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 ?

A

150 - 450

25
Q

when are schistocytes seen on blood film ?

A

in cases of thrombotic microangiopathies
like HUS and TTP

26
Q

what are the different types of microangiopathic hemolytic anemias ?

A

HUS
TTP
DIC

27
Q

what is the etiology inn TTP ?

A

in thrombotic thrombocytopenic purpura there is a deficiency in the ADAMST13 enzyme

28
Q

what are the lab findings associated with TTP ?

A

low platelet count
PT and PTT are unaffected
schistocytes
elevated LDH
elevated Bilirubin
low haptoglobin
negative coombs
raised reticulocyte count

29
Q

what is the function of ADAMST13 enzyme ?

A

degrades vWF multimers into monomers
the inability to break these multimers increases the likelihood of the formation of platelet thrombosis

30
Q

what is the management of TTP ?

A

emergency plasma ecxchange plus frresh frozen nplasma
steroids

31
Q

what is contraindicated in the treatment of TTP ?

A

platelet transfusion

32
Q

what is the characteristic pentad of TTP ?

A

MAHA
acute renal failure
thrombocytopenia
fever
neurological abnormalities

33
Q

how can you differentiate between HUS and TTP ?

A

there are more prominent neurological features in TTP than with HUS
also TTP presents with fever
distinction test is with ADAMST13

34
Q

what level of ADAMST13 can we diagnose TTP and exclude HUS ?

A

below 10 is usually diagnostic for TTP