Disseminated intravascular
coagulation
(DIC)
(defibrination syndrome)
It is a consumptive coagulopathy
that is caused by a wide variety of
serious disorders.
Etiology
* Infections --- G-ve bacterial sepsis
--- Other bacteria, fungi, Rocky
Mountain spotted fever, viruses,
malaria
Obstetric complications
--- Amniotic fluid embolism
--- Retained dead fetus
--- Abruptio placentae
--- Toxemia of pregnancy
--- Septic abortion
Malignancies
---- Pancreatic Ca
--- Adenocarcinomas
--- Acute promyelocytic leukemia (M3)
--- Other neoplasms
OTHERS
* Liver failure
* Acute pancreatitis
* Snake venom
* ARDS
* Blood transfusion reaction
Trauma
---- Shock
--- Brain injury
--- Crush injury
--- Burns
--- Hypothermia/ Hyperthermia
--- Fat embolism, ischemia, hypoxia
--- Surgery
Vascular disorders
--- Giant hemangiomas (KassabachMerritt syndrome)
--- Vascular tumors, Aortic aneurysms
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.
S&S
Asymptomatic --- low grade DIC showing
only Lab abnormalities
Thrombotic complications
--- Trousseau's syndrome
--- Gangrene of digits & extremities
--- Hemorrhagic necrosis of skin
--- Purpura fulminans
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
Dx
PT, aPTT & TT are prolonged
Thrombocytopenia,
Plasma Fibrinogen ↓
FDPs ↑ (measured by latex agglutination
or D-dimer assay)
Blood film --- Schistocytes, fragmented
RBCs
DDx
--- Coagulopathy of liver failure
--- TTP, HUS
℞
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
Thrombotic Thrombocytopenic
Purpura (TTP)
A rare disease characterized by a clinical
triad of severe thrombocytopenia,
Microangiopathic hemolytic anemia, &
Neurologic abnormalities.
Renal involvement
proteinuria, hematuria, RBC casts &
azotemia that is usually mild & remits
on ttt
Fever is a prominent feature
Pathology
involved organs show capillaries &
arterioles occluded by hyaline material
(platelet thrombi) & fibrin deposit in
blood vessel wall.
Symptoms typically wax & wane (due to
platelet aggregation & disaggregation)
Fluctuating neurological abnormalities
Association with pregnancy & oral
contraceptive
Dx
anemia, thrombocytopenia &
Microangiopathic changes of RBCs
(Schistocytes) in the absence of other
disorders
--- Minimal changes in coagulation
tests
--- Biopsy Skin, gum, or BM •
DDx
DIC, HUS, SLE & Evan's syndrome •
℞:
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
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
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
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
℞
Cryoppt •
Desmopressin •
Platelet transfusion •
EACA •