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Immune Thrombocytopenic Purpura

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Immune

Thrombocytopenic Purpura
IMMUNE THOMBROCYTOPENIC PURPURA

Name – Tharusha Dewuruwan


Age - 2 years 9 months
Sex - Boy
Address- Panadura

P/C – As requested by the GP due to low


Plt count, when investigating for blood
stained stools.
HPC-
 The child was quite well 3weeks back.
 He developed blood coated loose stools with mucus, 3times/day , lasted for
3days.
 Mild fever for 1day.

no chills or rigors.
responded to PCM.
 Mild abdominal pain at the onset of loose stools, lasted few minutes.

no vomiting.
 They have taken treatment from a GP. (Milcef 125mg bd) antibiotic syrup given.
 Condition settled on day3.
 Had normal bowel habits for 2weeks.
 After 2weeks, he developed 2nd episode of blood stained stools for 1day
duration, with mild fever. (101F)
 Mother noticed a bluish patch on the back of the child, which appeared
after falling from a swing.
 FBC was done by GP- Platelet count was 72,000/mm3 .
Differential diagnosis-

1. DHF
2. ITP
3. ALL
4. Drugs
5. HUS
6. WVD
7. DIC
8. Non accidental injuries
9. Rectal Polyp
Systemic inquiry-
Abdomen-mild abdominal pain, LOA
No vomiting, malena or gum
bleeding.
Skin- No Rashes
Rs –No cough, cold, wheezing, sob
ENT- No sore throat, epistaxis
CVS- No dyspnoea on exertion, palpitation,
Musculoskeletal- No joint pain
Renal- No haematuria
CNS- No fits, headache

No contact history of fever or diarrhoea


No outside food was taken.
PMH-
Acute
 gastroenteritis at 1 year 6 months.
No Bleeding disorders, fits, BA,
No history of measels, mumps, Rubella
Immunization H-
Up to date
JE and Hib vaccine not given.

Drug History-
Cefalexin 3weeks back.

FH-
No bleeding disorders
No consanguinity
No connective tissue or autoimmune disorders

SH-
Mother-28years, house wife
Father- 32years , mason
Both educated up to O/L
Nearest hospital- Panadura base hospital, can reach within 20 minutes by Three wheeler.
O/E-
 Looks well & active
 Weight 13.75kg (25th-50th centile)
 Height 97cm (75th-91st centile)
 Not pale
 Ecchymotic patches- back
right shoulder
B/L shins
bluish in colour
1-2 cm in diameter
 Bruises around venepuncture sites.
 No lymph node enlargement.
 ENT- no tonsillitis
no oral ulcer
no ear discharge
 Abdomen- tenderness over right iliacfossa
no hepatospleenomegaly
 Other systems- normal
Investigations-
 FBC- WBC- 10600

N-67%
L -20%
M-6.9%
E -1.7%
B -3.5%
Hb- 13.3g/dl
Plt -16000/mm3
PCV-38.6%
 Repeated FBC done- Plt -16000/mm3

35000/mm3
20000/mm3
57000/mm3
 SGOT- 40 u/l
 SGPT- 15 u/l
 PT - 11.9sec
 INR- 1.06
 APTT- test -32.7sec
control -35sec
 BT - 3 ½ min
 CT - 3min
 ESR -40min- 1st hour
 Hess’s test (+)ve
 Mono spot (-)ve
 Ds DNA (-)ve
 Blood picture-
RBC- normocromic normocytic
WBC-mild eosinophilia
few reactive lymphocytes
PLT -low
few large forms
 Flexible sigmoidoscopy- when Plt >50000/mm3
 ANA
Management
 Initially managed as blood & mucus diarrhoea.(furazolidone 50mg/6h given)
 QHT
 PCM 200mg/6h
 ORS- 50-100cc/loose stool
 FFP 140cc given when Plt count 16000/dl
 After hematological referral, management plan-
 To exclude 2ry causes -SLE screen
-USS abdomen
-ESR
-CRP
-drug history
-renal &liver functions test
 If bleeding + Plt <20000/mm3 - give IV Immunoglobulin(Ig 0.4g/kg/day)for 2-
3days
 Delay steroids, unless thrombocytopenia become chronic
 Bone marrow aspiration to exclude hematological malignancies
 Follow up with regular counts
 Review at the hematological clinic
Thrombocytopenia?
Reduction in platelet count
Purpura
◦ Occurs when platelet count
<20000/mm3
What causes thrombocytopenia?

