DISORDERS OF PLATELETS AND CLOTTING FACTORS

HEMORRAGIC DISORDER
Normal clot formation and lysis depend on
1. Intact blood vessels
2. an adequate number of functioning platelets
3. sufficient amounts of the 12 clotting factors (I to XIII; VI is no longer used)
4. a well-controlled fibrinolytic system
Four basic problems underlying hemorrhagic disorders
weak, damaged vessels that rupture easily or spontaneously
platelet deficiency (thrombocytopenia) resulting from:
o hypoproliferation
o excessive pooling of platelets in the spleen
o excessive platelet destruction
deficiency or total lack of one of the clotting factors
excessive or insufficient fibrinolysis
A. Immune Thrombocytopenic Purpura (ITP)
Most common thrombocytopenic disorder
2 forms
o Acute
Appears 1-6 weeks after a viral illness; self-limiting; remission occurs
within 6 months
Occurs predominantly in children
o Chronic
Other causes of thrombocytopenia
Was previously called Idiopathic Thrombocytopenic Purpura (cause was unknown)
Now considered an autoimmune disease that results in the destruction of platelets
RISK FACTORS
Viral infections
Sulfa-containing drugs, quinine
ETIOLOGY
Acquired disorder in which circulating platelets are destroyed by autoantibodies that
bind with antigens on the plaletet membrane
Platelets normal lifespan (7-10 days); with ITP 1-3 days
DX
PLT < 100,000/mm3
Prolonged bleeding time with normal coagulation time (all coagulation factors are
present & normal)
Increased capillary fragility as demonstrated by the tourniquet test
Positive platelet antibody screening
Bone marrow aspirate containing normal or increased number of megakaryocytes
(platelet precursors)
S/S/X
Petechiae (tiny hemorrhagic spots caused by intradermal or submucosal bleeding)
Ecchymosis (large, blotchy subcutaneous hemorrhagic areas)
Epistaxis
Bleeding from gums
Easy bruising
Heavy menses
COMPLICATIONS
Spontaneous cerebral hemorrhage
Severe hemorrhages from the nose, GIT & urinary system

 Bleeding into the diaphragm

Nerve pain resulting from pressure of hematomas on nerves
MX MNGT
High doses of corticosteroids inhibit the macrophage ingestion of the antibodycoated platelets
Plasmapheresis used as a short-term therapy until steroid therapy takes effect
If client is actively bleeding/requires surgery IV gammaglobulin to increase the
platelet count
Splenectomy
Immunosuppressive therapy blocks the binding receptors on macrophages so that
the platelets are not destroyed
o Vincristine, vinblastine, azathioprine, cyclophosphamide
NX MNGT
Avoid constipation, valsalva maneuver
Avoid flossing of the teeth
Use of soft-bristled toothbrush & electric razors
Refrain from vigorous sexual intercourse when PLT is < 10,000/mm3
COAGULATION DISORDERS
A. Disseminated Intravascular Coagulation
Complex syndrome of activated coagulation that results in bleeding and thrombosis
simultaneously.
BASICALLY a loss of balance between the clotting & lysing systems in the body
caused by the simultaneous presence of THROMBIN & PLASMIN
THROMBIN tips the balance toward the prothrombin state resulting in
thrombosis
PLASMIN triggers excessive clot lysis (fibrinolysis) in which clotting factors are
consumed are consumed to such an extent that generalized bleeding occurs
RISK FACTORS
Cancer (especially prostate cancer and leukemia)
Obstetric complications
Acute hemolysis; Allergic reactions
Trauma
Shock; Sepsis
ETIOLOGY (Four categories of causative factors)
Infection leading cause
Tissue coagulation factors traveling into the circulation
Damage to the vascular endothelium
Stagnant blood flow
DX
Prolonged PT & PTT
PLT (<100,000/mm3)
Fibrinogen degradation product (FDP) elevated (75%-100%)
D-dimer test
S/SX
Hemorrhagic manifestations:
o Purpura, ecchymosis & Petechiae on the skin, mucous membrane, heart lining
& lungs
o Prolonged bleeding from venipucture
o Severe, uncontrolled hemorrhage during surgery or childbirth
o Excessive bleeding from gums & nose
o Intracerebral & GI bleeding
o Renal hematuria

o Tachycardia & hypotension

o Dyspnea, hemoptysis & respiratory congestion
Microvascular thrombosis manifestations
o Oliguria & ARF
o Pulmonary emboli
o ARDS
o Delirium, convulsion & coma
o Tissue necrosis
PATHO
Precipitating factor

Tx underlying prob.

Tissue damage (e.g.

burns/trauma)

Endothelial
damage

tissue
thromboplastin

Intrinsic pathway of
coagulation

Extrinsic pathway of
coagulation
heparin

Occlusion of
small blood
Tissue necrosis

Intravascular coagulation
Prothrombin thrombin
fibrinogen fibrin
FFP, PLT
Cryoprecipitate

Production of
thrombi

Consumption of clotting
factors

Activation of fibrinolytic
system
Digestion of fibrin
clots

clotting
factors

Thrombocytop
enia

Infiltration of platelet
function
Bleedin
g

TEST
Platelet count

FUNCTION EVALUATED
Platelet number

PT
PTT
TT
Fibrinogen

Extrinsic pathway
Intrinsic pathway
Clot formation
Amount available for
coagulation
Local fibrinolysis
Fibrinolysis

D-dimer
Fibrin
degradation
products (FDPs)
MNGT

NORMAL RANGE
150,000450000/mm3
11-12.5 sec
23-35 sec
8-11 sec
170-340 mg/dl
0-250 ng/ml
0-5ug/ml

CHANGES IN DIC

Goals
o Identification & correction of the precipitating cause
Antibiotics for infection
o Reestablishing hemostasis by replacing missing blood components
o Supportive therapy to control hemorrhage & thrombosis
Avoid injections when possible
Apply pressure to bleeding sites
Turn & reposition client frequently & gently
B. Hemophilia
X-linked genetic disorder that results in a deficiency of coagulation factors
2 major forms
o Hemophilia A (classic form) deficiency of factor VIII; X-linked
o Hemophilia B (Christmas disease) deficiency of factor IX; X-linked
o Von Willebrands disease deficiency of factor VIII & defective platelet
dysfunction; autosomal dominant trait
S/SX
Hemarthrosis hallmark of the disease
Slow, persistent bleeding from cuts, trauma
Severe hemorrhaging from the gums after dental extraction
Epistaxis
GI bleeding
Hematoma
Hematuria
Intracranial bleeding
MNGT
Goals
o Stop topical bleeding as quickly as possible
Apply pressure or ice to the injured site
Packing the area with fibrin foam
Applying topical hemostatic agents such as thrombin
o Supply the missing factor causing hemorrhage
Blood transfusion
o Prevent complications leading to & caused by bleeding
Hemarthrosis
Joint immobilization
Ice pack
If pain is severe may aspirate blood from joints
Once bleeding stops
o Perform active ROM without weight-bearing
Pharmacological tx
o Aminocaproic acid (EACA, Amicar) fibrinolytic enzyme inhibitor that can
slow the dissolution of blood clots; useful in treating mucosal bleeding
o Desmopressin (DDVAP) induces a transient rise in factor VIII levels