anaemias

Question 1

True or false - you can give iron salts to patients with any type of anaemia

Answer 1

FALSE
Iron salts may be harmful if given to pt with anaemias other than those due to iron deficiency

Question 2

Iron salts should only be given to pt with anaemias due to ..

Answer 2

iron deficiency

Question 3

what is sickle cell disease caused by

Answer 3

structural abnormality of Hb resulting in deformed, less flexible RBC

Question 4

what is sickle cell crisis

Answer 4

infarcation of the microvasculature and restricted blood supply to organs results in severe pain

Question 5

usual management of sickle cell crisis

Answer 5

usually requires hospitalisation, fluid replacement, analgesia, and treatment of any concurrent infection.

Question 6

complications of sickle cell crisis

Answer 6

anaemia, leg ulcers, renal failure, and increased susceptibility to infection

Question 7

the following reduce the risk of infection in sickle cell disease/crisis

3x vaccine (+1), 1x penicllin

Answer 7

Pneumococcal vaccine, haemophilus influenzae type b vaccine, an annual influenza vaccine, and lifelong prophylactic penicillin reduce the risk of infection.

Hepatitis B vaccine should also be given if the patient is not immune.

Question 8

Why is supplementation with folic acid recommended in most forms of sickle cell disease?

Answer 8

varying degrees of haemolytic anaemia are present which is accompanied by increased erythropoiesis; this may increase folate requirements and supplementation with folic acid is recommended.

optimum dose should be discussed with a specialist.

Question 9

this cytotoxic drug can prevent acute chest syndrome, reduce the freq of painful crises, and reduce transfusion requirments in sickle cell disease

Answer 9

hydroxycarbamide

(beneficial effects may not be seen for several months)

Question 10

this -zumab is an option for preventing recurrent sickle cell crises (vaso-occlusive crises)

Answer 10

Crizanlizumab

Question 11

G6PD deficiency is more common in which sex?

Answer 11

males

Question 12

Which ethnicities is G6PD deficiency more common in (4)

Answer 12

• Africa, Asia, Mediterraenean, Middle East

Question 13

Individuals with G6PD deficiency are susceptible to developing the following anaemia when they take a number of common drugs or when they have an infection

Answer 13

acute haemolytic anaemia

Question 14

What is favism

Answer 14

People with G6PD deficiency are suscepible to developing acute haemolytic anaemia when they eat fava beans (broad beans)

Question 15

People with G6PD deficiency are suscepible to developing acute haemolytic anaemia when they eat the following beans

Answer 15

fava beans (broad beans) - this is termed as favism

Question 16

When prescribing drugs for patients with G6PD deficiency, the following three points should be kept in mind:

Answer 16

• G6PD deficiency is genetically heterogeneous; susceptibility to the haemolytic risk from drugs varies; thus, a drug found to be safe in some G6PD-deficient individuals may not be equally safe in others;
• manufacturers do not routinely test drugs for their effects in G6PD-deficient individuals;
• the risk and severity of haemolysis is almost always dose-related.

Question 17

T or F - the risk and severity of haemolysis in pt with G6PD deficiency is almost always dose-related.

Answer 17

true

Question 18

Drugs on UK market with definite risk of haemolysis in most G6PD-deficient individuals

Answer 18

• Dapsone and other sulfones
• Fluoroquinolones (including ciprofloxacin, moxifloxacin, norfloxacin, and ofloxacin)
• Methylthioninium chloride
• Nitrofurantoin
• Primaquine
• Quinolones
• Rasburicase
• Sulfonamides (including co-trimoxazole

Question 19

This antibiotic, commonly used for UTIs, has definite risk of haemolysis in most G6PD-deficient individuals

Answer 19

nitrofurantoin

Question 20

Very few G6PD-deficient individuals with chronic non-spherocytic haemolytic anaemia have haemolysis even in the absence of an exogenous trigger. What would happen if they were to be given drugs that have risk of haemolysis?

Answer 20

In these patients, exacerbation of haemolysis following oxidative stress, such as the administration of any of the drugs that have risk of haemolysis in pt with G6PD deficient pt, will occur.

Question 21

Drugs with possible risk of haemolysis in some G6PD-deficient individuals

Answer 21

Aspirin
Chloroquine
Menadione, water-soluble derivatives (e.g. menadiol sodium phosphate)
Quinine (may be acceptable in acute malaria)
Sulfonylureas

Question 22

This class of oral antidiabetic drugs has a possible risk of haemolysis in some G6PD deficient pt

Answer 22

SUs

Question 23

What can be used for immunosuppressive treatment for aplastic anaemia

Answer 23

Intravenous horse antithymocyte globulin in combination with ciclosporin

Question 24

What is Intravenous horse antithymocyte globulin in combination with ciclosporin used for

Answer 24

may be used as immunosuppressive treatment for aplastic anaemia.

Question 25

what is the response rate for IV horse antithymocyte globulin in combination with ciclosporin, vs ciclosporin alone for non severe aplastic anaemia

Answer 25

The response rate for non-severe aplastic anaemia is higher with this combination than with ciclosporin alone.

Question 26

This steroid is used for the prevention of adverse effects associated with antithymocyte globulin treatment (which is used for aplastic anaemia - IV horse antithymocyte globulin in combination with ciclosporin)

Answer 26

Prednisolone

Question 27

Prednisolone is used for the prevention of adverse effects associated with antithymocyte globulin treatment. Early reactions that may occur include …

Answer 27

fever, rash, fluid retention, rigors, acute respiratory distress syndrome, and anaphylaxis; serum sickness may occur 7–14 days later.

Question 28

Who can give antithymocyte globulin treatment and in which facilities

Answer 28

Antithymocyte globulin should be given under specialist supervision with appropriate resuscitation facilities

Question 29

Intravenous horse antithymocyte globulin in combination with ciclosporin, may be used as immunosuppressive treatment for aplastic anaemia.

What are some other treatment options (2)

Answer 29

ciclosporin alone or oxymetholone.

Question 30

This vitamin B is licensed for the treatment of idiopathic sideroblastic anaemia; the dose required is usually high.

Answer 30

pyridoxine HCl

Question 31

What is used to treat anaemia associated with erythropoietin deficiency in chronic renal failure and why?

Answer 31

Epoetins (recombinant human erythropoietins)
To increase the yield of autologous blood in normal individuals and to shorten the period of symptomatic anaemia in patients receiving cytotoxic chemotherapy.