smaller than normal and their haemoglobin content decreases. The morphologic characteristics of RBCs are
best quantified by the determination of mean corpuscular haemoglobin (MCH) and mean corpuscular
volume (MCV). Developmental changes in MCV require the use of age-related standards for diagnosis of
microcytosis . With increasing deficiency the RBCs become deformed and misshapen and present
characteristic microcytosis, hypochromia, poikilocytosis and increased red cell distribution width (RDW).
Iron deficiency is much commoner in more economically deprived communities, with up to 50% being
affected in some inner city areas.Detailed investigation is therefore unnecessary in this child, unless he fails
to respond to a trial of iron therapy.
11. The following are predisposing factors for iron deficiency anaemia:
A. Drinking unmodified cow's milk
B. Prematurity
C. Infant of diabetic mother
D. Intake of bottle milk
E. Excessive tea drinking
Ans:-ABE
Comments:
The predisposing factors/causes include:
Inadequate dietary intake of iron.
Drinking unmodified cow's milk (doorstep milk).
Prematurity and low birth weight.
Food stuffs and beverages which reduce iron availability including tea.
bone marrow is hypercellular with erythroid hyperplasia. Following iron therapy there is replacement of
intracellular iron enzymes and a subjective improvement within 24 hours. Within 48 hours there is a bone
marrow response, with reticulocytosis evident from 2 days, and peaking at about 7 days. The haemoglobin
level begins to increase from day 4 to day 30, and 3 months are required for complete repletion of iron
stores.
• In most cases of suspected iron deficiency a low Hb plus microcytosis with response to iron therapy
obviates confirms the diagnosis, and there is no need to measure ferritin.
19. The following are valid association in children with hypochromic anaemia:-
A. Normal serum ferritin &an elevated HbA2 point to beta thalassemia minor
B. Low ferritin & normal HbA2 exclude beta thalassemia minor.
C. Low ferritin point to iron deficiency.
D. Raised ferritin & normal hemoglobin electrophoresis point to anaemia of chronic
inflammatory disorder.
E. Dimorphic blood film is consistent with recent blood transfusion.
Ans:-ACDE
20. Which of the following statement regarding iron in infancy is false :-
A. About 4% of iron in fortified cow milk formula is absorbed by the infants
B. About 10% of iron in unfortified cow milk formula is absorbed by the infants
C. About 15% of iron in breast milk is absorbed by the infants
D. Absorption of iron by the infant is generally greater than in adults
E. Cow's milk and human milk have approximately the same iron content
Ans:- C
21. Hypochromic microcytic anaemia is seen in:
A. Alpha thalassemia
B. Sickle cell anemia
C. Autoimmune hemolytic anemia
D. Folate deficiency
E. Imerslund syndrome
Ans:- A
Comment:-
Hypochromic microcytic anemia is usually associated with:
a) Fe deficiency
b) Sideroblastic anemia:
- Primary X-linked recessive
- Secondary alcohol
- Malignancy
ABDULRAHMAN BASHIRE 6 CHILDREN HOSPITAL -- BENGHAZI
- Inflammation
In sideroblastic anemia there is Fe, but accumulation in the RBC mitochondria results in stippling on the
peripheral smear and ring sideroblast formation in the bone marrow.
Treatment is with pyridoxine (vitamin B6).
c) Lead poisoning
d) Beta-thalassemia (major or intermedia)
e) Alpha-thalassemia
f) Anemia of chronic disease
g) Copper deficiency
Sickle cell disease does not produce a microcytic anemia unless associated with thalassemia as in HbSb0.
Ans:-CD
24. Anaemia of acute infection and chronic disease is associated with all the following except:-
A. Shortened red cell survival
B. Impaired iron utilization
C. Iron stores are usually reduced
D. Impaired erythropoietin and bone marrow response
E. Usually mild anaemia with normocytic or microcytic red cells in acute infection
Ans:- C
25. Regarding blood indices:
A. The haemoglobin concentration of a 3 month old boy is higher than a 13 year old boy.
B. The mean corpuscular haemoglobin is low in megaloblastic anaemias.
C. The mean corpuscular haemoglobin concentration is low in iron deficiency anaemias.
D. The reticulocyte count increases with each year of life.
E. Reticulocytes are similar in size to mature red blood cells.
Ans:- C
Comments:
The haemoglobin at birth ranges from 13.7 - 21.1g/dl, falling to 13.0 - 20g/dl at 2 weeks of age, and to
between 9.5 and 14.5g/dl at 3 months. From there it rises gradually, with normal ranges between 6 months
and 6 years being 10.5 - 14.0g/dl, and between 7 and 12 years being 11.0 - 16.0g/dl. Adult normal ranges for
females are12.0 - 16.0g/dl, and for males 14.0 - 18.0g/dl. The mean cell haemoglobin (MCH) is the
haemoglobin divided by the red cell count. In health, this is usually between 27 and 32pg. Any disorder
which reduces red cell size reduces the amount of haemoglobin in the cell, and lowers the MCH. Likewise,
disorders increasing cell size raise the value. Means corpuscular haemoglobin concentration (MCHC) equals
the haemoglobin divided by the haematocrit. The normal range is 30 - 35g/dl. The MCHC is thus not the
number of grams of haemoglobin in 1dl of blood, but in 1dl of pure red cells without plasma. A low MCHC
is usually due to iron deficiency. The reticulocyte count in cord blood is 5%, paralleling the change from Hb
F to Hb A. From 2 weeks of age it drops to 1%, and remains at this level until adulthood. Once menses start,
females have a slightly higher reticulocyte count of around 1.6%. Reticulocytes are slightly larger than
mature red cells because of the little remaining nuclear material.
