Disorders Of Primary Hemostasis

Front 1

Marfan Syndrome

Back 1

Hereditary vascular defect; decrease in strength and elasticity of blood vessels

Long limbs, easy bruising, excessive bleeding

Front 2

Hereditary Hemorrhagic Telangiectasis

Back 2

Hereditary vessel defect; dilations of postcapillary venules in the dermis

Nosebleeds, GI bleeding

Front 3

Ehlers-Danlos Syndrome

Back 3

Hereditary vessel defect; characterized by defects in collagen production, leading to reduced collagen

Affects skin, ligaments, joints, and blood vessels

Skin is now thin and elastic

Front 4

Vascular System Disorders

Back 4

Structural abnormality

Normal PT and PTT, prolonged bleeding time due to damage to the lining of the blood vessels

Front 5

Scurvy/Vitamin C Deficiency

Back 5

Acquired vessel defect

Vitamin C needed for collagen synthesis

Bruising, petichiae, and bleeding gums

Front 6

Infections

Back 6

Acquired vessel defect

Can activate complement and lead to vascular permeability due to neutrophil migration to infection site

Front 7

Allergies

Back 7

Acquired vessel defect

Antigen/antibody complexes can accumulate and cause platelet aggregation, activating Factor XII in the coagulation cascade, leading to thrombosis and eventual necrosis of the tissues

Front 8

Mechanical Stress

Back 8

Acquired vessel defect

Caused by intense exercise, coughing fits, and spasms

Front 9

Paraproteinemia

Back 9

Acquired vessel defect

Associated with multiple myeloma and an overabundance of proteins

Front 10

Amyloidosis

Back 10

Acquired vessel defect

Amyloid from bone marrow deposited in tissues; leads to protein buildup

Front 11

Normal platelet count

Back 11

~150,000/uL

150-450 x 10^9/L

Front 12

Platelets < 50,000/uL

Back 12

Petichiae and spontaneous bruising

Front 13

Platelets < 20,000/uL

Back 13

Oncologists/physicians start to get worried

Front 14

Platelets < 10,000/uL

Back 14

Severe, spontaneous bleeding

Bleeding time and platelet studies aren't very accurate because there aren't enough platelets

Front 15

Thrombocytopenia Test Results

Back 15

Abnormal bleeding time

Normal PT, PTT, D-Dimer, and FDP (as long as there's no DIC)

Front 16

Causes of Thrombocytopenia

Back 16

Pseudothrombocytopenia, platelet satellitism, increased destruction, decreased bone marrow production, increased use, hypersplenism, massive transfusions, alcohol abuse

Front 17

Pseudothrombocytopenia

Back 17

Low platelet count, but not necessarily from thrombocytopenia

Hypercoaguable patients/patients who clump in EDTA are redrawn in a prewarmed blue top tube

Front 18

Increased Immune Platelet Destruction

Back 18

Heparin-induced antibodies and AIDS

Front 19

Increased Nonimmune Platelet Destruction

Back 19

Heparin, drug induced

Front 20

Decreased Bone Marrow Platelet Production

Back 20

Chemotherapy, decreased thrombopoietin, leukemias, bone marrow injury, bone marrow replacement with different materials

Front 21

Increased use of Platelets

Back 21

DIC

Front 22

Hypersplenism and Thrombocytopenia

Back 22

Platelet sequestration, overactive spleen, and splenomegalia

Front 23

Thrombocytopenia and Massive Transfusions

Back 23

This can happen if packed RBCs are the only component used

Front 24

Thrombocytopenia and Alcohol Abuse

Back 24

Ethanol can lead to hepatic cirrhosis

Front 25

Immune Thrombocytopenia (ITP)

Back 25

Most common form of thrombocytopenia, usually seen in children following a viral infection

