How to approach any bleeding disorders in the Paediatric Population.

1. APPROACH TO A
BLEEDING CHILD
D R . S U R A B H I P E R I W A L
1 S T Y R P G T
S C B M C H

2. CONTENTS
1. PHYSIOLOGY OF HEMOSTASIS
2. CLINICAL & LABORATORY EVALUATION OF HEMOSTASIS
3. CASE SCENARIO I
4. HEREDITARY CLOTTING FACTOR DEFICIENCIES
5. CASE SCENARIO II
6. PLATELET DISORDERS
7. CASE SCENARIO III
8. BLOOD VESSEL DISORDERS
9. SUMMARY

3. Hemostais
Coagulation
Cascade
Blood Vessel
Factors
Platelet
Factors

5. HEMOSTASIS

6. CASE SCENARIO I
• A 3year old Male child, presented to the Emergency Department with a knee swelling
after a trivial trauma 12 hours ago. Patients vitals were otherwise stable. On taking
detailed history, his mother gave history of prolonged bleeds from immunization sites,
small cut wounds that wouldn’t stop bleeding. She also gave history that the boy’s
cousin also suffered from a similar illness. The patient was not on any drugs, no
previous history of surgery.
• Lab tests showed -HEMOGLOBIN 12.3 g/dl (10.5-13.5)
HEMATOCRIT 35.4% ( 33-39 )
TLC 7900 (6000-11000)
TPC 3.68 lakhs ( 1.5-4.5 )
PROTHROMBIN TIME 11.3s ( 10-13s )
aPTT 42.2s ( 24-33s )

7. CASE SCENARIO I
• A 3year old Male child, presented to the Emergency Department with a knee swelling
after a trivial trauma 12 hours ago. Patients vitals were otherwise stable. On taking
detailed history, his mother gave history of prolonged bleeds from immunization
sites, small cut wounds that wouldn’t stop bleeding. She also gave history that the
boy’s cousin also suffered from a similar illness. The patient was not on any drugs,
previous history of surgery.
• Lab tests showed -
HEMOGLOBIN 12.3 g/dl (10.5-13.5)
HEMATOCRIT 35.4% ( 33-39 )
TLC 7900 (6000-11000)
TPC 3.68 lakhs ( 1.5-4.5 )
PROTHROMBIN TIME 11.3s ( 10-13s )
aPTT 42.2s ( 24-33s )

9. HEMOPHILIA A/B
• Deficiency of factor VIII or factor IX ( XLR ) Thus, mostly seen in boys.
• C/F – Easy bruising, Intramuscular hematomas, Spontaneous Hemarthrosis
• Patient may lose large volumes of blood into the iliopsoas muscle, verging on hypovolemic
shock, with only a vague area of referred pain in the groin & flexed hip.
• Patients with hemophilia slowly form a soft, friable clot.
• M/C bleeding disorder with severe bleeding manifestation.
• T/T – Factor Replacement Therapy. Maintain atleast 35 – 50 % range.
Dose – Desired VIII % Rise * Body Weight (Kg) * 0.5
Desired XI % Rise * Body Weight (Kg) * 1.3
PROTHROMBIN TIME N
aPTT RAISED
BLEEDING TIME N
PLATELET COUNT N
Mild – > 5 % Moderate – 1 to 5 % Severe – < 1 %

10. HEMOSTASIS HEMOPHILIA A

11. HEMOSTASIS HEMOPHILIA A
HEMOPHILIA B

12. HEMOSTASIS HEMOPHILIA A
HEMOPHILIA B
HEMOPHILIA C

13. HISTORY ~
• Presenting Complains – Prolonged bleeding, easy bruisability, bleeding P/R,
gums, nose.
• Age of presentation –
Neonatal – HDN, Factor 13 def., Congenital Amegakaryocytic
Thrombocytopenia
Infancy – TAR Syndrome
Toddler – Hemophilias, vWD, Ehlers Danlos Syndrome
1-4 yrs – Acute ITP
• Sex of the child –
Hemophilia, Wiskot Aldrich Syndrome are more common in Males
SLE, Chronic ITP are more common in Females
• History of Present Illness – Site, Duration, No. of episodes, Amount, Onset,

14. HISTORY ~
• Perinatal History – Birth Cephalhematoma
Umbilical Stump bleeding
Post-Circumcisional Bleeding
• Nutritional History – Protein Malnutrition
Vit C & Vit K deficiency
• Family History – H/O consanguinity (As many bleeding d/o have AR inheritance )
Known bleeding d/o in family or any other heritable medical d/o.
Male or Female predisposition
• Immunisation History – H/o bleeding from injection sites
• Treatment History – H/o transfusions
Intake of drugs predisposing to thrombocytopenia
Any dental/surgical procedures
• Menstrual History