Hereditary spherocytosis (HS) is a common cause of haemolysis and haemolytic anaemia, with a prevalence
of 1:5000 in Northern Europeans. Patients may be asymptomatic without anaemia and minimal haemolysis,
or have severe haemolysis. It is autosomal dominant, though it is occasionally transmitted as AR. 25% of
patients have no family history. The abnormality is in spectrin, a major component of the cytoskeleton. This
results in loss of membrane without a proportionate loss of volume, so the red cells end up as small spheres
rather than biconcave discs. There is an associated increase in cation permeability and transport, ATP
utilisation, and glycolytic metabolism. The cells have decreased deformability impairing splenic passage, so
the cells are prematurely destroyed. In addition to haemolysis, hypoplastic crises may be associated with
parvovirus infection. This may result in profoun anaemia with high output heart failure, hypoxia,
cardiovascular collapse and death. In HS the haemoglobin level is usually 6 g/dl, and the reticulocyte count
is elevated to 6-20%. The MCV is normal, but the MCHC is increased. Spherocytes have a smaller diameter
than normal cells. The diagnosis is confirmed by an osmotic fragility test. Splenectomy eliminates most of
the haemolysis associated with the disorder, but carries its own risks. In mild cases, folic acid 1mg/day is
administered to prevent secondary folate deficiency. For those with severe anaemia, and/or hypoplastic or
aplastic crises, splenectomy is recommended after the age of 5-6 years. Vaccines for pneumococcus,
meningococcus and haemophilus influenzae should be given prior to splenectomy, and prophylactic
penicillin continued for life.
41. All of the following statements regarding G6PD deficiency are true except :-
A. Symptoms usually develop in patients with G6PD deficiency 24-48 hr after ingesting a
substance with oxidative properties
B. Infection may result in hemolysis in patients with G6PD deficiency
C. A pregnant woman who ingests oxidative drugs may cause hemolytic anemia in a fetus with
G6PD deficiency
D. Enzyme activity in affected persons is 10% of normal or less
E. The usual dose of aspirin causes clinically relevant hemolysis in the A variety of G6PD
deficiency
Ans:- E
The usual doses of aspirin and trimethoprim sulfamethoxazole do not cause clinically relevant
hemolysis in the A- variety. However, aspirin administered in doses used for acute rheumatic fever (60–
100 mg/kg/24?hr) may produce a severe hemolytic episode. When hemolysis has occurred, supportive
therapy may require blood transfusions, although recovery is the rule when the oxidant agent is
removed.
42. All of the following statements about Glucose-6-phosphate dehydrogenase deficiency are false
except:
A. Is more common in women than in men.
B. Is caused by mutations of the G6PD gene on chromosome 4.
C. Often leads to chronic haemolysis.
D. Requires treatment with desferrioxamine to prevent iron overload.
E. Is associated with haemolytic crises induced by sulfonamides.
Ans:- E
G6PDH catalyses the synthesis of NADPH from the hexose monophosphate pathway. The NADPH is then
used to reduce glutathione and hence in conjunction with glutathione reductase reduces cellular oxidative
damage.
G6PDH enzyme deficiency, caused by mutations in the X-linked (Xq28) G6PD gene, affects over half a
billion people worldwide. It is a balanced polymorphism associated with resistance to falciparum malaria in
heterozygous females. This evolutionary advantage outweighs the small negative effect of affected
hemizygous males. At least 400 G6PD variants have been identified. The various mutations lead to altered
expression of G6PD or an altered half-life of the enzyme.
There are a number of disease manifestations:
- patients may be asymptomatic
- patients may present with neonatal jaundice
- usually the deficiency manifests as acute haemolytic crises provoked by oxidising stress e.g.
certain foods - fava bean, chemicals - naphthalene, certain drugs - dapsone, primaquine, chloroquine, aspirin
ABDULRAHMAN BASHIRE 11 CHILDREN HOSPITAL -- BENGHAZI
44. The following drugs should be avoided in all patients with glucose-6-phosphate dehydrogenase
deficiency:
A. Quinine
B. Nitrofurantoin
C. Sulphapyridine
D. Chloramphenicol
E. Chloral hydrate
Ans:- ABCD
Comments:
In glucose-6-phosphate-dehydrogenase deficiency (G-6-PD) subjects are susceptible to developing acute
haemolytic anaemia on taking a number of common drugs. Ingestion of fava beans may result in haemolysis
in severe cases, or when they are eaten raw. G-6-PD is genetically heterogeneous. The risk from drugs
therefore varies from patient to patient. There is no test available to identify potential risk in G-6-PD
deficiency. The risk of severity of haemolysis is almost always dose related. Drugs with a definite risk in
most G-6-PD deficient subjects include:
• Sulphonamides and Dapsone.
