Platelets And Coagulation Factor Disorders Flashcards
ITP Full form
Immune Thrombocytopenic purpura
Types of ITP
1) Acute ITP - Severe and acute onset
- affects children’s usually
2) Chronic ITP - Less severe and longer duration
- usually affects Adults
Clinical features of ITP
Petechiae
Purpura
Hemorrhagic bullae
Hematuria
Melena
Gum bleeding
Diagnosis of ITP
Bleeding time increases
Platelet count low (Thrombocytopenia)
PT And aPTT Normal
Coombs test Positive
Diagnosis of ITP
Bleeding time increases
Platelet count low (Thrombocytopenia)
PT And aPTT Normal
Coombs test Positive
Treatment of ITP
Acute ITP - Symptomatic treatment
Chronic ITP - iv Immunoglobulins
- Steroids
- Splenectomy in severe cases
HUS Full form
Hemolytic uremic syndrome
Types of HUS
Typical HUS
Atypical HUS
Typical HUS
Children’s with history of Gastroenteritis (E coli and Salmonella)
- release toxins that can damage endothelial cells and leads to formation of PLATELET RICH THROMBI
Atypical HUS
Due to mutation in complementary proteins (Factor H/I/CD-46)
- Excessive damage to endothelial cells
- formation of PLATELET RICH THROMBI
Triad Of HUS
1) MAHA
2) Renal Failure
3) Thrombocytopenia
Clinical features of HUS
Fever
Bloody diarrhea (Gastroenteritis)
Renal dysfunction (BUN High)
Purpura
Diagnosis of HUS
Bleeding time increases
Platelet count low
PT And aPTT Normal
Treatment of HUS
Hemodialysis
TTP Full form
Thrombotic thrombocytopenic Purpura
Pathogenesis of TTP
1) Deficiency of ADAMTS13
2) Antibody formation against ADAMTS13
Reduction in activity of ADAMTS13
Formation of vWF clumps
Leads to formation of PLATELET RICH THROMBI
Function of enzyme ADAMTS13
Production in liver
Example of metalloprotease
Breakdown vWF clumps in Normal individuals.
Reduces the chances of clumping
Deficiency of ADAMTS13 is known as
Upshaw-Schulman Syndrome
Pentad of TTP
MAHA
Renal Failure
Thrombocytopenia
Fever
CNS Features
Treatment of TTP
Plasmapheresis
DIC Full form
Disseminated intravascular coagulation
DIC is example of
Thrombo-Hemorrhagic disorder
Risk factors of DIC
Obstetrics causes
Infections
Cancer
Burns/Trauma/Surgery
Pathogenesis of DIC
Obstetrics/infections/cancer - can cause endothelial cell injury(Intrinsic pathway) - Formation of Widespread Thrombi
Surgery/burns/trauma - Release of tissue factors (Extrinsic pathway)
Formation of Widespread Thrombi can leads to
Vascular occlusion
Consumption of platelets and Clotting factors
Activation of Plasmin (Fibrinolytic pathway)
And can increase risk of Bleeding
Diagnosis of DIC
Low Hgb, deceased platelet count
Peripheral smear - SCHISTOCYTES +
Bleeding time increases, PT And aPTT also Increase
D-Dimer test - very specific and important finding
Clinical features of DIC
Brain - mc affected, dizziness, altered sensorium
Cardiac tissue - Dyspnea, deceased CO
Kidneys - Acute tubular necrosis
lungs
Endocrine glands - Excessive hemorrhage in Adrenal gland
Treatment of DIC
If infection - treat the primary cause
Infusion of fresh frozen plasma
Anticoagulant drugs
Angiopathic Hemolytic anemia
Blood vessel pathology that leads to RBC Destruction
2 types - Macro-AHA
And Micro-AHA
Causes of Macro-AHA
Artificial cardiac valves
Aortic stenosis
Synthetic vascular graft
Cavernous hemangioma - collection of blood - RBC Damage
Causes of Micro-AHA
HUS
TTP
DIC
Eclampsia
Scleroderma
Malignant HTN
Most common inheritable cause of Bleeding
Von willebrand Disease
Weibel-palade body produces
Von willebrand factor (vWF)
P-selectin
Function of vWF
Helps in process of Platelets adhesion
Carrier of Clotting factor 8
Pathogenesis of vWD
Low vWF - decrease platelet adhesion - bleeding time increases whereas platelet count normal
Also decrease in half life of CF8 - Decrease activity of intrinsic pathway - aPTT increases
Types of vWD
Inherited
Acquired
Subtypes of vWD
Type 1 vWD - AD, Low vWF anr most common
Type 2 vWD - AD, Functional defect of vWF(Qualitative), vWF level normal
Type 3 vWD - very low vWF, most severe nd Autosomal recessive
Clinical features of vWD
Mucosal Bleeding - Epistaxis
Increased menstrual loss
GI bleeding
During wisdom tooth extraction and tonsillectomy surgery bleeding increases
Diagnosis of vWD
Bleeding time increases
Platelet count Normal
aPTT - INCREASES
PT - Normal
+ve family history
Ristocetin test (RAT)
Platelet adhesion rapidly (interaction between gp1b and vWF
Treatment of vWD
Type 1 vWD - Desmopressin
recombinant vWF
Cryoprecipitate
Clotting Factors of Intrinsic pathway
Xii, xi, ix, viii
Clotting factors of Extrinsic pathway
Factor VII
Clotting factors of Common pathway
I, II, V, X
Hemophilia A Mode of inheritance
X-linked recessive
Hemophilia A defeciency of which Factor
Factor VIII
Clinical features of Hemophilia A
Male child - Trauma - tissue bleeding
H. Arthrosis (joint), muscle
Surgery - excessive bleeding
Diagnosis of Hemophilia A
Bleeding time and Platelet count Normal
PT normal
aPTT increases
Factor 8 levels in Hemophilia A
Mild - 6-50%
Moderate - 2-5%
Severe - <1%
Treatment of Hemophilia A
Desmopressin - stimulate endothelial cells - release factor 8
recombinant factor 8(HUMATE)
Cryoprecipitate
Hemophilia B mode of inheritance
X-linked recessive
Hemophilia B defeciency of which factor
Factor IX (Christmas disease)
Diagnosis of Hemophilia B
Same as Hemophilia A except Normal factor 8 levels and decreased Factor 9 levels
Hemophilia C mode of inheritance
Autosomal recessive
Hemophilia C deficiency of which Factor
Factor XI
Treatment of Hemophilia B
recombinant factor ix
Fresh frozen plasma
Factor Inhibitors means
Antibodies against clotting factors
Risk factors for Factor inhibitors
Pregnancy
Autoimmune disorders
B cell cancer
Recipient of Clotting factors
Must do things for aPTT test
Process in 2 hours
Take sample in plastic synringe