Antibodies from the B-lymphs bind to or coat the platelets and cause problems

Diagnosis based on exclusion

Mild to moderate bleeding, bruising, and petichiae

Main cause of death is intracranial hemorrhage

Front 26

Acute ITP

Back 26

Self-limiting

Mostly seen in children between ages 5-6 after a viral infection

Mild to moderate bleeding, bruising, and petichiae

Front 27

Chronic ITP

Back 27

Usually seen in middle-aged women

Diagnosis depends on patient history, exam, signs and symptoms, and exposure

Front 28

ITP Treatment

Back 28

Usually not required for children

Corticosteroids, IVIg, splenectomy, Rituximab (Anti-CD20 that destroys B-lymphs), chemotherapy, immunosuppressive therapy

Front 29

Heparin-Induced Thrombocytopenia (HIT)

Back 29

Immune mediated destruction of platelet

Heparin binds to PF4 and stimulates the immune system to produce an antibody

The patient may have a normal platelet count before the administration of unfractionated heparin

Front 30

Thrombocytosis

Back 30

Increased platelet count above the reference range

> 450 x 10^9/L

Front 31

Causes of Thrombocytosis

Back 31

Increased platelet production in bone marrow, post-splenectomy, myeloproliferative disorders

Front 32

Increased Platelet Production in Bone Marrow

Back 32

Can be caused by acute blood loss or hemolysis, IDA, some tumors, and acute infections

Front 33

Thrombocytosis Post-Splenectomy

Back 33

All platelets are now in circulation rather than 1/3 being stored in the spleen

Front 34

Thrombocytosis and Myeloproliferative Disorders

Back 34

Disease states

Abnormal platelet functions and bleeding times

Primary Polycythemia Vera

Front 35

Primary Thrombocytosis

Back 35

Megakaryocytic proliferation and maturation that bypasses normal mechanisms

Uncontrolled production of platelets

Front 36

Primary Thrombocytosis Range

Back 36

> 1000 x 10^9/L

Giant, bizarre platelets

Front 37

Disease States of Primary Thrombocytosis

Back 37

CML, Polycythemia Vera, refractory anemia with ringed sideroblasts, essential thrombocythemia (ET)

Front 38

Essential Thrombocythemia (ET)

Back 38

Over 1 million giant, bizarre platelets

Patients may suffer spontaneous hemorrhage due to abnormal platelet function

Front 39

ET Lab Results

Back 39

Abnormal platelet function and platelet aggregation studies

Normal PT and PTT

Front 40

Secondary (Reactive) Thrombocytosis

Back 40

An increase in platelets by normal mechanisms

Returns to normal by treating the underlying condition

Front 41

Secondary (Reactive) Thrombocytosis Range

Back 41

< 1000 x 10^9/L (1 million)

Front 42

Causes of Secondary (Reactive) Thrombocytosis

Back 42

Acute hemorrhage, surgery, post-splenectomy, IDA, hemolytic anemia, alcohol therapy (recovering alcoholic), chemotherapy, post-partum thrombocytosis, vigorous exercise

Front 43

Secondary (Reactive) Thrombocytosis Lab Results

Back 43

Normal bleeding time and platelet aggregation studies

Normal PT and PTT

Front 44

Qualitative Platelet Disorders

Back 44

Problems with platelet adhesion, aggregation, contraction, release of ADP, generation of thromboxane A2

Front 45

Clinical Symptoms of Qualitative Platelet Disorders

Back 45

Easy and spontaneous bruising, petichiae, mucosal bleeding, prolonged bleeding from trauma, prolonged bleeding time

Front 46

Qualitative Platelet Disorder Range

Back 46

Normal platelet count

150-450 × 10^9/L

May have bizarre or giant platelets

Front 47

Qualitative Platelet Disorder Lab Results

Back 47

Altered platelet function leads to prolonged bleeding time

Front 48

Hereditary Qualitative Platelet Disorders

Back 48

VonWillebrands, Bernard Soulier, Glanzmann's Thrombasthenia

Front 49

Acquired Qualitative Platelet Disorders

Back 49

Myeloproliferative disorders, renal failure, DIC, aspirin

Front 50

Bernard-Soulier Syndrome

Back 50

Problem with the adhesion of platelet to collagen due to the lack of GPIb/IX, which binds vWF

Rare, autosomal-recessive disorder

"Giant platelet syndrome"

