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1. Presenter- Dr. Ashok Das

2. Platelet disorders
Coagulation disorders
Vascular Abnormality/Vasculitis

3. Decreased Platelet Synthesis
Congenital:- TAR syndrome
Wiskott Aldrich Syndrome
Fanconi Anemia
X-linked amegakaryocytic
Storage disorders
Cyanotic heart disease

4. Decreased Platelet Synthesis
Acquired:- Infections-
Medications
Toxin
Radiations
Marrow suppression
Marrow infiltration
Vit B12, Folate deficiency
Aplastic anemia

5. Increased Platelet Destruction
(Destructive/Consumptive)
Immune :-
ITP
SLE
Drugs
HIV
Non-Immune:-
Microangiopathies
DIC
Neonatal sepsis
NAIT
Thrombosis
Type 2B/Platelet type vWD
Sequestration
Kassabach merritt syndrome

6. Immune Thrombocytopenic Purpura
Diagnosis of exclusion.
Management doesnot affect natural history of ITP.
Mild-moderate ITP:- Wait & watch
Family education & counselling
No NSAIDs
No violent contact/sports
No i.m injection
vaccinations

7. Severe ITP:-
1st line- IVIG or Corticosteroids or Anti D Ig single dose
 2nd line Agents-
•TPO-RA- Eltrombopag
Romiplostin (1-10 mcg/kg sc once a week)
• Rituximab
• Dapsone/Azathioprine/Danazole/MMF/Cys/Cyclophosphamide
Splenectomy

8. Treatment of ICH in ITP
Outcome: 80% case resolves within 3 month, 20 % progress to chronic ITP, <1% develop ICH
IVIG/anti D
+
Methyl-prednisolone
+
Supranormal dose of Platelet
(30ml/kg)
+
IV Tranexamic acid
(10-15mg/kg qds)

9. Drug induced Thrombocytopenia
Formation of Drug dependent antibodies
1-3 weeks after starting of drugs.
Diagnosis is clinical.
Common drugs:- Anti epileptics,Antibiotics-
Linezolid,vancomycin,Quinine,TMP-
SMX,Sulfonamide,Rifampicin,Piperacillin
Chemotherapy,Procainamide,Thaizide,heparin.
Resolves within 7-10 days after stopping offending drug.

10. FEVER
MAHA
Thrombocytopenia
Abnormal renal function Neurological abnormalities
Thrombotic
Thrombocytopenic
Purpura

11. Management of TTP
 Acquired (95%):-
 Plasma infusion (30ml/kg) until plasma exchange(effevtive 90-
95%) canbe started.
 IVIG
 Rituximab
 Corticosteroids
 Immunesuppresant-Cyclophosphamide,Azathioprine
 Monoclonal Antibody- Caplacizumab:Anti vwF nanobody.
Congenital TTP (5%)treated by repeated infusion of FFP.

12. Hemolytic Uremic Syndrome
 Clinical triad of MAHA,Acute Renal insuffiency,Thrombocytopenia.
 D+ HUS: Early and repeated dialysis , supportive care including anemia
correction,infection treatment and HTN control.
 Atypical HUS: Eculizumab
PEX only alternative.
Plasma infusion given Immunosuppression:
every 10-14 days Prednisolone
Cyclophosphamide
After PLEX is done
Anti FH+

13. Congenital Platelet Function Disorder
Others- Dense body deficiency
Grey platelet sydrome
Treatment:-Platelet transfusion during bleeding episode
Desmopressin o.3 mcg/kg iv
Definitive-SCT
Bernard soulier syndrome Glanzzmann
thrombasthenia
Inheritance AR AR
Defect GP Ib complex
(PLATELET-VVF receptor)
GP IIb-IIIa
(Platelet-fibrinogen receptor)
Platelet size & BT Big >11fl , BT-increased PL- normal size, BT-
prolonged
Platelet aggregation test RIPA-Absent
Normal with others agonist
RIPA-normal
Absent with others.

