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IMMUNETHROMBOCYTOPENIC

PURPURA(ITP)

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Usually acute, self-limiting disorder that resolves spontaneously.ISOLATED thrombocytopenia with otherwise normal blood counts withno clinically apparent alternate cause thrombocytopenia.

Clinical onset : acute with a spectrum of bleeding severity rangingfrom superficial to life-threatening.

Clinical Manifestation:i) Cutaneous bleedingii) Mucosal bleeding

iii) Hepatosplenomegaly and lymphadenopathy is absent

Majority will give history of a viral infection in the preceding 2-4 weeks.75% - remit spontaneously with 70% achieving platelet count of morethan 50 x 109/L by the 4th week of illness.

Diagnosis:Can be made clinically based principally on the

i) Historyii) Physical Examination (no stigmata of malignant disease etc)iii) Full blood count (isolated thrombocytopenia)iv) Peripheral blood smear (exclude abnormal cells,

megakaryocytes)

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HistoryShould focus on

1) factors that suggest another disease for whichthrombocytopenia is a complication

2) Signs and symptoms that differentiate mild, moderate andsevere bleeding tendencies

Other systemic illness- may manifest as a primary immune deficiency syndrome

Post-viral illness

- the severity of the dz not correlate with the degree ofthrombocytopenia.HIV infection

- may occur during the acute retroviral syndrome coincidentwith fever, rash and sore throat. Drug induced thrombocytopenia

- hx of all prescriptions and over the counter mediacations- eg: quinine and quinidine, heparin (routine flushing of IV

catheters), etc- other drugs associated with purpura include antibiotics, gold

salts, analgesics, neuroleptics, diuretics and antihypertensive. Bleeding tendencies

- TRO ICH headache, LOC, blurred vision- should re ort an hx of recent head trauma

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Physical Examination1) Findings that suggest another disease for which

thrombocytopenia is a complication2) That suggest serious internal bleeding

General health- ITP is a primary illness occurring in an otherwise healthy

person- signs of chronic disease, infection, wasting or poor nutrition

Vital signs- hypertension and bradycardia, tachycardia

Skin and mucous membrane- petechiae and ecchymoses, oozing from venepuncture site,

gingival bleeding and hemorrhagic bullae.- mild with relatively low risk of serious bleeding complication

may manifest as petechiae over the ankles in patients who areambulatory or on the back in patients who are bedridden.

Cardiovascular system

- distant low amplitude heart sounds accompanied by jugularvenous distention haemopericardium

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Abdomen- The presence of readily palpable spleen is not typical

Nervous System- asymmetrical finding- pupils size, extraocular muscles and eyes movement- balance and gait- funduscopic examination optic disc, retinal

haemorrhages

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Bone Marrow Examination :Indication :

- Atypical features : organomegaly, significantlymphadenopathy, abnormal blood counts, suspiciousperipheral blood smear.

- Before starting steroid therapy- Failure to respond to Immunoglobulin therapy- Persistent thrombocytopenia >6 months- Thrombocytopenia recurs after initial response to

treatment.

indicated if the patient is not responding to therapy or beforestarting steroids.Seldom required. Less than 4% of 127 children had a differentdiagnosis from ITP

Other Investigationsi) ANA and DNA Antibodies progressing to Chronic ITPii) CMV Serology under 1 year ageiii) Immunoglobulins recurrent infectioniv) HIV screening at risk (HIV parents)

v) Coagulation profile non-accidental injury, inheritedbleeding

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MANAGEMENT OF ACUTE ITP

1.Outpatient observation and monitoring those with platelet count >20x 109/L without bleeding tendencies.

+ precautions with physical activities, avoidance of contactsports and seeking immediate medical attention if bleeding occurs.(CPG)

repeat blood count within 7-10 days

Hospitalization is indicated if :i) severe life-threatening bleeding regardless of platelet countsii)platelet count

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Choice of treatment :

(Clinical Practice Guideline-2006)

IVIG 0.8g/kg as a single dose OR

Oral Steroids( Oral Prednisolone 2mg/kg/day for not more than 14

days OR

Oral Prednisolone 4mg/kg/day for 4 days.)

