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IOD > Haemolytic anaemia > Flashcards

Haemolytic anaemia Flashcards

1
Q

Define haemolytic anaemia

A

Increased peripheral destruction of RBCs

Bone marrow continues to produce cells

Increased consumption means BM has to work harder to compensate.

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2
Q

Describe the investigation- what are the components of a haemolysis screen

A

FBC: Normocytic anaemia

Blood film: Spherocytes or red cell fragments

Reticulocytes: Increased

LDH: Increased (due to cell turnover)

Bilirubin: Increased (unconjugated)

Haptoglobin: Decreased in intravascular haemolysis

DAT: Positive in some immune cases

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3
Q

3 examples of inherited haemolytic anaemias (non-immune)

A

SCD
Membrane disorders e.g. hereditary spherocytosis
G6PD deficiency

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4
Q

3 examples of acquired haemolytic anaemia (non-immune)

A

paroxysmal nocturnal haemoglobinuria

DIC

valve haemolysis

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5
Q

example of acquired haemolytic anaemia (immune)

A

AI- warm, cold, drug induced

alloimmune- transfusion reaction, HDN

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6
Q

What is haptoglobin

A

Haptoglobin- protein mopped up by free haemoglobin- destroyed RBC= lots of free Hb= decreased haptoglobin

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7
Q

difference in test results between intravascular and extravascular haemolysis

A

Intravascular- low haptoglobin, haemoglobinuria, haemoglobinaemia, hemosiderinuria

Extravascular- N haptoglobin, no haemoglobinuria, no haemoglobinaemia, no hemosiderinuria

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8
Q

3 types of auto-immune acquired haemolytic anaemia

A

warm
cold
drug induced

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9
Q

Mx of warm AIAH

A

steroids, bloods as necessarr, rituximab, splenectomy

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10
Q

what is autoimmune haemolytic anaemia?

A

autoantibodies directed against a patients own RBCs- usually IgG in warm and IgM in cold mediated.

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11
Q

What is the direct antiglobin test/ Coombs test?

A

blood sample from patient is incubated with antihuman antibodies (Coombs reagent). RBCs agglutinate bc the antihuman abs form links with the human abs on the RBCs.

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12
Q

Is warm AIAH DAT +?

A

Yes

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13
Q

Is warm AIAH DAT +?

A

Yes

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14
Q

2 triggers for cold AIAH

A

EBV, Mycoplasma pneumonia

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15
Q

Mx of cold AIAH

A

keep warm, blood warmers, occasionally chemo

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16
Q

What is MAHA?

A

microangiopathic haemolytic anaemia

17
Q

characteristics of MAHA

A

mechanical destruction (due to environment) of red blood cells and therefore fragments (schistocytes) on the blood film

Often associated thrombocytopenia
(Thrombotic Microangiopathy)

Predominantly an acquired intravascular haemolysis

18
Q

clinical px of TTP (thrombotic thrombocytopenic purpura)

A 
fever
renal failure
confusion
thrombocytopenia
MAHA on blood film
microthrombi 
low plt, Hb
high bili, LDH, creatinine
\+troponin
19
Q

5 causes of TTP

A 
idiopathic
HIV
pregnancy 
drugs 
congenital
20
Q

what does TTP stand for?

A

thrombotic thrombocytopenic purpura

21
Q

Pathogenesis of TTP

A

Endothelial cell damage releases ultra large vWF multimers into the bloodstream which bind to vWF receptors on platelets, causing aggregation.
Reduction of ADAMTS 13 (normally proteolyses vWF multimers)
with associated IgG antibody against ADAMTS 13
ADAMTS 13 is vWF cleaving protease
Reduction leads to platelet aggregation= low plt levels

22
Q

Tx for TTP

A

Plasma exchange, high-dose steroids, blood, folic acid
Rituximab
Aspirin and prophylactic LMWH when platelets recover

23
Q

Normal adult Hb chains

A

2 alpha, 2 beta

24
Q

Pathogenesis of SCA

A

Point mutation in the beta globin gene on chromosome 11
Amino acid substitution (Adenine to thymine)
Causes glutamic acid being substituted by valine at position 6

25
Q

Screening for SCA

A

heel prick at day 8
gel elctrophoresis
sickle solubility

26
Q

Describe the pathophysiology of SCD

A

sickle shape leads to vaso-occlusion and infarction resulting in downstream tissue death and inflammation

Irreversible sickling leads to haemolysis

This leads to release of Hb and consumption of NO leading to a functional deficiency.

27
Q

5 symptoms of SCD

A 
acute pain
acute chest syndrome
hyposplenism
nephropathy 
inflammation 
pulm HT
priapism
leg ulcers
cerebrovascular disease
retinopathy
28
Q

emergency events in SCD

A 
acute anaemia
acute painful crises
acute chest syndromes- hypoxaemia, chest pain, fever, infiltration
stroke
priapism 
abdominal/mesenteric crises
AKI 
papillary necrosis 
overwhelming infection
29
Q

emergency events in SCD

A 
acute anaemia
acute painful crises
acute chest syndromes- hypoxaemia, chest pain, fever, infiltration
stroke
priapism 
abdominal/mesenteric crises
AKI 
papillary necrosis 
overwhelming infection
30
Q

4 emergency mx of SCD

A

Pain relief within 30mins
Oxygen
Antibiotics
Blood transfusions

31
Q

9 aspects of long term SCD care

A 
Clinic follow up
Echocardiographs
Hip examinations
Retinal screening
Urinalysis: protein
BP monitoring
Pain relief
Hydroxycarbamide
Blood Transfusions

IOD (54 decks)

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