• Methylene blue
• Nitrofurantoin
• Primiquine
• Quinilones (including Ciprafloxacin, Nalidixic acid)
45. The following are associated with a definite risk of hemolysis in most glucose-6 phosphate
dehydrogenase deficient subjects:
A. Primaquine
B. Methyllene blue
C. Nalidixic acid
D. Co-trimaxazole
E. Nitrofurantoin
Ans:- ABCDE
ABDULRAHMAN BASHIRE 12 CHILDREN HOSPITAL -- BENGHAZI
glucose-6 phosphate dehydrogenase reduces NADP to NADPH. NADPH is used by glutathione reductase
to reduce (oxidised) glutathion (toGSH). This replenishes the supply of reduced-SH groups in the
erythrocyte membrane protecting it from oxidant stress.
Deficiency of G6PD renders the red cell membrane susceptible to oxidant stress and haemolysis may result
following exposure to an oxidising agent of the environment (drugs, fava beans, infections, DKA).
G6PD also reduces NAD to NADH which is used by methaemoglobin reductase. Therefore G6PD
deficiency may also result in increased oxidation of Hb (metHb) with the apperarance of Heinz bodies on
the blood film. Drugs that cause hemolysis in G6PD deficiency are oxidizing agent. The BNF gives two lists
of drugs as follows:
(1)- Definite risk of hemolysis in most: 2)-Possible risk in some:
*dapson. *aspirin (high dose).
*methylene blue. *chloroquine.
*nitrofurantoin. *menadione (vit K).
*Primaquine. * probencid.
*quinolones *quinidine,.
*sulphonamides. *quinine
50. Features which may be seen on the blood film of a patient with haemolysis include:
A. Polychromasia
B. Howell Jolly bodies
C. Elliptocytes
D. Target cells
E. Heinz bodies
Ans:-ABDE
Comments:
The following are common abnormalities of red cell morphology:
• Polychromasia: younger cells have a bluish tinge (basophilia), and are larger than average, and may
contain residual nuclear material (reticulocytes). The presence of many basophilic forms in a blood film
produces a multicoloured effect known as polychromasia.
• Microcytosis indicates small red cells, anisocytosis variation in size, and macrocytosis a large size.
Poikilocytosis indicates altered shape.
• Spherocytes may indicate hereditary spherocytosis, and may also be found in acquired haemolytic
anaemias, while elliptocytes are found in hereditary elliptocytosis.
• Schistocytes are fragments of red cells, as seen in DIC or haemolytic uraemic syndrome.
• In splenic dysfunction: target cells and red cells containing nuclear fragments known as Howell Jolly
bodies are seen. Pappenheimer bodies are iron containing inclusions, and when seen together with Howell
Jolly bodies suggest previous splenectomy or reduced splenic function.
• Heinz bodies: are denatured haemoglobin which result from deficiencies of enzymes of the penthose
phosphate pathway. This damages the red cell leading to haemolysis and removal of them by the spleen.
Patients who have received blood transfusions may have hepatomegaly or chronic hepatitis due to iron
overload; transfusion-associated viral hepatitis resulting in cirrhosis or portal hypertension also may be
seen. The gall bladder may contain bilirubin stones formed as a result of the patient's life-long hemolytic
state. Splenomegaly typically is observed as part of the extramedullary hematopoiesis or as a
hypertrophic response related to the extravascular hemolysis.
o Extremities may demonstrate skin ulceration.
o Iron overload also may cause endocrine dysfunction, especially affecting the pancreas,testes& thyroid.
54. In thalassemia:-
A. The diagnosis is made on blood film.
B. The anaemia is due to deficiency in synthesis of globin chain.
C. The anaemia is mainly due to hemolysis.
D. Splenectomy is the treatment of choice at diagnosis.
E. The serum iron level is normal at diagnosis.
Ans;- BE
ABDULRAHMAN BASHIRE 15 CHILDREN HOSPITAL -- BENGHAZI
Ans:- DE
Comments:
a+b-expected in sickle cell anaemia, c-x-ray usually shows fishmouth vertebrae (infarcts), d-growth +
development usually impaired, e-almost all are unable to produce concentrated urine
64. A healthy, 39-week gestation male weighing 3.5 kg is born to a mother who has chronic anemia.
The infant’s physical examination findings are normal, and his hematocrit is 49% (0.49). The
mother asks you about the need for vitamins and iron for her newborn son.
Of the following, the BEST response is that term babies need to begin iron therapy at
A. birth
B. 2 weeks of age
C. 4 months of age
D. 9 months of age
E. no time
Preferred Response: C
Term newborns have accrued sufficient iron stores in the latter part of gestation to sustain them for 3 to 4
months after birth; this is true even when the mother has anemia. Although human milk contains lower
quantities of iron, its bioavailability is greater and, therefore, breastfed infants do not require replacement
therapy until 4 months of age. Preterm infants miss out on iron accretion in utero during the last trimester of
pregnancy and may require iron supplementation if they are taking full-volume enteral feedings as early as 2
to 4 weeks of age.
