Platelets And Coagulation Factor Disorders Flashcards

1
Q

ITP Full form

A

Immune Thrombocytopenic purpura

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2
Q

Types of ITP

A

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

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3
Q

Clinical features of ITP

A

Petechiae
Purpura
Hemorrhagic bullae
Hematuria
Melena
Gum bleeding

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4
Q

Diagnosis of ITP

A

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

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5
Q

Diagnosis of ITP

A

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

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6
Q

Treatment of ITP

A

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

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7
Q

HUS Full form

A

Hemolytic uremic syndrome

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8
Q

Types of HUS

A

Typical HUS
Atypical HUS

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9
Q

Typical HUS

A

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

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10
Q

Atypical HUS

A

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

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11
Q

Triad Of HUS

A

1) MAHA
2) Renal Failure
3) Thrombocytopenia

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12
Q

Clinical features of HUS

A

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

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13
Q

Diagnosis of HUS

A

Bleeding time increases
Platelet count low
PT And aPTT Normal

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14
Q

Treatment of HUS

A

Hemodialysis

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15
Q

TTP Full form

A

Thrombotic thrombocytopenic Purpura

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16
Q

Pathogenesis of TTP

A

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

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17
Q

Function of enzyme ADAMTS13

A

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

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18
Q

Deficiency of ADAMTS13 is known as

A

Upshaw-Schulman Syndrome

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19
Q

Pentad of TTP

A

MAHA
Renal Failure
Thrombocytopenia
Fever
CNS Features

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20
Q

Treatment of TTP

A

Plasmapheresis

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21
Q

DIC Full form

A

Disseminated intravascular coagulation

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22
Q

DIC is example of

A

Thrombo-Hemorrhagic disorder

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23
Q

Risk factors of DIC

A

Obstetrics causes
Infections
Cancer
Burns/Trauma/Surgery

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24
Q

Pathogenesis of DIC

A

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

25
Q

Formation of Widespread Thrombi can leads to

A

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

26
Q

Diagnosis of DIC

A

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

27
Q

Clinical features of DIC

A

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

28
Q

Treatment of DIC

A

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

29
Q

Angiopathic Hemolytic anemia

A

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

30
Q

Causes of Macro-AHA

A

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

31
Q

Causes of Micro-AHA

A

HUS
TTP
DIC
Eclampsia
Scleroderma
Malignant HTN

32
Q

Most common inheritable cause of Bleeding

A

Von willebrand Disease

33
Q

Weibel-palade body produces

A

Von willebrand factor (vWF)
P-selectin

34
Q

Function of vWF

A

Helps in process of Platelets adhesion
Carrier of Clotting factor 8

35
Q

Pathogenesis of vWD

A

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

36
Q

Types of vWD

A

Inherited
Acquired

37
Q

Subtypes of vWD

A

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

38
Q

Clinical features of vWD

A

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

39
Q

Diagnosis of vWD

A

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

40
Q

Ristocetin test (RAT)

A

Platelet adhesion rapidly (interaction between gp1b and vWF

41
Q

Treatment of vWD

A

Type 1 vWD - Desmopressin
recombinant vWF
Cryoprecipitate

42
Q

Clotting Factors of Intrinsic pathway

A

Xii, xi, ix, viii

43
Q

Clotting factors of Extrinsic pathway

A

Factor VII

44
Q

Clotting factors of Common pathway

A

I, II, V, X

45
Q

Hemophilia A Mode of inheritance

A

X-linked recessive

46
Q

Hemophilia A defeciency of which Factor

A

Factor VIII

47
Q

Clinical features of Hemophilia A

A

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

48
Q

Diagnosis of Hemophilia A

A

Bleeding time and Platelet count Normal
PT normal
aPTT increases

49
Q

Factor 8 levels in Hemophilia A

A

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

50
Q

Treatment of Hemophilia A

A

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

51
Q

Hemophilia B mode of inheritance

A

X-linked recessive

52
Q

Hemophilia B defeciency of which factor

A

Factor IX (Christmas disease)

53
Q

Diagnosis of Hemophilia B

A

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

54
Q

Hemophilia C mode of inheritance

A

Autosomal recessive

55
Q

Hemophilia C deficiency of which Factor

A

Factor XI

56
Q

Treatment of Hemophilia B

A

recombinant factor ix
Fresh frozen plasma

57
Q

Factor Inhibitors means

A

Antibodies against clotting factors

58
Q

Risk factors for Factor inhibitors

A

Pregnancy
Autoimmune disorders
B cell cancer
Recipient of Clotting factors

59
Q

Must do things for aPTT test

A

Process in 2 hours
Take sample in plastic synringe