Front 51

Bernard-Soulier Lab Findings

Back 51

Abnormal platelet morphology

Prolonged bleeding time

Normal PT and PTT

Normal or slightly decreased platelet count

Normal platelet aggregation studies with ADP, collagen, and epinephrine

Abnormal platelet aggregation studies with ristocetin and vWF

Flow cytometry for CD42b/CD42a

Front 52

Therapy for Bernard-Soulier

Back 52

RBC and platelet transfusions

Front 53

vonWillebrand Disease

Back 53

Autosomal dominant disorder

The main hereditary platelet disorder seen among males and females

Affects both primary and secondary hemostasis

Decreased or abnormal form of vWF, which carries factor VIII (which is why it affects both primary and secondary hemostasis)

Front 54

Clinical Findings of vW Disease

Back 54

Superficial or mucosal bleeding, nosebleeds, heavy menses, petichiae

Front 55

Laboratory Findings of vW Disease

Back 55

Abnormal bleeding time due to adhesion problems

Abnormal platelet aggregation with ristocetin ONLY

Variable PTT because vWF carries Factor VIII, differentiating it from Hemophilia A

Front 56

Therapy for vW Disease

Back 56

DDAVP (desamino D-vasopressin)

Cryoprecipitate is old-school treatment

Front 57

Glanzmann's Thrombasthenia

Back 57

Rare platelet aggregation disorder

Defective GPIIb/IIIa; can't form the bridge for fibrinogen between the platelets

Defect of thrombasthenin or actomysin that causes abnormal platelet retraction

Front 58

GT Type I

Back 58

Subtype of Glanzmann's Thrombasthenia

No detectable levels of glycoproteins

Front 59

GT TYPE II

Back 59

Subtype of Glanzmann's Thrombasthenia

~15% glycoproteins

Front 60

GT TYPE III

Back 60

Subtype of Glanzmann's Thrombasthenia

50-100% glycoprotein, but still have clinical features associated with Glanzmann's

Front 61

Laboratory Findings of Glanzmann's Thrombasthenia

Back 61

Normal platelet count and morphology

Prolonged bleeding time

Abnormal clot retraction

Absent or severely diminished platelet aggregation studies with ADP, collagen, and epinephrine

Front 62

Therapy for Glansman's Thrombasthenia

Back 62

Platelet transfusions

Rarely, bone marrow transplants

Front 63

Platelet Storage Pool Disease

Back 63

Decrease of platelet dense granules

Deficient or abnormal release of ADP to cause abnormal platelet aggregation

Platelets appear normal on a Wright's stain

Front 64

Clinical Findings of Platelet Storage Pool Disease

Back 64

Frequent nosebleeds, easy bruising, heavy menstruation

Front 65

Lab Findings of Platelet Storage Pool Disease

Back 65

Prolonged bleeding time

Abnormal platelet aggregation

Front 66

Platelet Storage Pool Disease Deficiencies

Back 66

Seen in Chediak-Higashi and Wiscott-Aldrich

Front 67

Gray Platelet Syndrome

Back 67

Deficiency of alpha granules

Front 68

Lab Findings of Gray Platelet Syndrome

Back 68

Platelets appear agranular on Wright's stain

Prolonged bleeding time, usually following dental surgery or minor trauma

Normal platelet aggregation studies

Mild thrombocytopenia

Front 69

Defective Thromboxane A2 Synthesis

Back 69

Similar to storage pool disease

Abnormalities in platelet secretion

Arachidonic acid is not released from phospholipids, so there is no conversion of Thromboxane A2 to enhance platelet activation

Can be a mild bleeding disorder

Front 70

Acquired Disorders of Platelet Function

Back 70

Chronic renal failure (accumulation of waste products in the blood), drugs, multiple myeloma, DIC, essential thrombocythemia (ET)

Front 71

Drugs That can Cause Acquired Disorders of Platelet Function

Back 71

Aspirin (inactivates cyclooxygenase and prevents release of Thromboxane A2)

Alcohol (large amounts over long periods of time cause platelet dysfunction)

Antibiotics (penicillins and cephalosporins alter platelet function; Plavix prevents platelet aggregation; antibiotics can block ADP and epinephrine receptors)