DICIt is not a primary disease entity but rathera pathologic process secondary to anunderlying illness.Most features of DIC are attributable to thegeneration of thrombin or a thromboplasticsubstance and plasmin from a variety ofinciting events.The levels of protein C and antithrombin IIIare 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
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 bleeding From 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-Dimer Tx: Antifibrinolytic therapy with e-aminocaproic acid (EACA) or trans-p-aminomethyl-cyclohexane carbolyxix acid (AMCA).
HELLP Syndrome Pregnant women Severe preeclampsia Microangiopathic hemolytic anemia Elevated liver enzymes Low platelets (Thrombocytopenia) Elevated D-Dimer Tx: Delivering the fetus, FFP, Plasmapheresis.
Thrombotic ThrombocytopenicPurpura (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 TTPMost of the cases: Unknown Obstetric 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: Vincristine Immunoglobulin Aspirin + Persantine Splenectomy