This document discusses hemostasis and various bleeding disorders. It defines hemostasis and describes the primary and secondary stages. The primary stage involves platelet plug formation from platelet adhesion, activation, and aggregation. The secondary stage activates the intrinsic and extrinsic coagulation pathways. The document outlines various signs and symptoms of bleeding disorders and provides examples. It discusses screening tests like complete blood count, prothrombin time, activated partial thromboplastin time, and bleeding time. Finally, it analyzes several case scenarios involving abnormal coagulation test results.

1. Dr M.Sanjeevappa
M.D.(Paeds)
Asst.Professor,
Dept. of Paediatrics
Govt.Medical College
Ananthapuramu.

2. Dr M.Sanjeevappa
M.D.(Paeds)
Asst.Professor,
Dept. of Paediatrics
Govt. Medical College
Ananthapuramu.

3.  Definition: Hemostasis is a protective
response of the body to limit and reverse loss of
vascular integrity and prevent excessive blood
loss.
 primary stage of hemostasis: formation of a
“platelet plug”
 platelet adhesion.
 platelet activation.
 platelet aggregation.

4.  secondary stage of hemostasis: activation
of coagulation cascades.
 Intrinsic pathway.
 Extrinsic pathway.

7. HISTORY:
 Exaggerated bleeding responses to
commonly encountered events are
suggestive of an underlying bleeding
disorder.
Examples :
 Epistaxis lasting for more than 15 minutes
despite pressure application on the side of
the nostril.

8.  Significant blood loss from a dental
procedure lasting more than 24 hours or
requiring blood transfusion.
 Bruising out of proportion to inflicted trauma.
 Menorrhagia characterized by heavy
menstrual bleeding lasting for 7 days or loss
of more than 80 mL of blood per cycle.

9.  X-linked disorders : detailed history of
bleeding problems in the maternal
grandfather, uncles, and cousins.
Eg : Hemophilia A.
 Autosomal recessive conditions : history
of consanguinity is important.
Eg : FXIII deficiency.

10.  Mucosal and subcutaneous bleeding :
involving gums or nasal mucosa,
petechiae, and bruising suggestive of
disorders of platelets and blood vessels or
vWD.
 spontaneous or excessive bleeding: into
soft tissues, muscles, joint and delayed
surgical bleeding suggestive of disorders
of coagulation factors.

11.  Acquired disorders such as immune
thrombocytopenic purpura (ITP) present
over days.
 vWD or hemophilia present over months
to years.

12.  A male infant presenting with painful bleeding in
the joint after a fall suggestive of hemophilia.
 Healthy child with purpuric rash following a viral
syndrome suggestive of ITP.
 A teenage girl with excessive menstrual bleeding
and pallor suggestive of vWD.
 Petechial rash and severe circulatory collapse in
a teenager suuggestive of vasculitis or DIC from
meningococcemia or sepsis.

13. Screening Tests :
 Complete blood count (CBC).
 Peripheral blood smear (PBS).
 Prothrombin time (PT).
 Activated partial thromboplastin time
(aPTT).
 Bleeding time.
 Platelet function analyzers (PFAs).

14.  To determine platelet count.
 Presence of anemia the microcytic
hypochromic type.
 suppression of all blood cell lines-
suggestive of malignancies such as
leukemia and lymphoma.

15.  To confirm thrombocytopenia.
 Platelet morphology :
Giant platelets – Bernard-Soulier disease.
Normal and large platelets – ITP
Smaller than normal platelets
-Wiskott -Aldrich syndrome.
 To identify abnormal cells such as blasts.

16.  PT evaluates the function of the extrinsic and
common pathways of coagulation.
 So PT measures the functioning of TF, FVII
and FX, FV, prothrombin, and fibrinogen.
 PT also indicate integrity of the vitamin K-
dependent coagulation factors (II, VII, & X).

17.  aPTT measures the functioning of
intrinsic and common pathways of
coagulation.
 so, sensitive to deficiencies of factors
XII, XI, IX, and VIII, X, V, II, and I as
well as their inhibitors such as
heparin.

19.  Defects in the primary phase of
hemostasis (vWD and platelet
dysfunction).
 Defects in the secondary phase
(FXIII deficiency).
 Defects in the fibrinolytic pathway.

21. Case Scenario 2: A Child With
Significant Bleeding,
Normal PT,
Prolonged aPTT,
Normal platelet count.

22.  Possibility of deficiency of factors in the
intrinsic pathway, especially FVIII, FIX,
and FXI.
 Inhibitors such as lupus anticoagulant and
heparin contamination.
 Secondary FVIII deficiency caused by
vWD can also present with these
laboratory findings.

24. Case Scenario 3: A Child With
Significant Bleeding History
With Prolonged PT,
Normal aPTT, and
Normal Platelet Count

25.  suggestive of FVII dysfunction.
Isolated FVII deficiency is a rare
entity

26. Case Scenario 4: A Child
With Significant History of
Bleeding,
With Prolongation of PT
as Well as aPTT and
a Normal Platelet Count

27.  scenario is the hallmark of vitamin K
deficiency.

29. Case Scenario 5: Child With
Significant Bleeding and
Prolongation of PT and aPTT
and Thrombocytopenia

30.  This scenario is seen in DIC or
consumptive coagulopathy.

31. Case Scenario 6: Child Presenting
With Skin or Mucosal Petechiae and
Thrombocytopenia,
With Normal PT and aPTT

32.  immune thrombocytopenic purpura.

33. THANK YOU