Differences in the Clinical Manifestations of Disorders of Primary and Secondary Hemostasis

Manifestations Defects of Primary Hemostasis

(Platelet Defects)
Defects of Secondary Hemostasis (Plasma Protein
Defects)
Onset of bleeding after trauma Immediate Delayed (hours or days)
Sites of bleeding Superficial skin, mucous membranes, nose, gastrointestinal
and genitourinary tracts
Deep (joints, muscle, retroperitoneum)
Physical findings Petechiae, ecchymoses Hematomas, hemarthroses
Family history Autosomal dominant Autosomal or X-linked recessive
Response to therapy Immediate; local measures effective Requires sustained systemic therapy








EXPLORAREA HEMOSTAZEI PRIMARE
TESTELE DE SCREENING :
Timpul de sangerare - TS
Numararea placutelor sanguine






Primary Hemostatic (Platelet) Disorders
1. Defects of platelet adhesion
- von Willebrand's disease
- Bernard-Soulier syndrome (absence or dysfunction of GpIb/IX)
2. Defects of platelet aggregation
- Glanzmann's thrombasthenia (absence or dysfunction of GpIIb/IIIa)
3. Defects of platelet release
Decreased cyclooxygenase activity
- Drug-induced (aspirin, nonsteroidal anti-inflammatory agents)
- Congenital
Granule storage pool defects
- Congenital
- Acquired
Uremia
Platelet coating (e.g., penicillin or paraproteins)
4. Defect of platelet coagulant activity
- Scott's syndrome








Evaluation of Platelet Function
Bleeding time
Modified Ivy method
Skin incision (time to stop bleeding)
Global screen of platelet role in hemostasis
von Willebrand factor assays
vWF Ag (immunoassay of total vWF protein)
vWF R:Cof (bioassay of vWF that measures ability of patient plasma to support agglutination of normal platelets in the presence of ristocetin)
Factor VIII (coagulation assay of factor VIII bound and carried by plasma vWF)
Platelet aggregometry
Measures platelet aggregation in response to a panel of agonists, usually ADP, collagen, arachidonic acid, and epinephrine
Membrane glycoproteins
Presence of glycoproteins Ib-IX and IIb-IIIa can be measured using monoclonal antibodies and flow cytometry
Platelet granule content
Dense granules (electron microscopy or uptake and retention of radiolabeled serotonin)
Alpha granules (electron microscopy and/or immunoassays for platelet-associated proteins - vWF, fibrinogen, platelet factor four



EXPLORAREA HEMOSTAZEI SECUNDARE (COAGULAREA)
TESTE DE SCREENING :
TCG TC (aPTT)
TIMPUL DE PROTROMBINA (QUICK) = PT
TIMPUL DE TROMBINA
FIBRINOGENEMIA

Relationship between Secondary Hemostatic Disorders and Coagulation Test Abnormalities
Prolonged partial thromboplastin time (PTT)
No clinical bleeding (factors XII, HMWK, PK)
Mild or rare bleeding (factor XI)
Frequent, severe bleeding (factors VIII and IX)
Prolonged prothrombin time (PT)
Factor VII deficiency
Vitamin K deficiency - early
Warfarin anticoagulant ingestion
Prolonged PTT and PT
Factor II, V, or X deficiency
Vitamin K deficiency - late
Warfarin anticoagulant ingestion
Prolonged thrombin time (TT)
Mild or rare bleeding, afibrinogenemia
Frequent, severe bleeding, dysfibrinogenemia
Heparin-like inhibitors or heparin administration
Prolonged PT and/or PTT not corrected with normal plasma
Specific or nonspecific inhibitor syndromes
Clot solubility in 5 M urea
Factor XIII deficiency
Inhibitors or defective cross-linking
Rapid clot lysis
a2 plasmin inhibitor



Dg. diferential : deficit de FPC / deficit de VK
TESTUL KOLLER
DEFICIT FPC DEFICIT DE VK
APTT / PT APTT / PT

Administrare de VK (i.m.)
8 12 ore
APTT / PT (nu se corecteaza) APTT / PT = N (se corecteaza)
TEST KOLLER (-) TEST KOLLER (+)


EXPLORAREA FIBRINOLIZEI
TLCS
TLCE
D-DIMER


SEMNIFICATIA D-DIMERULUI

SINDROM FIBRINOLITIC SINDROM FIBRINOLITIC
PRIMAR SECUNDAR

APTT = N APTT
PT = N PT
TT = N TT
Fibrinogenemie (disproportionat) Fibrinogenemie
Tr-citopenie Tr-citopenie
TLCS, TLCE TLCS, TLCE
PDF +++ PDF +++
D-Dimer = N D-Dimer