anaemias Flashcards
True or false - you can give iron salts to patients with any type of anaemia
FALSE
Iron salts may be harmful if given to pt with anaemias other than those due to iron deficiency
Iron salts should only be given to pt with anaemias due to ..
iron deficiency
what is sickle cell disease caused by
structural abnormality of Hb resulting in deformed, less flexible RBC
what is sickle cell crisis
infarcation of the microvasculature and restricted blood supply to organs results in severe pain
usual management of sickle cell crisis
usually requires hospitalisation, fluid replacement, analgesia, and treatment of any concurrent infection.
complications of sickle cell crisis
anaemia, leg ulcers, renal failure, and increased susceptibility to infection
the following reduce the risk of infection in sickle cell disease/crisis
3x vaccine (+1), 1x penicllin
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
Why is supplementation with folic acid recommended in most forms of sickle cell disease?
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.
this cytotoxic drug can prevent acute chest syndrome, reduce the freq of painful crises, and reduce transfusion requirments in sickle cell disease
hydroxycarbamide
(beneficial effects may not be seen for several months)
this -zumab is an option for preventing recurrent sickle cell crises (vaso-occlusive crises)
Crizanlizumab
G6PD deficiency is more common in which sex?
males
Which ethnicities is G6PD deficiency more common in (4)
- Africa, Asia, Mediterraenean, Middle East
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
acute haemolytic anaemia
What is favism
People with G6PD deficiency are suscepible to developing acute haemolytic anaemia when they eat fava beans (broad beans)
People with G6PD deficiency are suscepible to developing acute haemolytic anaemia when they eat the following beans
fava beans (broad beans) - this is termed as favism
When prescribing drugs for patients with G6PD deficiency, the following three points should be kept in mind:
- 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.
T or F - the risk and severity of haemolysis in pt with G6PD deficiency is almost always dose-related.
true
Drugs on UK market with definite risk of haemolysis in most G6PD-deficient individuals
- 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”
“Dangerous Fumes Make New Patients Quite Rapidly Suffer”
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
This antibiotic, commonly used for UTIs, has definite risk of haemolysis in most G6PD-deficient individuals
nitrofurantoin
Which common abx gave definite risk of causing haemolysis in most G6PD deficient people
nitro
fluoroquiolones
sulfonamides
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?
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.
Drugs with possible risk of haemolysis in some G6PD-deficient individuals
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”
“A Curious Man Quietly Sipped Nectar”
Aspirin
Chloroquine
Menadione
Quinine
SUs
Naphthalene in mothballs
these have possible risk of causing G6PD deficiency in some people
This class of oral antidiabetic drugs has a possible risk of haemolysis in some G6PD deficient pt
SUs
what is aplastic anaemia
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
What can be used for immunosuppressive treatment for aplastic anaemia
Intravenous horse antithymocyte globulin in combination with ciclosporin
What is Intravenous horse antithymocyte globulin in combination with ciclosporin used for
may be used as immunosuppressive treatment for aplastic anaemia.
what is the response rate for IV horse antithymocyte globulin in combination with ciclosporin, vs ciclosporin alone for non severe aplastic anaemia
The response rate for non-severe aplastic anaemia is higher with this combination than with ciclosporin alone.
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)
Prednisolone
Prednisolone is used for the prevention of adverse effects associated with antithymocyte globulin treatment. Early reactions that may occur include …
fever, rash, fluid retention, rigors, acute respiratory distress syndrome, and anaphylaxis; serum sickness may occur 7–14 days later.
Who can give antithymocyte globulin treatment and in which facilities
Antithymocyte globulin should be given under specialist supervision with appropriate resuscitation facilities
Intravenous horse antithymocyte globulin in combination with ciclosporin, may be used as immunosuppressive treatment for aplastic anaemia.
What are some other treatment options (2)
ciclosporin alone or oxymetholone.
This vitamin B is licensed for the treatment of idiopathic sideroblastic anaemia; the dose required is usually high.
pyridoxine HCl
What is used to treat anaemia associated with erythropoietin deficiency in chronic renal failure and why?
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.
which epoetins are long acting
long-acting agents darbepoetin alfa and methoxy polyethylene glycol-epoetin beta
epoetins 2 CI
Pure red cell aplasia following erythropoietin therapy; uncontrolled hypertension
common SE for epoetins
Arthralgia; embolism and thrombosis; headache; hypertension (dose-dependent); influenza like illness; skin reactions; stroke
important info re epoetins and hypertensive crises
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.
Pt on epoetin develops pure red cell aplasia. What should you do
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.
Patient on epoetin develops HTN. what should you do
interrupt treatment
MHRA/CHM advice: Recombinant human erythropoietins: very rare risk of severe cutaneous adverse reactions
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.
MHRA Erythropoietins—haemoglobin concentration
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
…. toxicity (can impair the response to erythropoietin)
aluminium