15. PL ATELET DISORDERS
• EXCESSIVE BLEEDING AFTER
SUPERFICIAL CUTS & WOUNDS
• PETECHIAE, PURPURA
• RARE
• RARE
• RARE
• BT
• M = F
COAGULATION DISORDERS
• BLEEDING AFTER DEEP WOUNDS
• RARE
• ECHHYMOSIS
• HEMARTHROSIS
• HEMATOMA
• CT
• M > F
COMMON PRESENTATIONS

16. CLINICAL EXAMINATION
• Presence of any Dysmorphic Features, Absent Radius or Thumb Hypo/Aplasia.
• Vitals Signs – Pulse, RR, HR, BP, Temperature
• Anthropometry – for signs of Malnutrition
1. Skin – Telangiectasias, Petechiae, Purpura, Ecchymosis
2. Mucosal Membranes – Nasal / Gingival Bleeding
3. Musculo-Skeletal – Hematoma, Hemarthroses, Joint contractures, Bony
deformities, Joint Laxity
4. Abdomen – Hepatomegaly, Splenomegaly
5. Cardio-Vascular – TAR Synd is associated with TOF & ASD
6. Nervous System – Maybe altered in CNS bleeds
7. Any other Mass, Lymph Node Swellings

17. Petechiae – Pin-point, Flat, Round, Red spots under the skin
surface caused by intradermal
Purpura – Purple Coloured spots & patches on the skin &
mucus membranes. Group of adjoining
Ecchymosis – Flat, blue or purple patch measuring
1cm or more in diameter.

18. Joint Contracture in the Left Elbow in a patient
With Hemophilia A.
The Elbow joint is a target joint in this patient.

19. INVESTIGATIONS
• PLATELET COUNT – Thrombocytopenia ( ITP, TTP, Leukemia, Infective causes )
• HEMOGLOBIN – Anaemia ( Maybe severe & require transfusion in some
cases )
• TOTAL LEUKOCYTE COUNT – Raised ( Infections, Leukemia )
Decreased ( Bone Marrow Infiltrative D/O )
• ESR / CRP – Indicative of any Vasculitis viz. Henoch – Schonlein Purpura, SLE
• URINE RE / ME – To look for bleeding manifestations
• XRAY OF THE AFFECTED JOINT
• NCCT – If patient has any Neurological Symptoms owing to ICH.

20. TESTS FOR COAGULATION ~
TEST MECHANISM TESTED NORMAL RANGE DISORDERS
PROTHROMBIN TIME EXTRINSIC &
COMMON PATHWAY
10-13 secs DEFECT IN VIT. K
DEPENDANT FACTORS,
LIVER DISEASE, DIC
ACTIVATED PARTIAL
THROMBOPLASTIN
TIME
INTRINSIC &
COMMON PATHWAY
25-40 secs HEMOPHILIAS, vWD, DIC
BLEEDING TIME HEMOSTASIS,
CAPILLARY &
PLATELET FUNCTION
2-9 mins THROMBOCYTOPENIAS,
THROMBASTHENIAS,
vWD
PLATELET COUNT NUMBER 150,000 - 450,000 /
mm3
THROMBOCYTOPENIAS
THROMBIN TIME COMMON PATHWAY 11-15 secs AFIBRINOGENEMIA /
DYSFIBINOGENEMIA

21. History & Physical Examination
Normal Decreased
Platelet Count
EVALUATION OF A BLEEDING CHILD
Coagulation Screen ( PT,
aPTT, TT)
Normal Abnormal
Acute Illness
Yes No
• Purpura Fulminans
• Meningococcus
• Varicella

22. P U R P U R A
F U L M I N A N S
Seen in a D7 old neonate with
Meningococcal Infection
leading to Severe Sepsis & DIC.

23. Platelet Count – N
Coagulation Screen – N
Chronic Illness
No Yes
Bleeding History
• R/O Non-Accidental
Trauma
Factor VIII:C, VWF:Rco,
VWF Ag
Normal
Abnormal
• Platelet Dysfunction
• Variant vWD
• Mild F XI or F XIII def.
• Mild Hemophilia or Trait
• vWD
• Mild Hemophilia A
or Carrier

