PATH LAB Flashcards

1
Q

During primary homeostasis, what occurs in these stages:

  1. adhesion
  2. activation
  3. aggregation
A
  1. adhesion: platelets adhere to subendothelium via vWF
  2. activation - platelets activated resulting in shape change, release of ADP and thromboxane A2
  3. aggregation - more platelets recruited to form haemostatic plug
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2
Q

What does PT and APTT measure? and what does it mean if they are prolonged?

A

PT - extrinsic and common pathway
– FVII deficiency inherited or due to DIC, warfare, vit K deficiency
APTT - intrinsic and common pathway
– haemophilia (FVIII deficiency), FIX + FXI deficiencies, VWD

Both prolonged = FX deficiency

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3
Q

What factors are in the extrinsic pathway and what triggers it?

A

Tissue factor activates FVII

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4
Q

What factors are in the intrinsic pathway and what triggers it?

A

Surface contact - FXII, XI, IX, VIII

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5
Q

What is DIC and what would investigations show?

A

DIC - systemic activation of blood coagulation resulting in intravascular coagulation and depletion of platelets, coagulation factors and fibrinogen and bleeding complications
Investigations: low platelets, prolonged INR (PT), SPTT, TT and decreased fibrinogen and factors

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6
Q

During group and screen for transfusions, what is the forward group, reverse group and control?

A

Forward - patient RBC + anti-A/B/RhD antibodies
Reverse - patient serum + group A / B cells to confirm group
Control - patient RBC + no antibody to ensure no spontaneous agglutination

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7
Q

What is a crossmatch?

A

Patient serum + donor RBC to check for agglutination

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8
Q

Where does haematopoiesis occur in adults?

A

pelvis, sternum and vertebral bodies

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9
Q

How is acute leukaemia defined and what are the types?

A

> 20% blast cells in bone marrow at presentation

  • AML: failure of myeloid cells to differentiate beyond blasts (Auer rods indicate faggot cells and AML)
  • ALL: in bone marrow, lymphoid precursors proliferate and replace normal HSC
  • APL: subtype of AML, accumulation of abnormal promyelocytes, bleeding tendency, presence of t(15;17) mutation = PML-RAR-a
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10
Q

What is the diagnosis of APL based on?

A
  • butterfly/bottom cells, and faggot cells
  • flow cytometry
  • fluorescent probe for chromosome t(15;17) translocation
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11
Q

What is bleeding in APL associated with?

A

Thrombocytopenia - BM failure
XS coagulation - DIC
Hyperfibrinolysis

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12
Q

What is the treatment for APL?

A

All-trans-retinoic-acid (ATRA) - drive promyelocytes into mature neutrophils
- gives AML one of the best prognosis
Arsenic tetroxide - induces apoptosis and PML-RAR-a degradation

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13
Q

What are the 4 main causes of angioedema?

A
  1. allergic angioedema
  2. Drug-induced angioedema - ACE inhibitors, statins
  3. idiopathic angioedema - stress, alcohol, food
  4. Hereditary angioedema - genetic mutation in C1 esterase inhibitor gene
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14
Q

What are the 3 main categories of drug induced angioedema?

A
  1. specific IgE - penicillin, cephalosporin
  2. Non-specific (intolerance) - NSAIDS
  3. Kinin-dependent (ACEi, ARBs)
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15
Q

What is the mechanism for angioedema associated with ACE inhibitors?

A

ACEi block renin/bradykinin pathway - decreased production of AT2 and increased bradykinin levels
Increased bradykinin - permeability and vasodilation
– smoking and ACE induced cough increased risk for this reaction

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16
Q

What is the most common type of hereditary angioedema?

A

Type I HAE: mutation in SERPING1 gene with decreased levels of C1 esterase inhibitor

17
Q

What are the effects of C1 esterase inhibitor deficiency?

A
  • increased C1 level
  • abnormal activation of complement
  • activation of coagulation/fibrinolysis pathway - increased clotting
18
Q

What is the most reliable test for HAE?

A

Serum c4 level

19
Q

What can treat acute attacks of HAE?

A
  1. C1-INH concentrate
  2. Kallikrein inhibitor
  3. Selective bradykinin B2 receptor antagonist

fresh frozen plasma - for prophylaxis

20
Q

Abnormal susceptibility to infections presents as:

A
  • abnormally recurrent infections
  • persistent infection
  • severe/atypical infection
21
Q

What results in increased susceptibility to fungal infections?

A

defects in cell mediated immune response

22
Q

What is the relationship between a/b disorder and coeliac’s?

A

Patients with IgA deficiency have 10x risk of developing coeliac disease