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HEMATOLOGY 2 > DISORDERS OF PRIMARY HEMOSTASIS – PLATELET DISORDERS > Flashcards

DISORDERS OF PRIMARY HEMOSTASIS – PLATELET DISORDERS Flashcards

1
Q

a term wherein there’s a CHANGES IN
PLATELET FUNCTION

A

THROMBOCYTOPATHY

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2
Q

conditions associated with platelet aggregation

A
  • glanzmann thrombasthenia
  • hereditary afibrinogenemia
  • acquired defects of aggregation
    ✓acquired von willebrand disease
    ✓ acquired uremia
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3
Q

conditions associated with platelet adhesion

A

− Bernard-Soulier Syndrome
− Von Willebrand Disease
− Acquired Defects of Platelet Adhesion
✓ Myeloproliferative/ Lymphoproliferative
Disorders, Dysproteinemias
✓ Antiplatelet Antibodies
✓ Cardiopulmonary Bypass Surgery
✓ Chronic Liver Disease
✓ Drug-Induced Membrane Modification

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4
Q

congenital disorders of platelet function based on platelet membrane defects

A

Glanzmann’s Thrombasthenia
Bernard-Soulier Syndrome
Storage Pool Deficiency (Granule Defect)

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5
Q

congenital disorders of platelet function based on platelet release

A

primary secretion defects (Enzymatic Pathway Defects)

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6
Q

In quantitative, we have two ___

A

thrombocytosis and thrombocytopenia

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7
Q

what causes the decrease in plt or thrombocytopenia

A

increase PLT destruction
Decrease platelet production
abnormal platelet distribution

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8
Q

what causes the decrease in plt or thrombocytosis

A

PRIMARY/ ESSENTIAL
THROMBOCYTOSIS

REACTIVE/ SECONDARY
THROMBOCYTOSI

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9
Q

clinical manifestation of bleeding disorders

A

superficial bleeding
deep tissue bleeding

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10
Q

in clinical manifestation of bleeding, superficial bleeding includes

A

petechiae
epistaxis (nose bleeding)
gingival bleeding

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11
Q

in clinical manifestation of bleeding, deep tissue bleeding includes

A

hematomas
hemarthrosis (joint bleeding)

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12
Q

hemarthrosis means bleeding in __

A

joints

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13
Q

In bernard soulier syndrome, ____complex is missing from the platelet surface or exhibits abnormal function

A

GP Ib/IX/V

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14
Q

BERNARD-SOULIER SYNDROME is also known as

A

giant platelet disorder

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15
Q

why BSS is called as giant platelet disorder

A

because it can grow from 5-8 um up to 20 um

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16
Q

BSS is characterized by ___

A

giant platelets and thrombocytopenia

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17
Q

who first studied bernard-soulier syndrome,, where they described two children from a consanguineous family who had a sever bleeding disorder characterized by mucocutaneous hemorrhage

A

1948, bernard and soulier

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18
Q

They identified an abnormality in platelet GPIb as the cause of the functional defect

A

1975, Nurden and Caen

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19
Q

Six different feature features may contribute to the hemorrhagic diathesis in BSS

A

thrombocytopenia
von willebrand’s factor is deficient (carrier of factor VIII and for adhesion)
abnormal platelet interaction with thrombin
abnormal plt coagulant activity
abnormal plt interactions with P selectin - P selection functions in adhesion
abnormal plt concentrations with leukocyte integrin a MB2

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20
Q

Laboratory features of BSS

A

normal responses to ADP, epinephrine, collagen and arachidonic acid

do not respond to ristocetin and thrombin

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21
Q

treatment for BSS

A

no tx as it is congenital

platelet transfusion - therapy of choice - however causes production ALLOANTIBODIES; thus PLATELETPHERESIS is better

management : desmopressin for enhancement of plt function

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22
Q

DDAVP or desmopressin means

A

1-deamino-8-D-arginine vasopressin

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23
Q

this 1-deamino-8-D-arginine vasopressin functions as

A

increases the function of PLT

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24
Q

what is missing in glanzmann thrombasthenia

A

GbIIb- IIIa responsible for aggregation

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25
Q

mode of inheritance of glanzmann thrombasthenia

A

rare autosomal recessive disease

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26
Q

who studied glanzmann thrombastenia

A

1918, eduard glanzmann

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27
Q

which deep tissue bleeding manifestation is not present in glanzmann’s thrombasthenia

A

deep hematoma
and recurrent hemarthrosis are not present

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28
Q

Manifests in the neonatal period or infancy, with bleeding after circumcision and frequent epistaxis and gingival
bleeding

A

GLANZMANN’S THROMBASTHENIA

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29
Q

Originally a bleeding disorder associated with abnormal in vitro clot retraction and normal platelet count

A

GLANZMANN’S THROMBASTHENIA

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30
Q

Hemorrhagic manifestation of glanzmann thrombasthenia

A

petechiae
purpura
menorrhage
GIT bleeding and hematuria

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31
Q

Bleeding is most common from mucosal surfaces (easy bruisability, epistaxis, gingival bleeding, prolonged
bleeding for minor cuts and menorrhagia)

A

glanzmann thrombasthenia

32
Q

in glanzmann thrombasthenia, __ and ___ are seen in infants associated with crying

A

facial petechiae and subconjunctiva hemorrhages

33
Q

Laboratory features of GLANZMANN THROMBASTHENIA

A

normal platelet count and platelet morphology
lack of plt aggregation in response to all plt activating agent (ADP, collagen, thrombin, epinephrine)
ristocetin-induced binding of VWF to plts and the resulting plt agglutination are normal
cbc is normal
procoagulant activity is diminised
GT platelets are not as activated as are normal plts

