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Primary hemostasis disorders Flashcards

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1
Q

Primary hemostasis disorders

A

vascular disorder

Platelet disorder

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2
Q

Secondary hemostasis disorders

A

Coagulation factor disorders

Fibrinolytic disorder

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3
Q

Primary hemostasis characteristics

A

“superficial bleeding at the surface” –> mostly skin and mucous membrane bleeds

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4
Q

Epistaxis

A

nose bleed

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5
Q

petechiae

A

pinpoint bruising

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6
Q

purpura

A

bleed under the skin resulting in larger flat areas (bruises)

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7
Q

PH bleedings

A

prolonged bleeding of minor cuts
spontaneous gingival bleeds

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8
Q

Mostly skin and mucous membrane bleeds

A

primary hemostasis

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9
Q

Vascular phase disorders

A

Before platelet plug forms, excess RBCs leave vessel and start to pool under skin or mucous membrane.

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10
Q

Vascular phase disorders

A

Primary Purpura

Secondary Purpura

Vascular and connective tissue disorders

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11
Q

Primary purpura

A

Disorders that results in bruising not associated with any specific disease

Mild bruising with minimal trauma.

Example: Bruising from sneezing, light physical touch, loss of subcutaneous tissue.

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12
Q

Secondary purpura

A

result of another disease process

Ex: infections or allergic reactions

Metabolic imbalances (Scurvy - Vit C def.)

Abnormal blood vessel structure –> leads to increased blood permeability

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13
Q

example of vascular and connective tissue disorder

A

Hereditary hemorrhagic Talangiectasia (HHT)

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14
Q

HHT disease

A

Arteries and veins become abnormally connected

Development of telangiectasis (red line patterns) on mucous membranes and skin

Lesions develop on the tongue, lips, palate, face, hands, nasal mucosa and GI tracts

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15
Q

Quantitative disorders

A

Thrombocytopenia

Thrombocytosis

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16
Q

Qualitative PLT disorder

A

Congential disorders of PLT function
- PLT membrane
- PLT secretion
- Coagulation

Acquired qualitative platelet disorders

Related: von Willebrand Disease

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17
Q

Thrombocytopenia

A

Platelets must be present in adequate numbers to maintain normal hemostasis

Thrombocytopenia: <150,000/ul

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18
Q

PLT<60,000/ul

A

unexpected bleeding

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19
Q

PLT<10,000/ul

A

spontaneous bleeding/hemorrhaging

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20
Q

thrombocytopenia is due to

A

deficient platelet production

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21
Q

Myelophthsisc conditions

A

BM is infiltrated

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22
Q

aplasia

A

BM is hypocellular (mostly fat)

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23
Q

Ineffective erythropoiesis (and thrombopoiesis)

A

megaloblastic anemia

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24
Q

Congenital disorders, examples

A

May Hegglin
Chediak Higashi
Bernard-Soulier

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25
Thrombocytopenia: May-Hegglin Syndrome
Inherited PLT disorders, resulting in abnormally large and misshapen platelets (giant platelets)
26
WBC have increased Doyle-like bodies
May Hegglin syndrome
27
Hypersplenism
Spleen normally sequesters 30% of platelets When it is enlarged (splenomegaly), it sequesters more
28
Kasabach - Merit Syndrome
Rare disorder with giant hemangiomas (vascular tumors) that are believes to be sequestering platelets from circulation
29
Immune (idiopathic) thrombocytopenia purpura (ITP)
autoantibody to self-platelets
30
post transfusion purpura
alloantibody to donor platelets
31
neonatal isoimmune purpur a
maternal antibody to baby platelets
32
drug induced thrombocytopenia
drug bound antibody to self platelets
33
Heparin induced thrombocytopenia and thrombosis
abnormal antibodies formed that activate platelets and get used up making non-needed clots.
34
secondary immune platelets destruction disorders
All present with ITP-like thrombocytopenia that is due to some other disease process - SLE - Infections (viral - HIV, hepatitis), or parasitic (malaria) - Lymphoproliferative disorders (Hodgkins, non-hodgkins lymphoma)
35
TTP - Thrombotic Thrombocytopenic Purpura
Fibrinolysis inhibited + deficiency in ADADMTS-13, leads to increased platelet aggregation and thrombosis
36
Hemolytic Uremic Syndrome (HUS)
Infection by E.Coli O157;H7 or others that lead to platelet destruction, anemia, acute kidney failure.
37
Disseminated Intravascular Coagulation (DIC)
Accelerated platelet destruction + coagulation factor consumption (micro thrombi)
38
Thrombocytopenia: increased platelet destruction Results in:
Microangiopathic thrombocytopenia - conditions that result in thrombosis (blood clot formation) in small vessels (capillaries and arterioles)
39
Thrombocytosis - PLT count
>450,000 PLT/ul
40
Primary thrombocytosis
often due to myeloproliferative disorders Platelet proliferation is not due to thrombopoietin levels
41
Reactive thrombocytosis
Often caused by a reaction to something and can either be transient or chronic
42
acute hemorrhage
platelet counts increase after to try to replenish lost TRANSIENT
43
post splenectomy
spleen no longer sequestering platelets CHRONIC
44
Drugs
Epinephrine causes rapid transient increase
45
qualitative disorders of platelet function
congenital
46
Platelet membrane problem
Bernard soulier syndrome Glanzmanns thrombasthenia
47
secretion release problem
storage pool deficiency primary secretion defects
48
Bernard Soulier syndrome
deficiency of platelet GPIb/IX complex Rare Bleeding due to : - thrombocytopenia - Abnormality of platelet-vWF binding in platelet adhesion abnormality of platelet - thrombin interaction
49
Glanzmann Thrombobasthenia
Deficiency of platelet GPIIb/IIIa receptor (needed for fibrinogen bridges) Deficiency causes abnormal clot retraction --> patient forms loose clot
50
Secretion release problem - storage pool deficiency
granule proteins present/absent and platelet morphological appearance
51
Gray-platelet syndrome
deficiency of alpha granules with granular appearance of platelets
52
dense granule deficiencies
associated with congenital abnormalities including Oculocutaneous albinism
53
hermansky - pudlak syndrome
neutropenia, pulmonary fibrosis, prolonged bleeding
54
Chediak Higashi
Oculocutaneous albinism + dense granule deficiency + thrombocytopenia GIANT AZUROPHILIC GRANULES in WBC
55
secretion defects
- due to enzymatic pathway - granules are present, but not released properly - presentation is similar to storage pool deficiencies
56
Platelet coagulation defects
PLT disorders that result in thrombin not being generated rare! symptoms close to hemophilia
57
non-immune cause
PLT dysfunction by systemic illness or medication more common than congenital qualitative PLT disorders
58
EX of qualitative PLT disorders
Uremia (renal disease) Liver disease Paraproteinemias - excess immunoglobin proteins (MM, WM) Myeloproliferative disorders - PV, idiopathic, myelofibrosis, essential thrombocyothemia
59
Von willebrand disease
Most prevalent inherited bleeding disorder worldwide - inherited deficiency of vWF - often confused with primary PLT disorder - missing VIII:vWF
60
vWF disease classified:
by ABO blood type, lowest in O type 1 = 70-80%, quantitative decrease in vWF, mild bleeding type 2 = 15-20%, qualitative abnormality in vWF structure, mild to moderate bleeding type 3 = very rare, absent levels of vWF, severe bleeding, similar to hemophilia A
61

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