Iron supplementation is not required at birth except in the rare circumstance of congenital anemia. Iron
supplementation is required for normal hematopoiesis and brain growth and function, and if not provided by
6 months of age, characteristically leads to iron deficiency anemia.
65. You are called to the delivery room to evaluate an infant who has been delivered by spontaneous
vaginal delivery. The term infant weighs 3.6 kg and has some grunting respirations. You decide to
observe her in the newborn intensive care unit. One hour later, you are notified that the infant is
experiencing pronounced respiratory distress and oxygen saturations in the 70% range in the
right hand and in the 50% range in the right foot. There is poor perfusion. There are no murmurs,
but there is a pronounced precordial lift and a loud second heart sound.
Of the following, the MOST likely diagnosis for this infant is
A. congenital diaphragmatic hernia
B. congenital toxoplasmosis
C. hypoplastic left heart syndrome
D. persistence of the fetal circulation
E. tetralogy of Fallot
Preferred Response: D
Determining the cause of cyanosis and hypoxemia in the neonate is critically important, but it can be
difficult, especially during the first few minutes of the presentation. Diagnostic considerations in these
potentially critically ill infants may include persistence of the fetal circulation, with or without meconium
aspiration, and cyanotic congenital heart disease. The neonate described in the vignette clearly is
experiencing a disturbance in the process of oxygen delivery, and several clues suggest persistence of the
fetal circulation. Detecting this condition requires a clear understanding of the ductus arteriosus and its
function in the transitional circulation.
In the normal heart, the right atrium and right ventricle deliver desaturated blood to the organ of
oxygenation. In the fetus, this organ is the placenta, and its fetal blood supply is via the umbilical artery,
which arises from the fetal descending aorta. The ductus arteriosus provides a fetal shunting pathway,
allowing the right side of the heart to deliver desaturated blood to the placenta by shunting this blood away
from the high-resistance pulmonary arteries and into the descending aorta. This direction of flow is due in
part to the slightly higher fetal pulmonary vascular resistance compared with fetal systemic vascular
resistance because the lungs are filled with fluid and the placenta is a low-resistance circuit.
At birth, when the lungs expand with air and the placenta is removed from the circulation, pulmonary
vascular resistance falls and systemic vascular resistance increases. This leads to a reversal of flow across
the ductus arteriosus (from the system into the pulmonary circuit). Over
subsequent hours and days, the ductus arteriosus begins the process of spontaneous closure.
ABDULRAHMAN BASHIRE 18 CHILDREN HOSPITAL -- BENGHAZI
When the pulmonary vascular resistance exceeds the systemic vascular resistance, right-to-left shunting
across the ductus arteriosus persists as long as the ductus arteriosus is patent. Neonates who have severe
lung disease, including pneumonia, lung collapse, and pulmonary hypoplasia, may have pulmonary vascular
resistance that exceeds the systemic vascular resistance. When this occurs in the presence of a patent ductus
arteriosus, there is right-to-left shunting of blood in a pattern that is similar to the fetal circulation. Hence,
the term “persistence of the fetal circulation,” or perhaps better stated, “persistent pulmonary hypertension
of the newborn.” This condition leads to highly desaturated blood entering the descending aorta, often
evidenced by lower oxygen saturation in the lower body than in the upper body. When this process results
from pulmonary pathology, there is likely intrapulmonary ventilation-perfusion (V-Q) mismatch as well,
yielding the return of relatively desaturated blood to the left heart, which then delivers inadequately
oxygenated blood to the aorta.
Signs of an underlying pulmonary process may include grunting to maintain alveoli and small airway
patency, tachypnea, and ultimately respiratory distress and failure, as described for the infant in the vignette.
The oxygenation saturation pattern of the infant supports the diagnosis of persistence of the fetal circulation.
She has no murmur because she has no turbulent blood flow in her heart, and her precordial lift results from
the increased volume, pressure, and work load imposed on the right ventricle. Poor perfusion is caused by
failure (diminished ventricular function) of the right ventricle and its impact on the left ventricle.
Neonates who have diaphragmatic hernia often present with respiratory distress and a shift of the
precordium to the right chest because the gastrointestinal contents typically occupy the left thorax.
Infants who have toxoplasmosis may have signs and symptoms of systemic infection, but they are
unlikely to present solely with pulmonary pathology.
The hypoplastic left heart syndrome relies on right-to-left shunting at the ductus arteriosus to supply
systemic blood flow, but affected infants have normal lungs and low pulmonary vascular resistance, with
oxygen saturation typically in the 80% to 90% range.
Tetralogy of Fallot usually presents with the murmur of pulmonary stenosis, and when the ductus
arteriosus is present, blood flow is left to right because pulmonary vascular resistance and the lungs are
normal
haemoglobin switching occurs and the amount of HbF (fetal haemoglobin) decreases and the amount of HbS
(sickle haemoglobin) increases. This is why the sickle test, which detects the presence of sickle
haemoglobin, is often not positive until 6-9 months of age and cannot be guaranteed until one year of age.
Clinical problems include strokes and these are common, occuring in 25% of patients with homozygous
sickle disease by the age of 45 years. Patients with severe clinical manifestations of sickle disease have been
treated with allogeneic bone marrow transplantation from an HLA-identical sibling. Autologous bone
marrow transplantation is where the patients own bone marrow is replaced and would not be of benefit in
this condition.
