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Question 1

Which supplement is advised to take to a patient with sickle cell disease?

Answer 1

Folate (folic acid)

Sickle cell disease is a genetic condition where red blood cells become rigid and shaped like sickles, or crescent moons, instead of round. This abnormal shape can cause blockages in blood vessels, leading to pain, organ damage, and other health problems.

Folic acid is advised for patients with sickle cell disease because they often have an increased rate of red blood cell turnover, leading to a higher need for folic acid. Folic acid helps in the production of red blood cells, which can be depleted due to the condition.

In sickle cell disease, red blood cells are fragile and can break down more easily than normal. This increased fragility leads to a higher rate of red blood cell turnover, where the body needs to produce new red blood cells more frequently to replace the ones that are lost or damaged.

Question 2

What drug can reduce the frequency of sickle cell crises

Answer 2

Hydroxycarbamide

Question 3

What are the individuals with G6PD deficiency susceptible to developing what?

Answer 3

Acute haemolytic anaemia

“G6PD,” which stands for glucose-6-phosphate dehydrogenase. It’s an enzyme that plays a crucial role in protecting red blood cells from oxidative stress. When deficient, it can lead to a condition called G6PD deficiency, which can cause acute hemolytic anemia under certain conditions.

Question 4

Which foods can cause acute haemolytic anaemia?

Answer 4

Fava beans, broad beans, fresh fava beans

Question 5

State the drugs with definite risk of haemolysis in most G6PD-deficient individuals?

Answer 5

These drugs can induce oxidative stress in red blood cells, leading to the destruction of red blood cells in individuals with G6PD deficiency

Antimalarial drugs like fluoroquinolones

Rasburicase

Sulfonamide antibiotics/ Sulfones such as sulfamethoxazole (part of Bactrim or
Septra)

Nitrofurantoin, an antibiotic commonly used for urinary tract infections

Dapsone, which is used to treat leprosy, dermatitis herpetiformis, and certain other conditions

Question 6

What is used to treat anemia associated with erythropoietin deficiency in chronic renal failure?

Answer 6

Epoetin’s

In chronic renal failure, the kidneys are unable to produce enough erythropoietin, which is a hormone responsible for stimulating the production of red blood cells in the bone marrow. This deficiency in erythropoietin leads to a condition called anemia of chronic kidney disease (CKD)

Question 7

Which erythropoietin is used to treat anemia in preterm neonates of low-birth weight?

Answer 7

Epoetin beta

Question 8

Which erythropoietin has a longer half-life and can be administered less frequently?

Answer 8

Darbepoetin

Question 9

State the MHRA warning for epoetin’s?

Answer 9

Steven-johns syndrome

toxic epidermal necrolysis

Steven-Johnson syndrome is a severe skin reaction that typically occurs as a reaction to medication or an infection. It causes painful blisters, skin peeling, and can affect mucous membranes, including those in the mouth and eyes. It’s considered a medical emergency and requires immediate attention.

Toxic epidermal necrolysis (TEN) is a severe skin condition where the top layer of the skin separates from the lower layers due to a reaction to medication or infection. It causes widespread blistering and skin shedding, often accompanied by fever and other systemic symptoms. It’s a medical emergency requiring urgent treatment.

Question 10

Advise patients of symptoms of severe skin reactions, stop treatment if:

Answer 10

They develop widespread rash / blistering

Question 11

What are the symptoms of iron deficiency anaemia?

Answer 11

Lethargy
shortness of breath
heart palpitations
pale skin

Question 12

State the conditions in which prophylaxis is required with iron?

Answer 12

Malabsorption
gastrectomy
menorrhagia
haemodialysis
pregnancy
premature infants

Question 13

What patient counselling advice do you give to patients taking iron?

Answer 13

Take with or after food, and take with glass of orange juice to aid absorption

Question 14

State the MHRA advice for intravenous iron?

Answer 14

Caution with every IV dose

Monitor for 30 mins after each injection

High risk in allergies

Avoid in pregnancy, especially first trimester

Question 15

Define megaloblastic anaemia:

Answer 15

Most result from a lack of either vitamin B12 or folate

Megaloblastic anemia is a type of anemia characterized by the presence of unusually large and immature red blood cells called megaloblasts in the bone marrow. This condition is typically caused by a deficiency in vitamin B12 or folic acid, which are essential for normal red blood cell production. Without adequate levels of these vitamins, red blood cells fail to mature properly,

Question 16

State one cause of megaloblastic anaemia:

Answer 16

Pernicious anemia in which lack of gastric intrinsic factor resulting from an autoimmune gastritis causes malabsorption of vitamin B12

This statement means that pernicious anemia is caused by an autoimmune condition where the body attacks the cells in the stomach that produce a substance called intrinsic factor. Intrinsic factor is needed to absorb vitamin B12 from food. Without enough intrinsic factor, the body can’t absorb vitamin B12 properly, leading to a deficiency and causing pernicious anemia.

Question 17

State treatment of B12 neuropathy:

Answer 17

Hydroxocobalamin or cyanocobalamin

Vitamin B12 plays a crucial role in maintaining the health of nerves and the myelin sheath, which is the protective covering of nerve fibers. When there’s a deficiency of vitamin B12, nerve cells can become damaged over time, leading to a condition called peripheral neuropathy.

