haematological emergencies Flashcards

1
Q

what is acute hemolytic transfusion reaction ?

A

acute intravascular hemolysis due to ABO blood group incompatibility

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2
Q

what is the presentation with acute hemolytic transfusion reaction ?

A

chest pain
shortness of breath
back pain
fever
tachycardia
orr shock

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3
Q

what are the complications of acute hemolytic transfusion reaction ?

A

1- acute renal failure
2- disseminated intravascular coagulopathy (DIC)
3- cardiovascular collapse and death

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4
Q

what is the management for acute hemolytic transfusion reaction ?

A

1.stop the transfusion immediately
2.give IV fluids
3.if there is hemolysis give loop diuretics
4. if there is refractory hypotension use IV vasopressors

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5
Q

what are the labs used to check for hemolysis ?

A

send a peripheral smear CBC
LDH is high in hemolysis
haptoglobin is low in hemolysis
indirect bilirubin is high
direct coombs test
check creatinine
check urine
coagulation profile

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6
Q

what happens in type 1 hypersensitivity reaction after blood transfusion ?

A

patient presents with hives and pruritus without fever , bronchospasm or shock

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7
Q

what is the management for type 1 hypersensitivity reaction to blood transfusion ?

A

stop the transfusion
give epinephrine
corticosteroids
bronchodilators
IV fluids
supplementary oxygen

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8
Q

how can we protect against anaphylaxis ?

A

IgA deficient donors blood is available
washed cells are also preferred

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9
Q

what is the most common type of transfusion related reaction ?

A

simple febrile reaction

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10
Q

how does the simple febrile reaction happen ?

A

recipients antibody reacts to the donors leukocytes

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11
Q

what is the difference in presentation between simple febrile reaction and anaphylaxis ?

A

simple febrile reaction the patient presents with fever with no urticaria no bronchospasm no shock

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12
Q

how do we avoid simple febrile reactions ?

A

leukoreduction of the transferred component

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13
Q

what is the management for simple febrile reactions ?

A

slow down the transfusion and give paracetamol

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14
Q

what is the presentation of transfusion associated acute lung injury ?

A

non cardiogenic pulmonary oedema
can cause ARDS

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15
Q

what antibody is associated with TRALI ?

A

anti HLA

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16
Q

when does TRALII most commonly happen ?

A

after plasma transfusion but any plasma containing product can cause it

17
Q

what is the management of TRALI ?

A

1- supplemental oxygen and ventilation
2- central line should be placed for haemodynamic support just in case there is hypotension

18
Q

what is the management of TRALI ?

A

diuretics if the cause is CHF

19
Q

what is delayed transfusion reaction ?

A

exaggerated response to a foreign red cell antigen
presents 5 days to a week post transfusion
patient presents with jaundice, anemia and fever

20
Q

what are the triggers for DIC ?

A

severe sepsis or infection
solid malignancies
pregnancy
haematological malignancies
APL

21
Q

what are the two types of DIC ?

A

overt ( decompensated) and non overt (early )

22
Q

what are the differential diagnosis of DIC ?

A

1- hepatic cirrhosis
2- HUS and TTP
3- pregnancy related thrombocytopenia
HELLP
4- heparin induced thrombocytopenia

23
Q

what is the treatment approach for DIC ?

A

generally - treat the underlying condition

if asymptomatic and only lab evidence - give LMWH

if there is thromboembolism - LMWH

if there is bleeding - LMWH

24
Q

what is the normal platelet count ?

25
when are schistocytes seen on blood film ?
in cases of thrombotic microangiopathies like HUS and TTP
26
what are the different types of microangiopathic hemolytic anemias ?
HUS TTP DIC
27
what is the etiology inn TTP ?
in thrombotic thrombocytopenic purpura there is a deficiency in the ADAMST13 enzyme
28
what are the lab findings associated with TTP ?
low platelet count PT and PTT are unaffected schistocytes elevated LDH elevated Bilirubin low haptoglobin negative coombs raised reticulocyte count
29
what is the function of ADAMST13 enzyme ?
degrades vWF multimers into monomers the inability to break these multimers increases the likelihood of the formation of platelet thrombosis
30
what is the management of TTP ?
emergency plasma ecxchange plus frresh frozen nplasma steroids
31
what is contraindicated in the treatment of TTP ?
platelet transfusion
32
what is the characteristic pentad of TTP ?
MAHA acute renal failure thrombocytopenia fever neurological abnormalities
33
how can you differentiate between HUS and TTP ?
there are more prominent neurological features in TTP than with HUS also TTP presents with fever distinction test is with ADAMST13
34
what level of ADAMST13 can we diagnose TTP and exclude HUS ?
below 10 is usually diagnostic for TTP