It is not possible for the baby to have sickle cell disease (HbSS).
74. Which of the following statement is false regarding the pathophysiology of sickle cell disease:-
A. Infection is associated with increased anemia by suppression of RBC production
B. Conversion of RBC from normal biconcave discs to sickle form require the deoxygenation of
haemoglobin
C. Hypoxiemia and acidemia promote sickling by decreasing oxygen saturation of Hb
D. Dehydration doesn't promote sickling
E. Sickle cell increase whole blood viscosity producing local ischemia
Ans:-D
75. Which one of the following clinical manifestation isn't common to sickle cell disease:-
A. Vaso-occlusive crisis is more common than aplastic crisis
B. Hyperhemolytic crisis is often associated with red cell G6PD deficiency
C. Aplastic crisis is often associated with viral or bacterial infection
D. Salmonella sepsis is commonly associated with salmonella osteomyelitis
E. Hand-Foot syndrome may be initial manifestation of sickle cell disease
Ans:-D
76. The following are seen in sickle-cell anaemia
A. dactylitis
B. retardation of secondary sexual characteristics.
C. pathognomic fundal changes
D. cardiac signs simulating mitral stenosis
E. normal urinary concentrating ability only if the sickle-cell trait is present
ABDULRAHMAN BASHIRE 21 CHILDREN HOSPITAL -- BENGHAZI
Ans:-ABCD
Comments:
Dactylitis - inflammation of the fingers is a feature of SCD due to expansion of bone marrow. B -
aconsequnce of ill health with hypogonadotrophic hypogonadism. A so called black sunburst retinopathy is
pathognomonic of sickle cell disease. Retinal vein thrmbosis is also seen. Poor urinary concentration is a
feature of the disease but is unaffected with the trait.
78. A 10 year old West African boy presents with Hb 8g/dl and pains in his legs. Sickle cell disease is
unlikely if:
A. He is jaundiced.
B. He has gross splenomegaly.
C. Puberty began at 12 years.
D. His urine osmolality is 800mosmol/L, specific gravity 1022.
E. There is a mid systolic murmur.
Ans:-BCD
Comments:
Sickle cell anaemia is characterised by severe chronic haemolytic disease resulting from premature
destruction of brittle, poorly deformable erythrocytes. Other manifestations are due to ischaemia resulting
from vascular occlusion by masses of sickle cells. The clinical course is typically associated with crises.
The manifestations vary considerably with age.
Newborns: haemolytic anaemia from 2-4 months as fetal haemoglobin is replaced by Hb S, acute sickle
dactylitis (hand-foot syndrome).
Pre-school: acute painful vaso-occlusion crises, affecting extremities.
School children: painful crises affecting head, chest, abdomen, back, the site being typical for an any
individual patient. Episodes may be precipitated by intercurrent illness.
Late changes:
o infarction of bone marrow or bone.
o splenic infarction between 6 and 60 months contributing to autosplenectomy.
o pulmonary infarcts (acute chest syndrome).
o stroke caused by cerebrovascular occlusion -/+ hemiplegia.
o ischaemic damage to myocardium, liver and kidneys, with progressive impairment of renal
function and concentrating ability.
Spleen changes: in young children the spleen is enlarged, with occasional acute splenic sequestration.
Altered splenic function increases the risk of serious infection particularly meningitis sepsis caused by
pneumococci and haemophilus influenzae (polysaccharide encapsulated organisms). As the child ages, auto
splenectomy reduces spleen size. Cardiomegaly is invariably present in older children (sickle-related
ABDULRAHMAN BASHIRE 22 CHILDREN HOSPITAL -- BENGHAZI
cardiomyopathy), secondary haemosiderosis from increased iron absorption may damage liver, pancreas and
heart, and there may be gall stone formation. Puberty is frequently delayed, and chronic leg ulcers occur in
late adolescence.
79. Sickle cell trait is usually characterized by all of the following except:-
A. Red cell concentration of HbS is about 50%
B. Splenic infarction rarely occur except under extreme hypoxic condition
C. Reticulocyte count is usually elevated
D. In some individual hyposthnuria & hematuria may result from sickling in the renal medullary
capillaries
E. The incidence in American black is 1 in 12
Ans:- C
80. In paroxysmal nocturnal haemoglobinuria:-
A. The defect is a congenital abnormality of RBC production by the bone marrow.
B. Hamoglobinuria is a characteristic finding.
C. Venous thrombosis is a recognised complication.
D. The diagnostic test is Ham’s test.
E. Transforms into acute leukaemia in approx. 5%.
Ans:-BCDE
PNH is an acquired clonal defect of cell membranes that makes the cells more sensitive to lysis by
complement. This results in intravascular haemolysis and haemoglobinuria. Crises of haemolysis can be
triggered by infection, and chronic haemolysis may result in nephropathy. There is an increased risk of
venous thromboses (esp. in unusual sites such as saggital sinus or hepatic veins).The diagnostic test is
Ham’s test (acidified serum lysis). The disease may transform into acute leukaemia (in approx 5%).