(Paediatric Protocol-2008)

Oral prednisolone 4mg/kg/day for 7 days, then taper anddiscontinue at 21 days

IV Methylprednisolone 30mg/kg/day for 3 daysIV Immunoglobulin (IVIG) 0.8g/kg/dose for 1 day or 250mg/kg

for 2 daysIV Anti-Rh(D) immunoglobulin (50-75ug/kg) in Rhesus positive

patients

Both may shorten the thrombocytopenic phase in responsive cases butdo not influenced the outcome of disease.

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EMERGENCY TREATMENT

Serious bleeding eg: severe epistaxis, GIT bleeding or bleeding

causing a drop in Hb was seen in 17% cases. The most serious formof bleeding i.e Intracranial bleeding has a very low incidence of 0.1%to 0.5%.

Platelet transfusion-Need a rapid increase. Immediate administration of a larger than

usual (8-12 units per sq meter) transfusion of donor platelets plus IVmethylprednisolone 30mg/kg (maximum 1gm) over 20-30minuteshave been advocated.

Intravenous Methylprednisolone-Usual dose used is 30mg/kg/day for 3 days

Intravenous Immunoglobulins (IVIG)- Single dose of 0.8g/kg or 1g/kg has been shown to be as effectiveas doses of 0.25-0.5g/kg/day for 2 days in a randomized multicentrestudy.

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Intracranial Hemorrhage

50% mortality rateRisk of ICH highest with platelet count

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Management of Chronic ITP

Chronic ITP in children is defined as persistent thrombocytopeniaafter 6 months of onset, is strikingly different from that adults withcomplete, spontaneous remission seen in the majority.Occurs in 20%

As far as possible, allow the disease to remit spontaneously.However, other causes of thrombocytopenia eg: SLE should be

considered.

Asymptomatic, can be left without therapy with advice regardingprecautions during physical activities, contact sports,dental/surgical procedures and menses.

Symptomatic, may need short courses of treatment (as for acuteITP) during periods of relapse or for surgical proceduresIntermittent pulses of IVIGIntermittent anti-Rh(D) antibody treatmentIntermittent pulses of steroids

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(CLINICAL PRACTICE GUIDELINE)

For those with persistent bleeding problems, second line therapiesinclude

- Steroids= dexamethasone (oral 1mg/kg) given on 4

consecutive days every 4 weeks for 4 months OR= Oral methylprednisolone for 7 days - (30mg/kg) for

3 days followed by 20mg/kg for 4 days AND- Anti D immunoglobulin 45-50ug/kg Ig in Rh positive

patients.

SECOND LINE THERAPY SHOULD ONLY BE STARTED FOLLOWINGCONSULTATION WITH A PAEDIATRIC HAEMATOLOGIST.

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REFRACTORY ITP

Splenectomy is rarely indicated in children with ITP as spontaneousremissions continue to occur up to 15 years from diagnosis.

Risk of mortality from :- ITP (0.002%)- post splenectomy sepsis (1.4-2.7%)

Splenectomy is indicated when:

- persistence of disease after 12 months with- bleeding symptoms AND- platelet count

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For post-splenectomy failure or relapse, consider:

-danazol, vincristine, azathioprine, cyclophosphamide, alpha-interferon,staphylococcal protein A immunoadsorption, cyclosporine, colchichine or

dapsone.

Children who reach this stage should be managed by a paediatrichaematologist.

There is insufficient evidence to recommend third-line

immunomodulating agents.(Grade C)

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NEONATAL CARE

Overall incidence of thrombocytopenia in newborns born to mothers

with ITP is reported to vary from 14.3 to 37.5%

Prediction of neonatal thrombocytopenia is difficult, and there is norelationship with maternal platelet counts. Infants fromsplenectomized mothers were more likely to have thrombocytopeniaas were mothers who had presence of circulating antiplatelet antibody.

Thrombocytopenia is more likely if there is a previous sibling withthrombocytopenia.

Cordocentesis and foetal scalp sampling to measure foetal plateletcounts carry more risks than potential benefits and are not

recommended. The application of scalp electrodes for monitoring inlabour room should be avoided.

Cord blood platelet count should be done and the neonate withthrombocytopenia should be monitored daily.

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References :

1.Clinical Practice Guideline Management of ITP August 20062.Paediatric Protocols 2008

3.Emedicine-ITP

4.EMJ- Emergency Medicine Journal BMJ- Management of allergy,

rashes and itching 2004

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