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.

prophylactic abx: lifelong phenoxy, alt eryth

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

“Dangerous Fumes Make New Patients Quite Rapidly Suffer”

Question 19

“Dangerous Fumes Make New Patients Quite Rapidly Suffer”

Answer 19

Dapsone and other sulfones
Fluoroquinolones
Methylthionium chloride
Nitrofurantoin
Primaquine
Quinolones
Rabiscurase
Sulfonamides (including co trimox)

these drugs have definite risk of causing haemolysis in most G6PD deficient people

Question 20

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

Answer 20

nitrofurantoin

Question 21

Which common abx gave definite risk of causing haemolysis in most G6PD deficient people

Answer 21

nitro
fluoroquiolones
sulfonamides

Question 22

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 22

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 23

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

Answer 23

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

Naphthalene in mothballs also causes haemolysis in individuals with G6PD deficiency.

“A Curious Man Quietly Sipped Nectar”

Question 24

“A Curious Man Quietly Sipped Nectar”

Answer 24

Aspirin
Chloroquine
Menadione
Quinine
SUs
Naphthalene in mothballs

these have possible risk of causing G6PD deficiency in some people

Question 25

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

Answer 25

SUs

Question 26

what is aplastic anaemia

Answer 26

Aplastic anemia is a rare but serious blood condition that occurs when your bone marrow cannot make enough new blood cells for your body to work normally

Question 27

What can be used for immunosuppressive treatment for aplastic anaemia

Answer 27

Intravenous horse antithymocyte globulin in combination with ciclosporin

Question 28

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

Answer 28

may be used as immunosuppressive treatment for aplastic anaemia.

Question 29

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

Answer 29

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

Question 30

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 30

Prednisolone

Question 31

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

Answer 31

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

Question 32

Who can give antithymocyte globulin treatment and in which facilities

Answer 32

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

Question 33

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 33

ciclosporin alone or oxymetholone.

Question 34

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

Answer 34

pyridoxine HCl

Question 35

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

Answer 35

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

Chemotherapy can cause anemia, making people feel weak and tired. The treatment helps these patients recover their red blood cells more quickly, so they don’t feel the symptoms of anemia for as long.

Essentially, this treatment boosts blood production to help healthy people who need to store their own blood and to support chemotherapy patients who need to recover from anemia faster.

Question 36

which epoetins are long acting

Answer 36

long-acting agents darbepoetin alfa and methoxy polyethylene glycol-epoetin beta

Question 37

epoetins 2 CI

Answer 37

Pure red cell aplasia following erythropoietin therapy; uncontrolled hypertension

Question 38

common SE for epoetins

Answer 38

Arthralgia; embolism and thrombosis; headache; hypertension (dose-dependent); influenza like illness; skin reactions; stroke

Question 39

important info re epoetins and hypertensive crises

Answer 39

CI in uncontrolled HTN
HTN is a common SE, dose related
In isolated patients with normal or low blood pressure, hypertensive crisis with encephalopathy-like symptoms and generalised tonic-clonic seizures requiring immediate medical attention has occurred with epoetin.

Question 40

Pt on epoetin develops pure red cell aplasia. What should you do

Answer 40

treatment with erythropoietins must be discontinued and testing for erythropoietin antibodies considered. Patients who develop pure red cell aplasia should not be switched to another form of erythropoietin.

Question 41

Patient on epoetin develops HTN. what should you do

Answer 41

interrupt treatment

Question 42

MHRA/CHM advice: Recombinant human erythropoietins: very rare risk of severe cutaneous adverse reactions

Answer 42

Very rare cases of severe cutaneous adverse reactions, including SJS and toxic epidermal necrolysis, in patients treated with erythropoietins; some cases were fatal.

More severe cases were recorded with long-acting agents (darbepoetin alfa and methoxy polyethylene glycol-epoetin beta).

seek immediate medical attention if they develop widespread rash and blistering; these rashes often follow fever or flu-like symptoms—discontinue treatment permanently if such reactions occur.

Question 43

MHRA Erythropoietins—haemoglobin concentration

Answer 43

Overcorrection of haemoglobin concentration in patients with chronic kidney disease may increase the risk of death and serious cardiovascular events, and in patients with cancer may increase the risk of thrombosis and related complications

Question 44

…. toxicity (can impair the response to erythropoietin)

Answer 44

aluminium