24. VON WILLEBRAND DISEASE
• Most Common Inherited Bleeding Disorder
• Functions of vWF – 1. Tethers platelets to sub-endothelium
2. Carrier protein for Factor VIII
• C/F – Mucosal Bleeding, Epistaxis, Menorrhagia, Easy Bruising, Surgical Bleeding
TYPE 1 TYPE 2A TYPE 2B TYPE 2M TYPE 2N TYPE 3
Most Common Most Severe
Mucosal
Bleeds, Easy
Bruising
Defect in vWF
multimerisation
Inc. binding &
clearance of
vWF &
platelets
Defective
binding of vWF
to platelet
GpIb
Defective
binding of vWF
with F VIII
Hemarthroses
& CNS bleeds
also seen
vWF
concentrate
maybe given
Desmopressin,
vWF
concentrate
C/I -
Desmopressin
vWF
concentrate
vWF
concentrate

25. History & Physical Examination
Normal Decreased
Platelet Count
EVALUATION OF A BLEEDING CHILD
Coagulation Screen ( PT, aPTT, TT)
Normal
AbnormalAbnormal
Corrects Does Not Correct
1:1 mixing of patient : normal plasma

26. Platelet Count – N
Coagulation Screen – AbN
1:1 mixing CORRECTS screen
Abnormal PT Abnormal aPTT Abnormal TT
Only PT Abnormal
• Factor VII def.
• Early Oral Anticoagulant
Rx
Both PT & aPTT Abnormal
• Oral Anticoagulant Rx
• Liver Disease
• Vitamin K deficiency
• Mild DIC
• F II, V, X deficiency
Abnormal PT, aPTT, TT &
Thrombocytopenia
• DIC
• Severe Liver Failure

27. FACTOR VII DEFICIENCY
• Rare, Autosomal Bleeding Disorder.
• C/F – Hemarthroses, Mucosal Bleeding, Spontaneous Intracranial Bleeds, Epistaxis,
Menorrhagia
• T/T – Factor VII concentrates
FFP ( Although, half life of factor VII is 2 – 4 hrs )
PT RAISED
aPTT NORMAL

28. HEMORRHAGIC DISEASE OF
NEWBORN
• Late Hemorrhagic Disease in Breast fed Newborns
• Etiology – Lack of oral intake as Breast milk is deficient in Vit K
Long term use of broad spectrum Antibiotics
Liver Disease
Malabsorption
• T/T – Prophylactic Vit K is indicated in all newborns

29. LIVER DISEASE
• All clotting factors are exclusively produced in the liver, except F VIII
• But, bleeding episodes occur in only 15% of patients d/t concomitant reduction in anti-
coagulant proteins.
• T/T of coagulopathy is reserved for cases with clinical bleeding.
• T/T – Vitamin K therapy
Fresh Frozen Plasma
Cryoprecipitate
Desmopressin ( to augment hemostasis before Liver Biopsy )

30. Platelet Count – N
Coagulation Screen – AbN
1:1 mixing CORRECTS screen
Abnormal PT Abnormal aPTT Abnormal TT
Bleeding History
Absent Present
Factor VIII:C, VWF:Rco, VWF Ag
Factor IX, XI, X
• Factor XI or XII def
• Mild Hemophilia B
• Prekallikrein/Kininogen def
• vWD
• Hemophilia A or B
• Severe F XI def

31. 1:1 mixing study
Corrects Does not Correct
Abnormal TT
• Hypofibrinogenemia
• Dysfibrinogenemia
• Heparin Toxicity
Reptilase Time
Normal Raised
• Fibrin Split Products

32. AFIBRINOGENEMIA / DYSFIBRINOGENEMIA
• Rare, Autosomal Recessive disorder
• Patients present in Neonatal period with GI bleeding, Umbilical Stump Bleeding,
Hematomas due to minor birth trauma
• Dysfibrinogenemia can present with Thrombosis.
• T/T – Human Fibrinogen Concentrate
Fresh Frozen Plasma
Cryoprecipitate
PT RAISED
aPTT RAISED
TT RAISED

33. HEMOSTASIS
AFIBRINOGENIMIA/
DYSFIBRINOGENIMIA

34. SUMMARY
PT aPTT TT DEFICIENCY
RAISED N N Factor VII
N RAISED N Factor VIII, IX, XI, XII
RAISED RAISED N Factor X
N N N Factor XIII
RAISED RAISED RAISED DIC
RAISED RAISED RAISED Afibrinogenemia
RAISED RAISED RAISED Heparin Toxicity

35. PLATELET
ADHESION &
AGGREGATION
DEFECTS

36. PLATELETS
• Produced by Megakaryocytes under the control of Thrombopoetin
• Life Span – 8 to 10 days
• Normal Range – 1.5 to 4.5 lakhs.
• THROMBOCYTOPENIA – Less than 1.5 lakh.
Causes : Premature destruction
Decreased production
Splenic Trapping