Thrombocytopenia

Increased consumption

Bone marrow failure


Immune
Splenomegaly
Non immune

Primary
ITP Secondary - DIC
- TT P
- HUS
- L ive r di se ase
What Is Immune
Thrombocytopenic
Purpura?
Pathogenesis of ITP
( auto immune disorder, post viral)

Antibodies binds to platelets

Antibody coated platelets binds to the fc receptors of the macrophages in
the reticular endothelial system

Diminished t1/2 of the platelets in reticular endothelial system
IgG- spleen
IgE-liver
What are the presentations of ITP?
Acute Chronic
Onset acute Onset insidious
Lasts 4-6 weeks Lasts >6 months
Peak age of incidence is 2-6 years. Can occur in all ages
Sex F:M=1:1 Sex F:M=3:1
Presents as Bruising, petichial Presents with Prolonged history of
hemorrhages and bleeding from mucosal bruising and bleeding.
surfaces. Bleeding is less severe.
Severity of bleeding is less even with
Association with SLE, Rheumatoid
lower counts compared to marrow
disease and other collagen disorders.
failure due to functionally superior
In 10%-20% natural remission.
juvenile platelets.
Death is mainly due to uncontrolled
bleeding\ICH.
In 80% patients spontaneous remission
within 1-2 weeks.
Eosenophillia \ thrombocytosis common.
Eosenophillia \ thrombocytosis rare.
Bleeding manifestations in ITP..
Diagnosis of ITP…
Mainstay of the diagnosis is the Typical history, examination and
exclusion of secondary causes.

FBC- Platelet count reduced <20,000/ mm3 is


the only abnormality.
Other counts normal.
Hb normal.

 Blood picture- Low platelets which are increased or normal


in size.

Bleeding time- is prolonged but APTT and


PT are normal.
Bone marrow aspirate-
- Done before starting steroid
treatment.
- Shows increased or normal
number of megakaryocytes.
- Platelet turnover is increased.

Screening for secondary causes-


-ANA
-Coombs
-Double stranded DNA
-HIV screening
-Anti platelet antibodies
How to manage ITP?

Management of ITP

Specific Treatment Supportive treatment


Specific treatments
Corticosteroid therapy
mode of action
1. Inhibit platelet antibody production
2. Inhibit interaction between antibodies and platelets
3. Prolong platelet survival
4. Increase vascular stability
 Given if bleeding is present
 Only a short course of prednisolone is given 1-2mg/kg
over 2-3 weeks and should be discontinued irrespective of the
platelet count
(because response after ) this is unlikely
 Iv methyl prednisolone pulse therapy is more effective (given
for 3 days)
• Splenectomy

spleen is the main site of antibody production against platelets


as well as main site of destruction of platelets
indications
1. >4years child with severe ITP for >1 year Symptoms not
controlled by therapy
2. Life threatening hemorrhages if platelet count cannot be
controlled with platelets
• children after splenectomy are more susceptible to develop
post splenectomy sepsis secondary to infections by
encapsulated organisms. Risk is much higher if <5years.
• Meningococcal, pneumococcal, Hib vaccine should be given
prior to splenectomy
• Prophylactic penicillin to prevent gram +ve infections
IV Immunoglobulin

 Increase the platelet count by blocking Fc


receptors and protect the plt from the plt
antibodies.
 Some children respond within 48hrs
[rapid responders].
 Dose- 0.4mg/kg for 5 days
1mg/kg for 2 days
 Side effects- Vomiting, headache.
Supportive treatment

Treatment

Advice
- Usually remits spontaneously
-Side effects of steroids Restriction of physical Platelet transfusion
-Prevent trauma
-If headache/ vomiting admit immediately activity/outdoor in life threatening
-If splenectomy indicated vaccination
-Avoid NSAIDS games bleeding tendencies
-Avoid IM injections
Presentedby
Lopali
Shanaka
Rasangika
Prasad

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