A. Is defined as pancytopenia in the peripheral blood and blast cells in the bone marrow.
B. Has been associated with chloamphenicol eye drops.
C. Is preduced by hepatitis in 5-10% of cases
D. Is a feature of paroxysmal nocturnal haemoglobinuria
E. Can effectively be treated by growth factor injections
Ans:-CD
Aplastic anaemia is defined as the presence of pancytopenia in the peripheral blood and a hypocellular
marrow in which normal marrow is replaced by fat cells, abnormal cells are not found in either the
peripheral blood or bone marrow.
Causes include: drugs, viral infections and paroxysmal nocturnal hemoglobinemia.
The latter condition is an acquired defect of the red cell membrane which is associated with intravascular
hemolysis as the red cells are highly susceptible to complement lysis.
Many drugs have been implicated in aplastic anaemia but the evidence for many is slim.
Oral chloramphenicol not eye drops have been associated with aplastic anaemia, hepatitis, presumably of
viral origin is a precursor of aplastic anaemia in 5-10% of cases.
In the majority of cases no specific hepatitis virus is identified.
Definitive treatment is with immunosuppression or with allogenic stem cell transplantation in young patients
with HLA-matched doners.
Growth factors only have a role acutely to treat sever infection and are not effective as long term
treatment
84. These inborn errors have successfully been treated by bone marrow transplantation
A. Cystic fibrosis
B. Hurler's syndrome.
C. Gaucher's syndrome.
D. Fanconi's anaemia.
E. Chediak-Higashi syndrome.
Ans:-BCDE
85. Regarding leukocytes:-
A. Eosinophils play role in the defense against parasites
B. Mast cell are derived from basophils
C. Once migrated into the tissue, neutrophil is called a phagocyte
D. Lymphocytes contain myeloperoxidase
E. Band cells are immature neutrophils
Ans:-ABE
86. 5 year old child is found to have neutrophil count of 51´109 /L this may be due to :
A. Acute liver failure.
B. Corticosteroid therapy.
C. Brucellosis.
D. Aplastic anaemia.
E. Hypersplenism.
Ans:-ABC
4
Granulocytes survive for only 6–12 hr in the circulation and, therefore, daily production of 2 × 10
granulocytes/μL of blood is required to maintain a level of circulating granulocytes of 5 × 103/μL. In
contrast, lymphocytes can exhibit lifetimes measured in months or years and require daily renewal of
lymphocyte progenitors at rates substantially lower than the other hematopoietic progenitors. The mean
WBC count at birth is high, followed by a rapid fall beginning at 12 hr until the end of the 1st week.
Thereafter, values are stable until 1 yr of age. A slow, steady decline in the WBC count continues
throughout childhood until reaching the adult value during adolescence. Leukopenia in adolescents and
adults is defined as a total WBC count <4,000/μL.
87. Neutropenia :-
A. Is defined as circulatory count < 5´109/L.
ABDULRAHMAN BASHIRE 24 CHILDREN HOSPITAL -- BENGHAZI
Blood eosinophil numbers do not always reflect the extent of eosinophil involvement in disease-affected
tissues. The absolute eosinophil count, calculated as the white blood cell (WBC) count/μL × percent of
eosinophils, is usually <450 cells/μL in the blood and varies diurnally, being more abundant in the early
morning and diminishing as endogenous glucocorticoid levels rise.
Eosinopenia occurs after corticosteroid administration and with some bacterial and viral infections , The
absolute eosinophilia count is used to quantitate eosinophilia. Many diseases are associated with moderately
severe (1,500–5,000 cells/μl) or severe (>5,000 cells/μl) eosinophilia, Patients with sustained blood
eosinophilia may develop organ damage, especially cardiac damage as found in the idiopathic
hypereosinophilic syndrome, and should be monitored for evidence of cardiac disease. Many cases of
moderately severe eosinophilia often have no clear etiology.
92. The following conditions present with purpura & normal platelet counts:-
A. Osler-Weber-Rendu syndrome
B. Ehler –Danlos syndrome
C. Bernard—Soulier syndrome.
D. Wiskott-Aldrich syndrome.
E. Scurvy.
Ans:-ABE
93. The following are causes of thrombocytopenia:
A. B12 deficiency
B. Chronic myeloid leukaemia.
C. Splenectomy.
D. Bernard soulier syndrome
E. May-Hegglin anomaly.
Ans :-ABDE
B12 deficiency may be associated with a pancytopenia or with an isolated anaemia, thrombocytopenia or
leucopenia. Chronic myeloid leukaemia may be associated with a low, normal or high platelet count.
Bernard soulier syndrome and May Hegglin anomaly are congenital problems of platelet production and are
both associated with a thrombocytopenia. Splenectomy is associated with a thrombocytosis as well as
increased target cells and Howell Jolly bodies in the peripheral blood.
