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Hematology > Primary hemostasis disorders > Flashcards

Primary hemostasis disorders Flashcards

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
Q

Thrombocytopenia: May-Hegglin Syndrome

A

Inherited PLT disorders, resulting in abnormally large and misshapen platelets (giant platelets)

26
Q

WBC have increased Doyle-like bodies

A

May Hegglin syndrome

27
Q

Hypersplenism

A

Spleen normally sequesters 30% of platelets

When it is enlarged (splenomegaly), it sequesters more

28
Q

Kasabach - Merit Syndrome

A

Rare disorder with giant hemangiomas (vascular tumors) that are believes to be sequestering platelets from circulation

29
Q

Immune (idiopathic) thrombocytopenia purpura (ITP)

A

autoantibody to self-platelets

30
Q

post transfusion purpura

A

alloantibody to donor platelets

31
Q

neonatal isoimmune purpur a

A

maternal antibody to baby platelets

32
Q

drug induced thrombocytopenia

A

drug bound antibody to self platelets

33
Q

Heparin induced thrombocytopenia and thrombosis

A

abnormal antibodies formed that activate platelets and get used up making non-needed clots.

34
Q

secondary immune platelets destruction disorders

A

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
Q

TTP - Thrombotic Thrombocytopenic Purpura

A

Fibrinolysis inhibited + deficiency in ADADMTS-13, leads to increased platelet aggregation and thrombosis

36
Q

Hemolytic Uremic Syndrome (HUS)

A

Infection by E.Coli O157;H7 or others that lead to platelet destruction, anemia, acute kidney failure.

37
Q

Disseminated Intravascular Coagulation (DIC)

A

Accelerated platelet destruction + coagulation factor consumption (micro thrombi)

38
Q

Thrombocytopenia: increased platelet destruction

Results in:

A

Microangiopathic thrombocytopenia

  • conditions that result in thrombosis (blood clot formation) in small vessels (capillaries and arterioles)
39
Q

Thrombocytosis - PLT count

A

> 450,000 PLT/ul

40
Q

Primary thrombocytosis

A

often due to myeloproliferative disorders

Platelet proliferation is not due to thrombopoietin levels

41
Q

Reactive thrombocytosis

A

Often caused by a reaction to something and can either be transient or chronic

42
Q

acute hemorrhage

A

platelet counts increase after to try to replenish lost

TRANSIENT

43
Q

post splenectomy

A

spleen no longer sequestering platelets

CHRONIC

44
Q

Drugs

A

Epinephrine causes rapid transient increase

45
Q

qualitative disorders of platelet function

A

congenital

46
Q

Platelet membrane problem

A

Bernard soulier syndrome
Glanzmanns thrombasthenia

47
Q

secretion release problem

A

storage pool deficiency
primary secretion defects

48
Q

Bernard Soulier syndrome

A

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
Q

Glanzmann Thrombobasthenia

A

Deficiency of platelet GPIIb/IIIa receptor (needed for fibrinogen bridges)

Deficiency causes abnormal clot retraction –> patient forms loose clot

50
Q

Secretion release problem - storage pool deficiency

A

granule proteins present/absent and platelet morphological appearance

51
Q

Gray-platelet syndrome

A

deficiency of alpha granules with granular appearance of platelets

52
Q

dense granule deficiencies

A

associated with congenital abnormalities including Oculocutaneous albinism

53
Q

hermansky - pudlak syndrome

A

neutropenia, pulmonary fibrosis, prolonged bleeding

54
Q

Chediak Higashi

A

Oculocutaneous albinism + dense granule deficiency + thrombocytopenia

GIANT AZUROPHILIC GRANULES in WBC

55
Q

secretion defects

A
  • due to enzymatic pathway
  • granules are present, but not released properly
  • presentation is similar to storage pool deficiencies
56
Q

Platelet coagulation defects

A

PLT disorders that result in thrombin not being generated

rare!

symptoms close to hemophilia

57
Q

non-immune cause

A

PLT dysfunction by systemic illness or medication

more common than congenital qualitative PLT disorders

58
Q

EX of qualitative PLT disorders

A

Uremia (renal disease)
Liver disease

Paraproteinemias - excess immunoglobin proteins (MM, WM)

Myeloproliferative disorders - PV, idiopathic, myelofibrosis, essential thrombocyothemia

59
Q

Von willebrand disease

A

Most prevalent inherited bleeding disorder worldwide

  • inherited deficiency of vWF
  • often confused with primary PLT disorder
  • missing VIII:vWF
60
Q

vWF disease classified:

A

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
Q
A

Hematology (36 decks)

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