34
Q

treatment of glanzmann thrombasthenia

A

transfusion of normal plt
to reduce alloabtibodies, single donor plts apheresis
recombinant factor VIIa
hormonal therapy to control menorrhagia
antifibrinolytic therapy to control gingival hemorrhage or excessive bleeding after tooth extraction

35
Q

glanzmann thrombasthenia and bernard-soulier syndrome

plt count

A

normal in GT
decrease in BSS

36
Q

glanzmann thrombasthenia and bernard-soulier syndrome

morphology

A

normal in GT
giant in BSS

37
Q

glanzmann thrombasthenia and bernard-soulier syndrome

bleeding time

A

both prolonged - parehong plt function and problem eh

38
Q

glanzmann thrombasthenia and bernard-soulier syndrome

plt aggregation - ADP

A

abnormal in GT
normal in BSS

39
Q

glanzmann thrombasthenia and bernard-soulier syndrome

PLT AGGREGATION THROMBIN

A

both abnormal

40
Q

glanzmann thrombasthenia and bernard-soulier syndrome

plt aggregation - collagen

A

both abnormal

41
Q

glanzmann thrombasthenia and bernard-soulier syndrome

plt aggregation - epinephrine

A

abnormal GT
normal BSS

42
Q

glanzmann thrombasthenia and bernard-soulier syndrome

plt aggregation - ristocetin

A

normal GT
abnormal BSS

43
Q

what are the aggregating agent

A

ADP
THROMBIN
COLLAGEN
EPINEPHRINE
RISTOCETIN

44
Q

QUEBEC plt disorder is originally described as

A

Platelet Factor 5 quebec

45
Q

problem in quebec platelet disorder

A

platelet factor 5

46
Q

problem in quebec platelet disorder

A

inability to form clot all together as it lacks PF5

47
Q

Quebec platelet disorder

characterized by

A

bleeding - specific defect in plt function

48
Q

this plt disorder resembles BSS

A

MEDITERRANEAN MACROTHROMBOCYTOPENIA

49
Q

mode of inheritance of Mediterranean macrothrombocytopenia

A

autosomal dominant thrombocytopenia

in relation with BSS, it’s autosomal recessive

50
Q

Mediterranean macrothrombocytopenia has a gene mutation to the short arm of chromosome ___

A

17 (GP Ia)

51
Q

genotype and phenotype are equivalent to that of carrier of bernard soulier syndrome

A

Mediterranean macrothrombocytopenia

52
Q

PLATELET STORAGE POOL DISEASE

A

hermansky-pudlak syndrome
chediak higashi syndrome
wiskott-aldrich syndrome
gray plt syndrome

53
Q

mode of inheritance of hermansky-pudlak syndrome

A

rare autosomal recessive disorder

54
Q

patient shows albinism (oculocutaneous) and may have hemorrhagic events

A

hermansky-pudlak syndrome

55
Q

characterized by tyrosinase-positive oculocutaneous albinism, defective lysosomal function in a variety of cell types, ceroid-like deposition in the cells of the RES and profound platelet dense granule deficiency

A

hermansky-pudlak synrome

56
Q

HERMANSKY-PUDLAK
SYNDROME (HPS)

which chromosome is affected

A

chromosome 19

57
Q

Marked dilation and tortuosity of the surface connective tubular system (Swiss cheese
platelet)

A

HERMANSKY-PUDLAK
SYNDROME (HPS)

58
Q

is bleeding in hermansky pudlak syndrome severe?

A

,most bleeding is not severe

59
Q

An autosomal-recessive disorder, in which patients show albinism and giant lysosomal
granules in neutrophils

A

chediak higashi syndrome

60
Q

Storage pool defect on dense granules

A

chediak higashi syndrome

61
Q

Patients show frequent infections because of impaired phagocytic ability and death
usually occurs in childhood

A

chediak higashi syndrome

62
Q

lab findings in chediak higashi syndrome

A

Neutropenia is present
thrombocytopenia

63
Q

Partial oculocutaneous albinism

A

CHEDIAK-HIGASHI
SYNDROME

64
Q

Platelet dense granule deficiency and hemorrhages

A

CHEDIAK-HIGASHI
SYNDROME

65
Q

An X-linked recessive disorder in which patients show severe eczema, recurrent
infections, immune defects, thrombocytopenia and small platelets

A

WISKOTT-ALDRICH
SYNDROME

66
Q

WISKOTT-ALDRICH
SYNDROME triad

A

thrombocytopenia
eczema
recurrent infection

67
Q

diseases or conditions that patients with hermansky pudlak syndrome can acquire

A

albinism
lung disease (pulmonary fibrosis)
bleeding problem
vision loss
defect in plt granules

68
Q

Alpha granules are absent or greatly reduced

A

GRAY PLATELET
SYNDROME

69
Q

Gray platelet syndrome will result into leakage of

A

growth factor and cytokines in the marrow causing myelofibrosis

70
Q

mode of inheritance of Gray plt syndrome

A

autosomal recessive

71
Q

GRAY PLATELET
SYNDROM characteristics

A

thrombocytopenia
fibrosis of the marrow
large platelet

72
Q

plt satellitism cause

A

anticoagulant - ply surrounds the neutrophil

73
Q

ACQUIRED DEFECTS OF PLATELET FUNCTION

A

myeloproliferative disorders
cardiopulmonary bypass
paraproteinemia
liver disease
uremia

74
Q

MYELOPROLIFERATIVE
DISORDERS (MPD) is characterized by

A

polycythemia vera
idiopathic myelofibrosis
cmk
essential thrombocythemia

75
Q

Hemorrhagic manifestations of myeloproliferative disorders

A

epistaxis
ecchymosis
mucocutaneous bleeding from GUT and GIT

76
Q
A

HEMATOLOGY 2 (18 decks)

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