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ACQUIRED COAGULATION ABNORMALITIES

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ACQUIRED COAGULATION ABNORMALITIES ACQUIRED COAGULATION ABNORMALITIES - causes 1. Vitamin K deficiency 2. Liver disease Clotting factor inhibitors: circulating ... – PowerPoint PPT presentation

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Title: ACQUIRED COAGULATION ABNORMALITIES


1
ACQUIRED COAGULATION ABNORMALITIES
2
ACQUIRED COAGULATION ABNORMALITIES - causes
  • 1. Vitamin K deficiency
  • 2. Liver disease
  • Clotting factor inhibitors
  • circulating anticoagulants
  • complications of anticoagulant therapy
  • 4. Incraesed consumption or loss of the
    clotting factors
  • a) disseminated intravascular coagulation (
    DIC)
  • b) fibrinogenolysis (primary
    fibrinolysis)

3
Coagulation abnormalities of vitamin K deficiency
  • vitamin K is essential for the final
    postribosomal carboxylation of F II, VII, IX, X
    and the physiologic anticoagulants, protein C and
    protein S
  • Laboratory features
  • ? PT (prothrombin time) and ? F II, VII, IX, X
  • aPTT (activated partial thromboplastin time) may
    be prolonged in severe, protracted vitamin K
    deficiency
  • Levels of PIVKA-II (Proteins induced in vitamin K
    absence) are more sensitive than PT

4
Vitamin K deficiency-etiology
  • I. Inadequate supply
  • 1. Dietary deficiency (leafy green vegetables
    90-120mcg)
  • 2. Destroying the gut flora by administration
    of broad-spectrum antibiotics
  • II. Impaired absorption of vitamin K
  • 1. Biliary obstruction (gallstone,
    strictures, tumor)
  • 2. Malabsorption of vitamin K(sprue, celiac
    disease, ulcerative colitis)
  • 3. Drugs (cholestyramine)
  • III. Pharmacologic antagonists of vitamin K
    (coumarins, warfarin)

5
Abnormalities of hemostasis and coagulation in
liver diseases (1)
  • I. Decreased synthesis of coagulation factors
  • 1. Fibrinogen, protrombin, clotting F V, VII,
    IX, X, XI, XII, XIII, prekallikrein, high
    molecular weight kininogen
  • 2. Antiplasmins, antithrombin, protein C and
    protein S
  • II. Aberrant biosynthesis
  • 1. Of abnormal fibrinogenu
  • 2. Of abnormal analogues of prothrombin, F
    VII, IX, X

6
Abnormalities of hemostasis and coagulation in
liver diseases (2)
  • III. Deficient clearance
  • 1. Of fibrin monomers, fibrinogen
    degradation products (FDP)
  • 2. Of activated coagulation factors (IXa,
    Xa, Xia)
  • 3. Of plasminogen acivators
  • IV. Accelerated destruction of coagulation
    factors
  • 1. Intravascular coagulation
  • 2. Localized coagulation (hepatic cell
    necrosis)
  • 3. Abnormal fibrinolysis
  • V. Thrombocytopenia and platelet dysfunction
    (splenomegaly)

7
Treatment
  • Vitamin K doses 10mg
  • FFP (invasive procedure)
  • Prothrombin complex concentrates
  • Platelet transfusion
  • Antifibrynolytic agents (dental extraction)

8
Circulating anticoagulants
  • Clotting factor inhibitors are
  • autoantibodies (usually IgG) or
    alloantibodies (in hemophilia A)
  • that inactivate coagulation factors
  • - Laboratory test prolonged aPTT

9
Circulating anticoagulants
  • I. Antibodies to factor VIII (prolonged aPTT,
    normal INR)
  • 1. In hemophilia A
  • 2. Postpartum -several months after
    parturition in asociation with a first pregnancy
  • 3. Various immunologic disorders (rheumatoid
    arthritis, SLE, penicillin allergy)
  • 4. Older patients without underlying disease
  • II. Other spontaneous inhibitors (rarely)-
    against factors V, IX, XIII, fibrinogen,
  • III. Lupus anticoagulant (in 30 SLE, rheumatoid
    arthritis, HIV infection, in lymphoproliferative
    disorders, after drugs hydralazine, quinidine,
    penicillin)

10
Acquired hemophilia A
  • Common bleeding sites are
  • soft tissue, skin, and mucous membrane
  • Treatment Factor VIII bypassing agents
  • Recombinant activated factor VII
  • Plasma-derived factor eight-inhibitor bypassing
    agent
  • (FEIBA, also called activated prothrombine
    complex concentrate)
  • To eradicate the inhibitor is recommended

11
Disseminated intravascular coagulation

DIC
  • is an acquired syndrome characterized by
  • systemic intravascular activation of
    coagulation,
  • leading to fibrin deposition in the
    microvasculature
  • and small-vessels, contributing to organ
    dysfunction
  • consumption of platelets and coagulation factors
  • lead to thrombocytopenia and impaired
    coagulation
  • and may result in bleeding
    complications

12
Clinical conditions that may be complicated by DIC
  • Severe alergic/toxic reaction
  • Obstetrical conditions
  • Amniotic fluid embolism
  • Abruptio placentae
  • HELLP syndrome
  • Solid tumors
  • Sepsis/severe infection
  • Trauma
  • Malignancy
  • Acute leukemias
  • Kasabach-Merritt syndrome
  • Vascular abnormalities