Thrombocytopenia
II. Signs
A. Bleeding disorders
B. Purpura or Petechiae
III. Causes: Congenital
A. Glanzmann's Thrombasthenia (autosomal recessive)
1. Platelet membrane deficiency Glycoprotein IIb, IIIa
2. Defective binding of platelet Fibrinogen
3. Decreased platelet aggregation
B. Bernard-Soulier Disease (autosomal recessive)
1. Platelet membrane deficiency Glycoprotein Ib
2. Coagulation Factor X and Factor V deficiency
3. Large platelets and decreased platelet aggregation
C. Storage Pool Disease
1. Dense granule and/or alpha granule deficiency
2. Defective platelet release of ADP and Serotonin
IV. Causes: Acquired
A. Uremia
B. Chronic Liver Disease
ABDULRAHMAN BASHIRE 27 CHILDREN HOSPITAL -- BENGHAZI
C. Medications
1. Aspirin
2. Furosemide (Lasix)
3. Nitrofurantoin (Furadantin)
4. Heparin
5. Sympathetic blockers
6. Clofibrate (Atromid-S)
7. NSAIDs
97. The following statement concerning hereditary bleeding disorder are correct :-
A. HCV is common cause of liver disease in adults with sever hemophilia treated before the
1990.
B. Antenatal diagnosis of hemophilia A is possible.
C. Von-Willebrand disease is inherited as an autosomal recessive manner in the majority of
patient.
D. Children with hemophilia have normal prothrombin time.
E. In hemophilia A , spontaneous bleeding into the joint occur when the factor VIII
concentration is reduced to 20% of normal.
Ans:-ABD
98. The following condition are caused by an abnormality of Hb synthesis :-
A. Beta thalassemia
B. Congenital spherocytosis
C. Pyruvate kinase deficiency
D. Hemophilia A
E. Sickle cell disease.
Ans:-AE
99. Von Willebrand disease (vWD):-
A. Is caused by pualitative or quantitative deficiency of von Willebrand factor
B. The resulting bleeding disorder in vWD is characterized by petechiae
C. The platelet count is normal
D. The ristocetin co-factor activity will be decreased
ABDULRAHMAN BASHIRE 28 CHILDREN HOSPITAL -- BENGHAZI
108. In ITP:-
A. The purpuric rash is characteristically on the buttock and the extensor surface of the limbs.
B. Bone marrow examination characteristically reveals diminished numbers of megakaryocytes.
C. The infant of an affected mother may develop transient thrombocytopenia.
D. Steroid therapy is ineffective
E. The prothrombin time is prolonged
Ans :-C
109. Concerning ITP in childhood
A. Bed rest is indicated
B. Bone marrow examination is essential
C. Incidence is 1 in 1000 children per year
D. Platelet transfusion is necessary if the platelet count fall below 20
E. I/V immunoglobulin may be beneficial
Ans:-E
Antiplatelet antibodies bind to transfused platelets as well as they do to autologous platelets. Thus, platelet
transfusion in ITP is usually contraindicated unless life-threatening bleeding is present. (safe level of >20 ×
109/L,)
reaction are bound to haemoglobin which encourages the release of oxygen, since reduced
haemoglobin is less acid than the oxygenated forms.
Thus, the presence of reduced haemoglobin in the peripheral blood helps with the loading of carbon dioxide,
while the oxygenation which occurs in the pulmonary capillary assists in the unloading of it. The fact that
the deoxygenation of the blood increases its ability to carry carbon dioxide is often known as the Haldane
effect. Fetal haemoglobin contains g polypeptide chains in place of the b-chains of Hb A. Its resistance to
denaturation by strong alkali is used in its quantitations. Hb F is the predominant haemoglobin from 8 weeks
gestation, and constitutes 90% of the total haemoglobin of the 6 month fetus. At birth 70% of the total is Hb
F, and synthesis decreases rapidly postnatally, such that by a year, only 2% is present. Hb F has a greater
oxygen affinity than Hb A, so the growing fetus is preferentially ourished by oxygen in utero.
119. Hemoglobin
A. At 40 week gestation is 70-75% HbF
B. At 40 week gestation is 20-25% HbA
C. After age 3 is over 98% HbA
D. After age 3 year is approximately 3% HbF
E. In beta thalassemia minor contain elevated HbF & HbA2
Ans :-ACE
Two hemoglobin gene clusters are involved in the production of hemoglobin and are located at the end of
the short arm of chromosomes 16 and 11, respectively. On chromosome 16, there are 3 genes within the
alpha (α) gene cluster: zeta (ζ) and 2 α genes (α1, α2). On chromosome 11, there are 5 genes within the beta
(β) gene cluster: epsilon (ε), delta (δ), beta (β), and 2 gamma (γ) genes. The order of gene expression within
each cluster roughly follows the order of expression during the embryonic period, the fetal period, and
eventually childhood.
8 wk of fetal life, the embryonic hemoglobin are formed: Gower-1 (ζ2ε2), Gower-2 (α2ε2), and Portland
(ζ2γ2). In embryos of 4–8 wk gestation, the Gower hemoglobins predominate, but by the 3rd month they
have disappeared.
At 9 wk of fetal life, the major hemoglobin (Hb) is Hb F (α2γ2). Its resistance to denaturation by strong
alkali is the basis for determining the presence of fetal RBCs in the maternal circulation (the Kleihauer-
Betke test). After the 8th gestational wk, Hb F is the predominant hemoglobin; at 24 wk gestation it
constitutes 90% of the total hemoglobin. During the 3rd trimester, a gradual decline occurs, so that at birth
Hb F averages 70% of the total. Synthesis of Hb F decreases rapidly postnatally, and by 6–12 mo of age
only a trace is present.
