Lecture 11 - Autoimmunity In Hematology Flashcards

1
Q

Positive protective immune response

A
  • immunity
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2
Q

Negative protective immune response

A
  • self tolerance
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3
Q

Defective positive immune response

A
  • autoimmunity

- allergy

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4
Q
  • negative defective immune response
A
  • immunodeficiency

- infection - malignancy

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5
Q

Paroxysmal cold haemoglobinuria

A
  • first described autoimmune disease
  • something coats RBC and results in their lysis
  • called “erythrocytes coating factors”
  • ANA: antinuclear factors
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6
Q

Self/non self discrimination

A
  • during development in primary lymphoid tissue

- T and B lymphocytes learn to discriminate between self and foreign antigens

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7
Q

Self tolerance two signal hypothesis

A
  • in embryo: clonal deletion of T cell that bind to self molecules
  • but also occurs later: need two signals: an antigen and a costimulatory signal
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8
Q

Stimulate CD28

A
  • goes from anergy to activation and prloferation
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9
Q

Block B7

A
  • goes from activation and proliferation to anergy
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10
Q

Autoimmunity

A
  • precipitated by interaction of environment with genetic susceptibility: infection, drug
  • transient responses can occur after inflammation but only persist if genetic susceptibility
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11
Q

Common features of autoimmune disease

A
  • genetic predisposition
  • female preponderance
  • chronic fluctuating course
  • lymphoid infiltrate in tissues
  • presence of autoantibodies
  • response to immunosuppression
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12
Q

Autoimmune hemolytic anemia

A
  • antibodies to components of the erythrocyte membrane
  • may be detected +/- complement -> hemolysis
  • generally treatment is by immunosuppression
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13
Q

Type II cytotoxic reaction

A

Ab binds to RBC -> activation of complement pathway -> MAC complex punches whole in cell-> lysis

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14
Q

Immune thrombocytopenia

A
  • decrease in platelet numbers -> petecchia
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15
Q

Causes of thrombocytopenia: isolated/incidental

A
  • immune
  • gestational
  • occult liver disease
  • myelodysplastic syndrome
  • congenital
  • HIV infection
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16
Q
  • isolated, severe, symptomatic thrombocytopenia causes
A
  • immune

- drug induced

17
Q

Multisystem thrombocytopenia causes

A
  • SLE
  • Drug induced
  • sepsis
  • cancer
  • liver disease
  • lymphoma, leukemia
  • antiphospholipid syndrome
  • pregnancy
  • PNH
  • nutrient deficiency
18
Q

Idiopathic thrombocytopenic purpura

A
  • peripheral platelet destruction by antibodies
  • antibodies bind to antigens on platelets, often GP IIb-IIIa
  • removed particularly in spleen
  • occurs in young women
  • predisposed by viral infections: HIV, EBV
  • often associated with other autoantibodies
19
Q

Autoimmune thrombocytopenia

A
  • decreased number of platelets, often large
  • usually normal megakaryocytes in BM
  • treatment only if
20
Q

Antiphospholipid syndrome

A
  • venous arterial and small vessel thrombosis
  • pregnancy loss : 1st semester
  • preerm delivery for patients with severe pre-ecclampsia or placental insufficiency
  • migraine
  • livedo reticularis
  • cardiac/vascular disease
  • thrombocytopenia
  • heamolytic anaemia
  • stroke, TIA
  • chorea
  • ulcers
  • renal thrombotic microangiopathy
21
Q

Heparin induced thrombocytopenia

A
  • makes antibody to PF4
  • associated with thrombosis, not bleeding
  • occurs 5-10 days after exposure to heparin
  • 50% reduction in platelet
  • causes clotting in various tissues