13
ACUTE DIC-CLINICAL PRESENTATION
  • symptoms of underlying disease
  • symptom of local thrombosis
  • hemorrhagic diathesis
  • shock

14


Diffuse intravascular

coagulationMicrothrombosis secondary
fibrinolysis ? platelets


FDP clotting
factors Ischemic tissue damage
Microangiopathic Bleeding

anemia
tendency



15
Acute DIC - laboratory features
  • ? Increased D-Dimer level
  • ? FDP level
  • ? AT level
  • ? platelet level
  • Bload smear - schistocytes
  • ? fibrinogen level
  • ? TT (Thrombin time)
  • ? aPTT
  • ? PT (Prothrombin time)

16
Acute DIC diagnosis
  • The basis of the diagnosis is the knowledge of
    the underlying diseases
  • Patients suffering from acute DIC need urgent
    therapy
  • DIC should always be taken into consideration if
    a complex coagulation defect in combination with
    a underlying disease is observed

17
Diagnostic algorithm for the diagnosis of overt
DIC (1)
  • Risk assessment
  • Does the patient have an underlying disorder
    known to be associated
  • with overt DIC?
  • If yes, proceed

18
Diagnostic algorithm for the diagnosis of overt
DIC (2)
  • - Order global
    coagulation tests
  • Platelet count
  • (gt1000, lt1001, lt502)
  • Elevated fibrin-related markers
  • (FDP no increase0, moderate increase2,
    strong increase3)
  • Prolonged PT
  • (lt3sec. 0, gt3 but lt6 1, gt6sec. 2)
  • Fibrinogen level (gt1g/L0, lt1g/L1)
  • If 5 compatible
    with overt DIC

19
CHRONIC (compensated) DIC
  • In chronic DIC, the activation of the
    hemostatic system is minimal since negative
    feedback mechanisms as well as inhibitors can
    limit the activation process so that microthrombi
    do not occur and bleeding episodes are rare
    phenomena

20
Chronic DIC - etiology
  • 1. Obstetric complications eclampsia, the death
    fetus syndrom
  • 2. Vascular disorders
  • giant hemangiomas (Kasabach Merrit syndrome),
    Leriche
  • syndrome, Raynaud,s disease
  • 3. Carcinomas
  • 4. Hematology disorders myelofibrosis,
    polycythemia vera, PNH
  • 5. Reumathoid disorders SLE, sclerodermia
  • 6. Kidneys disorders glomerulonephritis, HUS
  • 7. Another vasculitis allergica, diabetes
    mellitus

21
PRIMARY FIBRINOLYSIS (FIBRINOGENOLYSIS)
  • DEFINITION
  • primary fibrinolysis occurs when plasmin is
    generated in the absence of DIC
  • ? This has been described in hepatic disorders,
    prostatic carcinomas, and cases without apparent
    cause
  • ? At present, most cases of primary fibrynolysis
    are iathrogenically induced during thrombolytic
    therapy

22


Plasminogen intrinsic
extrinsic
exogenous activation
activation
activationfactor XIa, XIIa, kallikrein
tPA, uPA
streptokinasekininogen

or APSAC PlasminFibrino
gen FibrinFDP FDP
D-Dimer
23
Acquired coagulation abnormalities - diagnostics
  • I History
  • II Physical examination
  • III Laboratory features
  • - morphology
  • - blood smear
  • - bleeding time
  • - prothrombin time (PT), INR
  • - aPTT
  • - thrombin time (TT)
  • - fibrinogen
  • - fibrin(ogen) degradation products (FDP)
  • - D-dimer
  • - antithrombin

24
PT aPTT
Platelet Fibrinogen TT FDP D-Dimer
AT
count Acute
DIC ? ? ?
? ? ?
? ? Chronic DIC
N ? N ? N ? N ?
? N ? ? ?
? Fibrinogenolysis N ?
? N ? ?
N N N
Heparin overdosage ? ?
N N N
N N N Dicumarol
? N? N
N ? N
N N
overdosage or


prothrombin complex


factors defficiency
Diferentiation of aquired coagulation
abnormalities
25
ACA DIC THERAPY
  • 1. Treatment of the underlying disorder
  • 2. Treatment of shock
  • 3. Replacement therapy
  • - platelet concentrates
  • - RBC
  • - FFP
  • - Cryoprecipitate (fibrinogen)
  • - Activated protein C (drotrecogin alfa)
  • Heparin treatment
  • unfractioned heparin or low-molecular
    weight heparin
  • acrocyanoza, purpura fulminans, dermal
    necrosis, venous thromboembolism

26
Treatment thrombosis predominantes
  • Continous infusion of UFH
  • Prophilactic doses of heparin or LMWH
  • Especially, severe purpura fulminans,
  • acral ischemia, vascular skin infarction

27
Treatment - bleedings
  • Transfusion of platelets or plasma (components)
    including FFP and/or prothrombin complex
    concentrate (fluid overload)
  • Severe hipofibrynogeneamia (lt1g/L)
  • FFP, fibrionogen concentrate and
    cryopercipitate

28
CASE PRESENTATION
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