14. Acquired Platelet Function Disorder
• Uremia-coagulopathy is an indication for dialysis
• Anti-platelet drugs- Aspirin

15. Hemophiloia A/B
SEVERITY FACTOR LEVEL
SEVERE <1% Spontaneous bleeding
MODERATE 1-5% occasinal joint bleed
Trauma/Sx
MILD >5% Rare joint bleed
major trauma/sx

16. • Diagnosis:-
• Clinical- Hemarthosis,Muscular haemorrhage
• Investigation- APTT: 2-3 times ULN
CBC,PBS,PC,TT,BT-normal
●Specific factor assay
●Mixing study
●Bethesda Test

17. Type of Haemorrhage Hemophilia A Hemophilia B
Mouth
Nose
Muscular
20IU/kg
may need alternate day till
resolution
40IU/kg
may need every 2-3 day interval
till resolution
Hemarthosis 40IU/kg on D1
20IU/kg on D2,4 until resolved.
Additional dose on alternate day
for 7-10 day if symptoms persists
80IU/kg on D1
40IU/kg on D2,4 until resolved.
Additional dose on alternate day
for 7-10 day if symptoms persists
Life Threatening haemorrhage
(ICH.,upper airway
bleeding,massive git bleeding)
60IU/kg factor VIII concentrate
then start infusion @ 2-4 IU/kg/hr
to maintain level >100IU/dl for 24
hours.
120IU/kg factor VIII concentrate
then 60 IU/kg every 12-24 hourly
to maintain factor level > 40 IU/kg

18. • Desmopressin: (Stimate)
effective only in mild hemophilia A (>5%)
Dose:- 1 puff (150mcg) if BW<50kg
2 puff (300mcg) if BW>50kg
:- Avoid violent trauma/contact,sports,NSAIDs
vaccinations
initiated after first episode of haemorrhage
20IU/kg thrice weekly for factor VIII
20IU/KG twice weekly for factor IX

19. Chronic Complications:- Chronic arthopathy
Development of inhibitor
Risk of transfusion related infections
 Treatment of Inhibitor:-
1.Desensitization program: high dose factor VIII & IX infused to saturate
inhibitor
2.Rituximab
3.FEIBA (50-100U/kg)
4.Recombinent factor VIIa.

20. Hemophilia C
AR
mild-moderate bleeding
aPTT is longer than it is in pt with Hemophilia A/B
Treatment:- FFP 10-15 ml/kg which increase plasma level
of factor IX is 20-30%

21.  Factor VII deficiency: FFP is ineffective.
Recombinent factor VIIa given.
 Congenital afibrinogenemia/dysfibrinogenemia: AR
Prolongation of PT,APTT,TT,RT
Treatment- FFP/cryoprecipitate
1 unit of cryoprecipitate contains 100-150 mg of factor I
 Factor XIII deficiency:-Delayed haemorrhage d/t instability of clot.
H/o delated u.cord separation
poor wound healing
Diagnosis:-Clot solubility positive in presence 5m
urea.
Treatment- FFP/cryoprecipitate

22. Acute Coagulation Disorders
 Liver disease
Vitamin K deficiency
Acquired inhibitor of coagulation factors

23. Acute Coagulation Disorders Management
Liver disease  FFP (10-15ml/kg)
 Vit K
 Desmopressin 0.3 mcg/kg iv
Vitamin K deficiency results in decrease synthesis of
vitamin k dependent facors.
seen in neonate,long term
antibiotics,intestinal
malabsorption,liver disease.
 Infants- 1-2 mg
 Children- 2-3 mg
 Adolescent- 5-10 mg
 max 3-5 dose.
Acquired inhibitor of coagulation
factors
LA in SLE
HIV

24. von Willebrand disease
 Most common hereditary bleeding disorder
 Presents with epistaxis,gum bleeding
prolonged bleeding after surgical procedure
excessive menstrual bleeding in girls
 Blood inv:- Platelet count,PT-normal
BT,aPTT-prolonged,PFA-altered
factor VIII-decreased

25. TYPE 1 TYPE 2 TYPE 3
AD AD AR
Most common (60-80%)
mild symptoms
Moderate bleeding Rare (<5%)
severe bleeding resembles
severe hemophilia A
partial deficiency of vWF Abnormal variant of vWF Complete deficiency of vWF
Type 2A Type 2B Type 2M Type 2N
(Autosomal
hemophilia)
Platelet type vWD
Absence
HMW
multimer
Increased platelet
binding function
of vWF
Decreased
platelet binding
function of vWF
Decreased factor
VIII binding function
of vWF
Increased platelet
binding function d/t
mutation in GP Ib.
Classification of vWD