This damage can disrupt the normal functioning of nerves,

Question 18

State treatment of folate deficiency:

Answer 18

Most causes are self-limiting or will yield a short course treatment with folic acid

Question 19

State treatment of folate-deficient megaloblastic anaemia:

Answer 19

Folic acid daily

Question 20

State prophylaxis in chronic haemolytic states, malabsorption, or in renal dialysis:

Answer 20

Folic acid is given daily or sometimes weekly

Chronic haemolytic states: Persistent conditions where red blood cells are continuously destroyed faster than they are produced in the body, leading to a long-term imbalance and resulting in ongoing anemia.

Question 21

State treatment for the prevention of methotrexate-induced side-effects in severe Crohn’s disease, rheumatic disease and severe psoriasis:

Answer 21

Folic acid

Question 22

State treatment of folate deficient megaloblastic anemia:

Answer 22

Folinic acid - but it is generally used in association with cytotoxic drugs; it is given as calcium folinate

Question 23

State which vaccinations and drug treatment a patient with sickle-cell disease is advised to take:

Answer 23

Pneumococcal vaccine

Haemophilus influenzae type b vaccine

Annual influenza vaccine

Hepatitis B if patient is not immune

Lifelong prophylactic penicillin can reduce risk of infection

Question 24

State which drug can prevent acute chest syndrome and reduce the frequency of painful crises and reduce transfusion requirement in sickle-cell disease:

Answer 24

Hydroxycarbamide

Question 25

State which drug is an option for preventing recurrent sickle cell crises (vaso-occlusive crises):

Answer 25

Crizanlizumab ## Footnote Vaso-occlusive crises in sickle cell disease happen when sickle-shaped red blood cells block small blood vessels, causing severe pain and tissue damage

Question 26

State treatment of aplastic anemia:

Answer 26

Intravenous horse antithymocyte globulin in combination with ciclosporin Ciclosporin alone Oxymetholone ## Footnote Aplastic anemia is a rare disorder where the bone marrow fails to produce enough blood cells, including red blood cells, white blood cells, and platelets. This deficiency can lead to fatigue, frequent infections, and excessive bleeding or bruising. Intravenous horse antithymocyte globulin (ATGAM) is a medication derived from horse serum used in the treatment of certain conditions. It works by suppressing the immune system, particularly T cells, which can be overactive and attack healthy tissues in conditions like aplastic anemia. By dampening this immune response, ATGAM helps to restore normal bone marrow function in aplastic anemia patients. However, because it is derived from horse serum, there is a risk of allergic reactions, so it is typically administered under close medical supervision.

Question 27

State treatment for idiopathic sideroblastic anaemia:

Answer 27

Pyridoxine hcl ## Footnote Idiopathic sideroblastic anemia is a rare blood disorder where the bone marrow produces abnormal red blood cells containing excess iron. This leads to problems with oxygen transport in the body. "Idiopathic" means the cause is unknown. Symptoms may include fatigue, weakness, and difficulty breathing.

Question 28

State what are epoetin's used for:

Answer 28

Used to treat anemia associated with erythropoietin deficiency in chronic renal failure, to increase the yield of autologous blood in normal individuals and to shorten the period of symptomatic anemia in patients receiving cytotoxic chemotherapy ## Footnote Autologous blood refers to blood that is donated by a person for their own use. Cytotoxic drugs, also known as chemotherapy drugs, are medications used to kill or inhibit the growth of rapidly dividing cells. While they primarily target cancer cells, they can also affect normal cells that divide rapidly, such as those in the bone marrow or hair follicles, leading to side effects like bone marrow suppression, hair loss, and gastrointestinal issues. Examples of cytotoxic drugs include methotrexate, cisplatin, and doxorubicin.

Question 29

State what epoetin is used for preventions of anaemia in preterm neonates of low birth-weight:

Answer 29

Epoetin beta Darbepoetin alfa (is a hyperglycosylated derivative of epoetin)

Question 30

State which drug is used for symptomatic anemia associated with chronic kidney disease:

Answer 30

Methoxy polyethylene glycol-epoetin beta

Question 31

State treatment of iron overload associated with haemochromatosis:

Answer 31

Repeated venesection Desferrioxamine mesylate ## Footnote Repeated venesection, also known as phlebotomy, refers to the process of drawing blood from a vein, typically for therapeutic purposes. In certain medical conditions, such as hemochromatosis or polycythemia vera, the body may produce too much iron or red blood cells. Repeated venesection is used as a treatment to reduce these excess levels.

Question 32

State the antidote for magnesium toxicity:

Answer 32

Calcium gluconate injection

Question 33

What can hypomagnesaemia often cause:

Answer 33

Secondary hypocalcaemia and also hypokalaemia

Question 34

Define neural tube defects:

Answer 34

Represents a group of congenital defects caused by incomplete closure of the neural tube within 28 days of conception The most common forms are anencephaly, spina bifida and encephalocele Pregnant women or who wish to become pregnant should be advised to take folic acid before conception and until week 12 of pregnancy ## Footnote This can cause serious health problems for the baby, including paralysis, learning difficulties, and even death

Question 35

A higher daily dose (5mg) is recommended for women at high risk:

Answer 35

Women who previously had an infant with neural tube defects Women receiving antiepileptic medication Women who have diabetes Women who have sickle-cell disease

Question 36

Define neutropoenia:

Answer 36

Characterised by low neutrophil count

Question 37

State the granulocyte-colony stimulating factors that can help increase the neutrophil count:

Answer 37

Filgastrim Lenograstim Pegfilgrastim Lipegfilgrastim ## Footnote Granulocyte-colony stimulating factors (G-CSF) are substances in the body that help stimulate the production of a type of white blood cell called granulocytes.