37. • THROMBASTHENIA – Platelet Count Normal
Qualitative defect in platelet function
• Bleeding Time can be affected by both Platelet Count & Platelet function. But is
dependent of many factors including technicians skill & patients co-operation.
• PFA-100 is a test which was used earlier, lacks specificity
• Platelet Function is tested by Platelet Aggregometry.
Agonists –
Collagen
ADP
Ristocetin
Epinephrine
Arachidonic Acid
 Clumping of platelets measured over
time by an automated machine.
 Release of granular contents
after platelet activation.
measures

38. BERNARD SOULIER SYNDROME
Severe Congenital platelet function disorder
Absence / Severe Def. of receptor for vWF on the platelets [ Gp Ib-IX ]
C/F – Thrombocytopenia
Giant platelets
Greatly prolonged Bleeding Time / PFA-100 Closure Time
Significant mucosal & GI bleeding
Inv – Absent Ristocetin Aggregation. Normal Aggregation with other agonists.
vWF – Normal
Confirmation by – Flow Cytometry of platelet Glycoproteins

39. GLANZMANN THROMBASTHENIA
• Congenital Disorder with severe platelet dysfunction
• Inv – Normal Ristocetin Aggregation. Abnormal / Absent aggregation with
other agonists.
Confirmation by – Flow Cytometry of platelet Glycoproteins
BLEEDING TIME RAISED
Closure PFA-100
Time
RAISED
PLATELET COUNT NORMAL

40. CASE SCENARIO II
A 4 yo FCh, came to the Emergency Department, with multiple petechial spots all over
the body,
mucosal bleeding in form of epistaxis & oral mucosal bleed. The child was playful. No
other systemic abnormality was seen. No previous H/O any other similar complains.
• Lab Tests show :
HEMOGLOBIN 9.1 g/dl (10.5-13.5)
HEMATOCRIT 31.2 % ( 33-39 )
TLC 8100 (6000-11000)
TPC 44,000 ( 1.5-4.5 )
PROTHROMBIN TIME 11.3s ( 10-13s )
aPTT 27s ( 24-33s )

41. CASE SCENARIO II
A 4 yo FCh, came to the Emergency Department, with multiple petechial spots all over
the body, mucosal bleeding in form of epistaxis & oral mucosal bleed. The child was
playful. No other systemic abnormality was seen. No previous H/O any other similar
complains.
• Lab Tests show :
HEMOGLOBIN 9.1 g/dl (10.5-13.5)
HEMATOCRIT 31.2 % ( 33-39 )
TLC 8100 (6000-11000)
TPC 44,000 ( 1.5-4.5 )
PROTHROMBIN TIME 11.3s ( 10-13s )
aPTT 27s ( 24-33s )

42. I D I O PAT H I C
T H R O M B O C Y TO P E N I C
P U R P U R A

43. IMMUNE THROMBOCYTOPENIC
PURPURA ( ITP)
• Sudden onset of generalized petechiae & purpura.
• H/O a viral infection within last 4 wks maybe present
• Splenomegaly, Lymphadenopathy, Bone pain, Pallor are rare.
• Classification :
No Symptoms
MILD Bruising, Petechiae, Minor Epistaxis, Little interference with daily
living
MODERATE Severe Skin & Mucosal lesions, Troublesome Epistaxis &
Menorrhagia
SEVERE Bleeding episodes requiring transfusions, Symptoms interfering
seriously with the quality of life.

44. TREATMENT ~
• Observation & Counselling about the benign nature of the disease.
• Oral Corticosteroids – 1 to 4 mg/kg/day shows rapid rise in platelets
• IVIG – 0.8 to 1 g/kg/day for 1-2 days
• IV Rituximab
• Thrombopoetin Recepter Agonists
• Splenectomy

45. Congenital Anomalies
No Yes
PMN Hypersegmentation / Macrocytes /
Dec. Serum Vit. B12
History & Physical Examination
Normal Decreased
Platelet Count
Complete Blood Count
Peripheral Smear
No Yes
Ill- appearing ?
Vit B12 or Folate
Deficiency

46. Congenital Anomalies
No Yes
History & Physical Examination
Normal Decreased
Platelet Count
Complete Blood Count
Peripheral Smear
Yes No
Ill- appearing ?
• Dyskeratosis Congenita
• Fanconi Anemia
• Trisomy 13 or 18
• Kasabach Merrit Syn., TAR Syn

47. DYSKERATOSIS CONGENITA
• Inherited Multisystem Telomere Disorder
• Atleast 2 of the 4 major criteria – Abnormal Skin Pigmentation
Nail Dystrophy
Leukoplakia
Pancytopenia d/t Bone marrow failure.
• T/T – only HSCT is curative.

