disseminated intravascular coagulation (dic). (defibrination syndrome) it is a consumptive...
TRANSCRIPT
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Disseminated intravascular coagulation
(DIC)
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(defibrination syndrome)
It is a consumptive coagulopathy
that is caused by a wide variety of serious disorders.
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Etiology
*Infections --- G-ve bacterial sepsis
--- Other bacteria, fungi, Rocky Mountain spotted fever, viruses, malaria
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Obstetric complications
---Amniotic fluid embolism
---Retained dead fetus
---Abruptio placentae
---Toxemia of pregnancy
---Septic abortion
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Malignancies
----Pancreatic Ca
---Adenocarcinomas
---Acute promyelocytic leukemia )M3(
---Other neoplasms
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OTHERS
*Liver failure
*Acute pancreatitis
*Snake venom
*ARDS
*Blood transfusion reaction
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Trauma
---- Shock
--- Brain injury
--- Crush injury
--- Burns
--- Hypothermia/ Hyperthermia
--- Fat embolism, ischemia, hypoxia
--- Surgery
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Vascular disorders
---Giant hemangiomas )Kassabach-Merritt syndrome(
---Vascular tumors, Aortic aneurysms
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Pathogenesis
It is primarily a thrombotic process although its clinical manifestations may be widespread hemorrhage .
The basic trigger irrespective of etiology is entry into the circulation of procoagulant substances activating coagulation factors & platelets leading to disseminated deposition of fibrin-platelet thrombi. In most cases the procoagulant stimulant is tissue factor )a lipoprotein that is not normally exposed to blood(, mucin in certain malignancies, proteases in
other malignancies, pancreatitis, and envenomation.
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S&S
Asymptomatic --- low grade DIC showing only Lab abnormalities
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Thrombotic complications
---Trousseau's syndrome
--- Gangrene of digits & extremities
--- Hemorrhagic necrosis of skin
--- Purpura fulminans
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Bleeding
---most common manifestation in acute cases
---Generalized & widespread ---Characteristically from the cannula
site --- oozes from mucosal surfaces &
orifices Clinical manifestations of the causative disease
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Dx
PT, aPTT & TT are prolonged
Thrombocytopenia ,
Plasma Fibrinogen ↓
FDPs ↑ )measured by latex agglutination or D-dimer assay(
Blood film --- Schistocytes, fragmented RBCs
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DDx
---Coagulopathy of liver failure
---TTP, HUS
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℞ •Identify & correct the cause
•No treatment --- asymptomatic cases )not ttt lab manifestations(
•Hemodynamic support •Blood component therapy --- FFP, platelets, cryoppt
•Drugs --- Inhibitors of coagulation & fibrinolysis • Heparin – theoretic benefit )if thrombosis
predominates( • -- may exacerbate bleeding
• Antifibrinolytics – EACA & tranexamic acid • -- Generally C/I in DIC
• -- may be useful in life-threatening bleeding
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Thrombotic Thrombocytopenic Purpura )TTP(
A rare disease characterized by a clinical triad of severe thrombocytopenia, Microangiopathic hemolytic anemia, &
Neurologic abnormalities.
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Renal involvement
proteinuria, hematuria, RBC casts & azotemia that is usually mild & remits on ttt
Fever is a prominent feature
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Pathology
involved organs show capillaries & arterioles occluded by hyaline material )platelet thrombi( & fibrin deposit in
blood vessel wall.
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Symptoms typically wax & wane )due to platelet aggregation & disaggregation(
Fluctuating neurological abnormalities
Association with pregnancy & oral contraceptive
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Dx
anemia, thrombocytopenia & Microangiopathic changes of RBCs )Schistocytes( in the absence of other
disorders
--- Minimal changes in coagulation tests
• --- Biopsy Skin, gum, or BM
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DDx
•DIC, HUS, SLE & Evan's syndrome
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:℞
•Untted pt dies within 3 months
•Plasmapheresis --- ttt of choice, cure 70%
• --- continued until plat count becomes normal
•Chronic & relapsing form is seen in 10% pts whose plasma contains large multimers of vWF
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Hemolytic Uremic syndrome )HUS(
A disease of infants & children rarely occurs in adults
Microangiopathic hemolytic anemia, with mild to moderate thrombocytopenia & NO Neurologic abnormalities
Acute renal failure is a prominent feature
Severe hypertension is a prominent feature
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GI S&S – abdominal pain, & diarrhea
Also seen in women in postpartum period or on oral contraceptives
Also seen in pts with cancer receiving mitomycin or cisplatin chemotherapy
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Von Willebrand disease
AD inheritance
The most common bleeding disorder
1-3% of population
Mild bleeding tendency after trauma or surgery
Bleeding from mucosae
Easy bruising
Bleeding time prolonged
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℞
•Cryoppt
•Desmopressin
•Platelet transfusion
•EACA
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