Platelets And Coagulation Factor Disorders

Question 1

ITP Full form

Answer 1

Immune Thrombocytopenic purpura

Question 2

Types of ITP

Answer 2

1) Acute ITP - Severe and acute onset
- affects children’s usually
2) Chronic ITP - Less severe and longer duration
- usually affects Adults

Question 3

Clinical features of ITP

Answer 3

Petechiae
Purpura
Hemorrhagic bullae
Hematuria
Melena
Gum bleeding

Question 4

Diagnosis of ITP

Answer 4

Bleeding time increases
Platelet count low (Thrombocytopenia)
PT And aPTT Normal
Coombs test Positive

Question 5

Diagnosis of ITP

Answer 5

Bleeding time increases
Platelet count low (Thrombocytopenia)
PT And aPTT Normal
Coombs test Positive

Question 6

Treatment of ITP

Answer 6

Acute ITP - Symptomatic treatment
Chronic ITP - iv Immunoglobulins
- Steroids
- Splenectomy in severe cases

Question 7

HUS Full form

Answer 7

Hemolytic uremic syndrome

Question 8

Types of HUS

Answer 8

Typical HUS
Atypical HUS

Question 9

Typical HUS

Answer 9

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

Question 10

Atypical HUS

Answer 10

Due to mutation in complementary proteins (Factor H/I/CD-46)
- Excessive damage to endothelial cells
- formation of PLATELET RICH THROMBI

Question 11

Triad Of HUS

Answer 11

1) MAHA
2) Renal Failure
3) Thrombocytopenia

Question 12

Clinical features of HUS

Answer 12

Fever
Bloody diarrhea (Gastroenteritis)
Renal dysfunction (BUN High)
Purpura

Question 13

Diagnosis of HUS

Answer 13

Bleeding time increases
Platelet count low
PT And aPTT Normal

Question 14

Treatment of HUS

Answer 14

Hemodialysis

Question 15

TTP Full form

Answer 15

Thrombotic thrombocytopenic Purpura

Question 16

Pathogenesis of TTP

Answer 16

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

Question 17

Function of enzyme ADAMTS13

Answer 17

Production in liver
Example of metalloprotease
Breakdown vWF clumps in Normal individuals.
Reduces the chances of clumping

Question 18

Deficiency of ADAMTS13 is known as

Answer 18

Upshaw-Schulman Syndrome

Question 19

Pentad of TTP

Answer 19

MAHA
Renal Failure
Thrombocytopenia
Fever
CNS Features

Question 20

Treatment of TTP

Answer 20

Plasmapheresis

Question 21

DIC Full form

Answer 21

Disseminated intravascular coagulation

Question 22

DIC is example of

Answer 22

Thrombo-Hemorrhagic disorder

Question 23

Risk factors of DIC

Answer 23

Obstetrics causes
Infections
Cancer
Burns/Trauma/Surgery

Question 24

Pathogenesis of DIC

Answer 24

Obstetrics/infections/cancer - can cause endothelial cell injury(Intrinsic pathway) - Formation of Widespread Thrombi
Surgery/burns/trauma - Release of tissue factors (Extrinsic pathway)

Question 25

Formation of Widespread Thrombi can leads to

Answer 25

Vascular occlusion Consumption of platelets and Clotting factors Activation of Plasmin (Fibrinolytic pathway) And can increase risk of Bleeding

Question 26

Diagnosis of DIC

Answer 26

Low Hgb, deceased platelet count Peripheral smear - SCHISTOCYTES + Bleeding time increases, PT And aPTT also Increase D-Dimer test - very specific and important finding

Question 27

Clinical features of DIC

Answer 27

Brain - mc affected, dizziness, altered sensorium Cardiac tissue - Dyspnea, deceased CO Kidneys - Acute tubular necrosis lungs Endocrine glands - Excessive hemorrhage in Adrenal gland

Question 28

Treatment of DIC

Answer 28

If infection - treat the primary cause Infusion of fresh frozen plasma Anticoagulant drugs

Question 29

Angiopathic Hemolytic anemia

Answer 29

Blood vessel pathology that leads to RBC Destruction 2 types - Macro-AHA And Micro-AHA

Question 30

Causes of Macro-AHA

Answer 30

Artificial cardiac valves Aortic stenosis Synthetic vascular graft Cavernous hemangioma - collection of blood - RBC Damage

Question 31

Causes of Micro-AHA

Answer 31

HUS TTP DIC Eclampsia Scleroderma Malignant HTN

Question 32

Most common inheritable cause of Bleeding

Answer 32

Von willebrand Disease

Question 33

Weibel-palade body produces

Answer 33

Von willebrand factor (vWF) P-selectin

Question 34

Function of vWF

Answer 34

Helps in process of Platelets adhesion Carrier of Clotting factor 8

Question 35

Pathogenesis of vWD

Answer 35

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

Question 36

Types of vWD

Answer 36

Inherited Acquired

Question 37

Subtypes of vWD

Answer 37

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

Question 38

Clinical features of vWD

Answer 38

Mucosal Bleeding - Epistaxis Increased menstrual loss GI bleeding During wisdom tooth extraction and tonsillectomy surgery bleeding increases

Question 39

Diagnosis of vWD

Answer 39

Bleeding time increases Platelet count Normal aPTT - INCREASES PT - Normal +ve family history

Question 40

Ristocetin test (RAT)

Answer 40

Platelet adhesion rapidly (interaction between gp1b and vWF

Question 41

Treatment of vWD

Answer 41

Type 1 vWD - Desmopressin recombinant vWF Cryoprecipitate

Question 42

Clotting Factors of Intrinsic pathway

Answer 42

Xii, xi, ix, viii

Question 43

Clotting factors of Extrinsic pathway

Answer 43

Factor VII

Question 44

Clotting factors of Common pathway

Answer 44

I, II, V, X

Question 45

Hemophilia A Mode of inheritance

Answer 45

X-linked recessive

Question 46

Hemophilia A defeciency of which Factor

Answer 46

Factor VIII

Question 47

Clinical features of Hemophilia A

Answer 47

Male child - Trauma - tissue bleeding H. Arthrosis (joint), muscle Surgery - excessive bleeding

Question 48

Diagnosis of Hemophilia A

Answer 48

Bleeding time and Platelet count Normal PT normal aPTT increases

Question 49

Factor 8 levels in Hemophilia A

Answer 49

Mild - 6-50% Moderate - 2-5% Severe - <1%

Question 50

Treatment of Hemophilia A

Answer 50

Desmopressin - stimulate endothelial cells - release factor 8 recombinant factor 8(HUMATE) Cryoprecipitate

Question 51

Hemophilia B mode of inheritance

Answer 51

X-linked recessive

Question 52

Hemophilia B defeciency of which factor

Answer 52

Factor IX (Christmas disease)

Question 53

Diagnosis of Hemophilia B

Answer 53

Same as Hemophilia A except Normal factor 8 levels and decreased Factor 9 levels

Question 54

Hemophilia C mode of inheritance

Answer 54

Autosomal recessive

Question 55

Hemophilia C deficiency of which Factor

Answer 55

Factor XI

Question 56

Treatment of Hemophilia B

Answer 56

recombinant factor ix Fresh frozen plasma

Question 57

Factor Inhibitors means

Answer 57

Antibodies against clotting factors

Question 58

Risk factors for Factor inhibitors

Answer 58

Pregnancy Autoimmune disorders B cell cancer Recipient of Clotting factors

Question 59

Must do things for aPTT test

Answer 59

Process in 2 hours Take sample in plastic synringe