DISSEMINATED INTRAVASCULAR COAGULOPATHYand OTHERS

Raúl H. Morales-Borges, MD, FICPS, FIACATHAshford Institute of Hematology & Oncology, P.S.C.

Coagulation Cascade

Coagulation Cascade

DIC

It is not a primary disease entity but rather a pathologic process secondary to an underlying illness.

Most features of DIC are attributable to the generation of thrombin or a thromboplastic substance and plasmin from a variety of inciting events.

The levels of protein C and antithrombin III are frequently low in DIC.

Definition of DIC

Widespread and ongoing activation of coagulation.

Leading to possible vascular or micro vascular fibrin deposition.

Compromising and inadequate blood supply to various organs.

Decreasing levels of procoagulant proteins.

Clinical Conditions Associated

Sepsis.

Trauma.

Organ Destruction (pancreatitis).

Malignancy.

Obstetrical.

Vascular abnormalities.

Severe hepatic failure.

Cont. Associated Conditions

Severe toxic or immunogical reactions.

snake bites

recreational drugs

transfusion reactions

transplant rejection

Pathogenesis

Microvascular fibrin deposition, tissue factor dependant.

Extrinsic pathways.

Sepsis/Endotoxemia generates tissue factor-FVII thrombin generation.

DIC

DIC

Amplification of Fibrin deposition.

Defective Physiological Anticoagulant systems.

Laboratories

PT / PTT

CBC

Fibrinogen

D-Dimer

Peripheral smear

Diagnosis

Treatment

Treat the underlying disorder.Plasma (FFP), Cryoprecipitate, and Platelet transfusions only, if risk of bleeding.Invasive procedures.Anticoagulants like: Inact FVIIa, Recombinant T.F. path. Inh. Recombinant NAPc2. are investigational.

Another modalities of Tx of DIC

Heparin for prevention of microthrombosis and organ damage

Antifibrinolytic (EACA) agents for hemorrhage despite above measures

Antithrombin III and/or Protein C concentrates when low levels found (?)

Use of Heparin

It is controversial.

Used in combination with EACA for special cases.

It’s used in Trousseau syndrome, purpura fulminans, and certain obstetric complications. Also in AML-M3.

Systemic Hyperfibrinolysis

Spontaneous bleedingFrom acute states such as heatstroke, hypoxia, hypotension, thoracic surgery, administration of thrombolytics, and neoplasms.Also found in Extracorporeal bypass, congenital alpha2-antiplasmin deficiency, and Liver transplant. Lab’s: decreased plasminogen and fibrinogen, elevated D-DimerTx: Antifibrinolytic therapy with e-aminocaproic acid (EACA) or trans-p-aminomethyl-cyclohexane carbolyxix acid (AMCA).

HELLP Syndrome

Pregnant womenSevere preeclampsia Microangiopathic hemolytic anemiaElevated liver enzymesLow platelets (Thrombocytopenia)Elevated D-DimerTx: Delivering the fetus, FFP, Plasmapheresis.

Thrombotic Thrombocytopenic Purpura (TTP)

Appears abruptly in previously completely normal healthy individuals.Pentad: Thrombocytopenia, Microangiopathic hemolytic anemia, Neurologic abnormalities, Abnormal Renal function, and Fever.Most of cases are women, white race.LDH is very high.Mortality is high within days.The 2 organs relatively spared by the vascular thrombi are the lungs and liver.

Causes of TTP

Most of the cases: UnknownObstetric emergencies

Infectious diseases

Drugs and Toxins

Neoplastic diseases receiving cytotoxic therapy

Auto-immune mediated diseases

Hemolytic Uremic Syndrome (HUS)

Most common in children

Associated with E. coli & Campylobacter jejuni.

Similar to TTP, but we see more kidney damage.

Tx of TTP / HUS

Corticosteroids

Plasmapheresis / exchange

Tx of the underlying disease

The goal is to achieve:LDH < 500 u/dl

Normal Platelets

Normal Hgb / Hct

Outcome of TTP / HUS

Relapse is common within the first month after the initial diagnosis (85%). At 2 months the relapse is in 15%.

Relapse after 5 years is extremely rare.

If patients do not respond to 1st line therapy, then use:

VincristineImmunoglobulinAspirin + PersantineSplenectomy

Gracias !

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