At approximately 1 mo of fetal life, Hb A (α2β2) appears, but does not become the dominant hemoglobin
until after birth, when Hb F levels start to decline. A minor hemoglobin, Hb A2(α2δ2) appears shortly before
birth and remains at a low level after birth. The final hemoglobin distribution pattern that occurs in
childhood is not achieved until at least 6 mo of age, and sometimes later.
The normal hemoglobin pattern is >95% Hb A, ≤3.5 Hb A2, and <2.5% Hb F.
Some Hb A (α2β2) can be detected in even the smallest embryos. Accordingly, it is possible as early as 16–
20 wk gestation to make a prenatal diagnosis of major β-chain hemoglobinopathies, such as thalassemia
major . Prenatal diagnosis is based on techniques that examine the rates of synthesis of β chains or the
structure of newly synthesized β chains. Earlier diagnosis is possible using molecular biology techniques
and sampling of chorionic villus tissue or amniotic fluid if DNA structural defects are a cause of the
hemoglobinopathies. Gene deletion disorders such as the α- thalassemias can be detected using the same
method.
By the 24th wk of gestation, 5–10% of Hb A is present. A steady increase follows, so that at term, Hb A
averages 30%. By 6–12 mo of age, the normal Hb A pattern appears. The minor Hb A component Hb
A2contains delta (δ) chains and has the structure α2δ2. It is seen only when significant amounts of Hb A are
also present. At birth, <1.0% of Hb A2 is seen, but by 12 mo of age the normal level of 2.0–3.4% is attained.
Throughout life, the normal ratio of Hb A to A2is about 30:1.
ABDULRAHMAN BASHIRE 33 CHILDREN HOSPITAL -- BENGHAZI
120. hemoglobin;
A. Is present in blood cells by the 14th day after gestation.
B. Is predominantly produced in the liver at term.
C. At term is mainly fetal hemoglobin.
D. Production increase after delivery.
E. Concentration at birth is 10-12 g/dl
Ans:- AC
Mesoblastic hematopoiesis occurs in extraembryonic structures, principally in the yolk sac, and begins
between the 10th and 14th days of gestation. By 6–8 wk of gestation the liver replaces the yolk sac as the
primary site of blood cell production, and by 10–12 wk extraembryonic hematopoiesis has essentially
ceased. Hepatic hematopoiesis occurs in the liver throughout the remainder of gestation, although
production begins to diminish during the second trimester as bone marrow (myeloid) hematopoiesis
increases. The liver remains the predominant hematopoietic organ through wk 20–24 of gestation
5- With regards RBC indices, it is the MCHC which affects the oxygen-dissociation curve, rather than
the MCH or MCV. In anaemia, the MCHC is reduced, and the curve is right shifted, increasing the
release of oxygen to the tissues.
Protein C is a vitamin K-dependent protein synthesised in the liver. It circulates in an inactive form, which
becomes activated by thrombin/thrombomodulin complexes on the surface of endothelial cells. Activated
protein C inhibits coagulation by degrading coagulation factors Va and VIIIa and resulting in decreased
thrombin formation. It requires protein S as a cofactor.
Causes of protein C deficiency are:
1) Congenital
* homozygous deficiency - those with severe deficiency - (protein C levels <1% of normal) present with
severe neonatal purpura fulminans, cerebral thrombosis and DIC. Those with milder defects have protein C
levels of 10 -24 % of normal. They can present with massive venous thrombosis as older children.
* heterozygous deficiency (protein C levels 30-40% of normal) most frequently presents after puberty with
deep venous thrombosis of the lower limb. It can also present with recurrent superficial thrombophlebitis
and DVT. Most thrombotic episodes occur spontaneously but known associated risk factors include surgery
with immobilisation, pregnancy and the oral contraceptive.
Many people remain asymptomatic. A positive family history of thrombosis is associated with an increased
risk of symptoms - + - 50% of individuals with heterozygous protein C deficiency and a family history of
thrombosis, will experience a thrombotic event.
2) Causes of acquired protein C deficiency:
* infections - meningococcal disease and varicella infection
* DIC
* hepatic disease
* drugs - warfarin, chemotherapeutic agents e.g. cyclophosphamide, methotrexate, 5-fluorouracil), l-
asparaginase
* malignancy - acute myeloid leukaemia
* chronic inflammation - inflammatory bowel disease
* haemolytic uraemic syndrome
* thrombotic thrombocytopaenic purpura
Patients with protein C deficiency are at high risk for warfarin-induced skin necrosis during initiation of
therapy with warfarin.
Factor V Leiden refers to a point mutation in the gene for factor V results in resistance of factor V to the
actions of activated protein C, and consequently a thrombophillic state. Protein C levels are not decreased
called a leukocytosis.A decreased number of white blood cells in the peripheral blood (choice b) is called a
leukopenia.
Hypersegmented granulocytes present in the peripheral blood (choice c) is a shift to the right.An increased
number of immature red blood cells in the peripheral blood (choice e) is a reticulocytosis.