1. Disseminated intravascular coagulation
(DIC)

2. (defibrination syndrome)
It is a consumptive coagulopathy
that is caused by a wide variety of serious disorders.

3. Etiology * Infections --- G-ve bacterial sepsis
--- Other bacteria, fungi, Rocky Mountain spotted fever, viruses, malaria

4. Obstetric complications
--- Amniotic fluid embolism
--- Retained dead fetus
--- Abruptio placentae
--- Toxemia of pregnancy
--- Septic abortion

5. Malignancies ---- Pancreatic Ca --- Adenocarcinomas
--- Acute promyelocytic leukemia (M3)
--- Other neoplasms

6. OTHERS * Liver failure * Acute pancreatitis * Snake venom * ARDS
* Blood transfusion reaction

7. Trauma ---- Shock --- Brain injury --- Crush injury --- Burns
--- Hypothermia/ Hyperthermia
--- Fat embolism, ischemia, hypoxia
--- Surgery

8. Vascular disorders --- Giant hemangiomas (Kassabach-Merritt syndrome)
--- Vascular tumors, Aortic aneurysms

9. 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.

10. S&S
Asymptomatic --- low grade DIC showing only Lab abnormalities

11. Thrombotic complications
--- Trousseau's syndrome
--- Gangrene of digits & extremities
--- Hemorrhagic necrosis of skin
--- Purpura fulminans

12. 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

13. Dx PT, aPTT & TT are prolonged Thrombocytopenia, Plasma Fibrinogen ↓
FDPs ↑ (measured by latex agglutination or D-dimer assay)
Blood film --- Schistocytes, fragmented RBCs

14. DDx
--- Coagulopathy of liver failure
--- TTP, HUS

15. ℞ No treatment --- asymptomatic cases (not ttt lab manifestations)
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

16. Thrombotic Thrombocytopenic Purpura (TTP)
A rare disease characterized by a clinical triad of severe thrombocytopenia, Microangiopathic hemolytic anemia, & Neurologic abnormalities.

17. Renal involvement
proteinuria, hematuria, RBC casts & azotemia that is usually mild & remits on ttt
Fever is a prominent feature

18. Pathology
involved organs show capillaries & arterioles occluded by hyaline material (platelet thrombi) & fibrin deposit in blood vessel wall.

19. Symptoms typically wax & wane (due to platelet aggregation & disaggregation)
Fluctuating neurological abnormalities
Association with pregnancy & oral contraceptive

20. Dx
anemia, thrombocytopenia & Microangiopathic changes of RBCs (Schistocytes) in the absence of other disorders
--- Minimal changes in coagulation tests
--- Biopsy Skin, gum, or BM

21. DDx
DIC, HUS, SLE & Evan's syndrome

22. ℞: 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

23. 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

24. 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

25. 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

26. ℞
Cryoppt
Desmopressin
Platelet transfusion
EACA