48. Platelet Count – Decreased
Well appearing child
No Congenital Anomalies
Present Not Present
Medications / Immunisations
Radiations / Toxins
Drug Induced
Live
Vaccination
Toxin
Mediated
Macrothrombocyt
es
Other
Morphological
Platelet Changes
Yes No
• Hermansky Pudlak Syn
• Gray Platelet Syn • ITP
• Hereditary Thrombocytopenia
• Bernard Soulier Syn
Bone Marrow
Finding
Inc. / N
Megakaryocytes
Dec
Megakaryocyt
es
• Leukemia
• Aplastic Anemia
• Drug-induced
• Myelodysplasia

49. History & Physical Examination
Normal Decreased
Platelet Count
Complete Blood Count
Peripheral Smear
No Yes
Ill- appearing ?
PT, aPTT, TT
ProlongedDIC Normal

50. DISSEMINATED INTRAVASCULAR
COAGULATION ( DIC )
• Life-threatening diseases associated with Hypoxia, Acidosis, Tissue Necrosis, Shock, Endothelial
Damage may lead to DIC.
• C/F – Hemorrhagic Picture ( d/t resulting deficiency of F V, VIII, Prothrombin, Fibrinogen &
Platelets
Shock
Anemia ( Microangiopathic Hemolytic )
Tissue Necrosis
• Investigations – Prolonged PT, aPTT, TT
Decreased Platelet Count
Peripheral Smear – Schistocytes
D – dimer Assay – Fibrinogen Degradation Pdts formed d/t coagulation &
Cytokine & Chemokine activation.
Alteration of Endothelial function.
Formation of micro-vascular thromboses
Consumption of Pro & Anti Coagulant
factors.
PT RAISED
aPTT RAISED
TT RAISED

51. DIC..
• Treatment – Treat the Trigger
Correct Shock, Hypoxia, Acidosis
• Blood Components used as Replacement Therapy in cases of hemorrahage.
1. Fresh Frozen Plasma
2. Cryoprecipitate
3. Platelet Infusions
• Prognosis depends upon outcome of primary disease & prevention of end-organ
damage

52. Platelet Count – Decreased
Ill appearing child
Splenomegaly
Portal HTN
Male, Eczema, Small Platelets,
Recurrent Infections
Lymphadenopathy
Hepatosplenomegaly
Abdominal Mass
SVC Syndrome
Acute Febrile Illness
Chronically Ill Appearing
PT, aPTT, TT – N
• Gauchers Dis
• Malaria
• Portal HTN
• Wiskott
Aldrich
Syndrome • Lymphoma
• Neuroblastoma
• Leukemia
• HIV
• Auto-immune/CTD
• HUS/TTP
HIV, ANA, RFT
• Sepsis
• EBV
• CMV
• Varicella
• Malaria, DHF
• Hanta Virus, Parvo
Virus

53. CASE SCENARIO III
• A 2 year 4 months old FCh came with history of reddish pin point spots on body & a
greenish blue patch on left side of abdomen for last 10 days.
O/E- Cervical & Axillary Lymph Nodes were enlarged.
Liver 5cm, Spleen 4cm
• Lab Findings – Hemoglobin 8.9 g/dl
Platelet Count 11,000
TLC 67,540
Blast Cells 80%

54. ACUTE LEUKEMIA

55. BLOOD VESSEL DISORDERS
•Henoch Schonlein Purpura
Palpable Purpura ( more on the lower limbs, buttocks – gravity dependent
)
MusculoSkeletal Involvement ( Arthritis, Arthralgias )
GI Manifestations ( Pain, Vomiting, Diarrhoea, Paralytic Ileus, Malena )
Renal Involvement ( Microscopic Hematuria, Proteinuria, Nephritis/Nephrotic
)
Neurologic Manifestations
• T/T – Oral Corticosteroids
IVIG

56. •Ehler Danlos Syndrome
Connective tissue disorder at the level of Collagen structure & function.
Bleeding manifestations more common in Type VI (VASCULAR TYPE)
Tissue fragility & arterial rupture is seen
Majority suffer from a major vascular event within 20yrs of age.
Rupture of great vessels, Dissecting Aortic aneurysm common
Associated with high morbidity & mortality.

57. REFERENCES
1. NELSON TEXTBOOK OF PAEDIATRICS
2. CLINICAL PAEDIATRICS, ARUCHAMY
3. PRACTICAL ALGORITHMS IN PAEDIATRIC HEMATOLOGY & ONCOLOGY, R. H. SILLS

58. THANK YOU.