13. bleeding disorders

Question 1

Haemostasis

Answer 1

Mechanism which maintains the integrity of the circulatory system after vascular damage

Question 2

haemostasis achieved by

Answer 2

vessel constriction  platelet plug (primary haemostasis)  activation of the coagulation cascade and fibrin deposition (secondary haemostasis)

Question 3

Lab for primary hemostasis

Answer 3

Platelets count
Bleeding time
Platelets function assay
Platelets agrigometry
PM and BM study

Question 4

normal PTT and PT time

Answer 4

PTT 25-37
PT 10-13

Question 5

If ptt increased and pt normal problem in

Answer 5

intrinsic

Question 6

if ptt and pt increase

Answer 6

common pathway

Question 7

if ptt normal and pt increase

Answer 7

extrinsic pathway problem

Question 8

which factor has longest and shortest half life

Answer 8

longets factor 2
shortest factor 7

Question 9

The clinical significance of hemorrhage depends on

Answer 9

Volume of the bleed – rapid loss > 20% of the blood volume can cause hemorrhagic (hypovolemic) shock,

Rate of bleeding – chronic or recurrent external blood loss results iron deficiency anemia

Location – trivial in subcutaneous tissues vs deadly in the brain

Question 10

Defects in blood vessel

Answer 10

Vasculitis
Scurvy
Amyloidosis
Malignant HTN
Uterine atony

Question 11

henoch scholein purpura clinical manifestation

Answer 11

palpable purpura
joint pain
blood in urine
pain in abdomen

Question 12

primary heamostasis defect because of

Answer 12

defect in platlet either qualitatively or quantitavily

von willebrand diesease both

Question 13

qualitative defect in platelet divided into 2

Answer 13

genetic and acquired

Question 14

genetic qualitative defect in platelet

Answer 14

Bernard-Soulier syndrome

Glanzmann thrombasthenia

Question 15

Bernard-Soulier syndrome

Answer 15

defect in glucoprotein Ib-IX-V
enlarged platlets

Question 16

Glanzmann thrombasthenia

Answer 16

defect in glycoprotein IIb-IIIa

Question 17

acquired qualitative defect in platelet

Answer 17

uremia
drugs - asprin

Question 18

quantitative defects include

Answer 18

thrombocytopenia

idiopathic thrombocytopenic purpura

thrombotic microangiopathy - ttp - hus - dic

waterhouse fridrichsen syndrome

trousseaus syndrome

Question 19

Thrombocytopenia

Answer 19

(< 150,000 platelets/μL)

Question 20

causes of thrombocytopenia

Answer 20

decreased production
ineffective production
increased destruction
increased sequestration
dilution

Question 21

idiopathic thrombocytopenic purpura

Answer 21

primary immune thrombocytopenia
pathogenesis antibody against platlet antigen

Question 22

thrombotic microangiopathy divided into

Answer 22

ttp
hus
dic

Question 23

thrombotic thrombocytopinic purpura ttp

Answer 23

low ADAM13 (cleaves von wille)
platelet aggregation
lysis

Question 24

5 characteristics of ttp

Answer 24

fever
thrombocytopenia
microangiopathic hemolytic anemia
neurologic
renal failure

Question 25

Hemolytic uremic syndrome hus
cause pathogenesis clinical manifestation

Answer 25

shiga toxin
endothelial damage
thrombocytopenia

clinical
renal failure
hemorragic diarrhea

Question 26

Disseminated intravascular coagulation
dic
cause

Answer 26

tf release
obg comlication
trauma
gm -ve endotoxins
cancer

Question 27

DIC lab findings

Answer 27

↓platelet count,
prolonged PT/PTT,
decreased fibrinogen, and
↑ fibrin split products (D dimers).

Question 28

waterhouse fridrichsen syndrome

Answer 28

adrenal gland failure
cause bacterial infection

Question 29

trousseus syndrome

Answer 29

recurrent venous thrombi formation due to procooagulant factors formed by cancer cells
pancreatic cancer

Question 30

von willebrand diesease
plus lab finding

Answer 30

autosomal dominant
normal platlet count
normal pt
increased ptt
increased bleeding time

Question 31

Defects of secondary hemostasis are in

Answer 31

coagulation factors

Question 32

2 types of secondary heamostasis defect

Answer 32

hereditary and acquired

Question 33

hereditary include

Answer 33

hemophilia a b and c

Question 34

hemophilia a
and lab

Answer 34

x linked recessive
defect in factor 8

ptt prolonged
normal everything

Question 35

hemophilia b

Answer 35

x linked recessive
defect in factor 9 (christmas factor)

Question 36

hemophilia c

Answer 36

defect in factor 11
autosomal recessive

Question 37

acquired secondary heamostasis defects

Answer 37

vit k deficiency
warfarin
liver failure
deficient transfer of factors in transfusion
coagulation factor inhibitors

Question 38

mixed study

Answer 38

The patient’s plasma is collected and mixed in equal parts with normal pooled plasma that contains all clotting factors.The mixture is then subjected to a series of clotting tests, such as the activated partial thromboplastin time (aPTT) or prothrombin time (PT), depending on the suspected coagulation pathway involved.The clotting times of the patient’s mixture are compared to the clotting times of a control sample (normal plasma mixed with normal pooled plasma).

Corrected or normalized clotting times: If the abnormal clotting times of the patient’s mixture correct or normalize after mixing with normal plasma, it suggests a factor deficiency as the cause of the coagulopathy. The presence of adequate clotting factors in the normal plasma compensates for the deficiency observed in the patient’s plasma.Persistent abnormal clotting times: If the clotting times of the patient’s mixture remain prolonged or abnormal after mixing with normal plasma, it suggests the presence of an inhibitor. An inhibitor can neutralize or interfere with the activity of clotting factors, leading to impaired blood clotting.