Lecture 27 - Platelets Flashcards

1
Q

What type of HLA do platelets have

A

Class I

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2
Q

What are HPA

A
  • human platelet allo-antigens
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3
Q

What is GPIIb/IIIa

A
  • it binds natural ligands and causes aggregation
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4
Q

What is GPIa/IIa

A
  • binds collagen and takes part in adhesion and activation
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5
Q

What is GP1b/V/IX

A
  • binds von Willebrand factor and stimulates adhesion
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6
Q

What are platelet allo-antibodies

A
  • antibodies to non-self platelet specific antigens may be developed following exposure to non-self platelet HPA
  • anatobodies to non-self class I HLA may be developed following exposure
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7
Q

What is fetal/neonatal allo-immune thrombocytopenia

A
  • mom develops IgG antibodies to the baby’s platelet allo-antigens
  • passive thrombocytopenia
  • major platelet antigens are expressed as early as 18-19 weeks gestation
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8
Q

What are the clinical aspects of FNAIT

A
  • approx 60% of cases detected in first pregnancy
  • recurs frequently in subsequent pregnancies
  • intracranial haemorrhage in 10-30% of cases
  • thrombocytopenia for 1-3 weeks post partum
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9
Q

Laboratory detection of FNAIT

A
  • antibody screen in materna serum
  • AB screen done during gestation if previous affected pregnancy
  • HPA alloantigen typing of mom and dad
  • anti-HPA-1A most common
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10
Q

Methods used to detect HPA Antibodies

A
  • PIFT-FFC
  • SPAA
  • ACE
  • MAIPA
  • Lunimex
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11
Q

What is the follow up testing to antibody ID

A
  • if antibodies were detected do HPA genotyping
  • if HLA class I antibodies were detected
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12
Q

What is the treatment of FNAIT

A
  • transfusion with maternal washed platelets - preferably apheresis
  • unrelated HPA typed donors are often used
  • percutaneous umbilical blood sampling can be performed to monitor platelet count in utero
  • treatment of mum with IVIg during pregnancy
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13
Q

What is post transfusion purpura

A
  • rare event characterized by an acute episode of severe immune thrombocytopenia approximately one week (5-12 days) post transfusion
  • amnestic response to previous exposure
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14
Q

What is the PTP frequency

A

1 per 2 million RBCs

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15
Q

What occurs in PTP

A
  • destruction of patient’s own platelets as well as transfused platelets result in platelet counts less than 15
  • mechanism unknown
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16
Q

What is the treatment of PTP

A
  • IVIg
  • corticosteroids
  • platelet transfusion
  • plasmapheresis
  • in future use HPA matched units and IVIg pre-transfusion
17
Q

What is refractoriness to platelets

A
  • multiply transfused patients can develop anti-platelet antibodies with a frequency of 50-90%
  • most common antibody causing refractoriness is anti-HLA class I
18
Q

What is the clinical definition of PLTRE

A
  • one hour post transfusion counts are required that show unsatisfactory platelet increment
  • often it is recommended to have at least two episodes of unsatisfactory platelet increment before refractoriness is investigated
  • corrected count increment is used to assess the success
19
Q

What are alternate causes of thrombocytopenia

A
  • fever
  • splenomegaly
  • DIC
  • drug related immune destruction
20
Q

What is the antibody testing algorithm

A
  • class I HLA antibody screen
  • if no HLA antibodies detecting HPA antibody may be done
  • HLA typing if required
  • HPA genotyping if required
21
Q

What is the treatment of PTR

A
  • use of HLA matched units
  • IVIg
  • continuous monitoring of PRA following future transfusions
22
Q

What are the methods for HLA Ab testing in refractory patients

A
  • serology methods
  • solid phase assays