IMMUNE THROMBOCYTOPENIC PURPURA (ITP)By : FIKRI ABDULLAH ZAWAWI KATHIRAVAN KANIASAN

CASE REPORTName : Kudryova JulieAddress : Kurovice 37,76s 52 Miskovice U HolesovaDate of Birth : 31.5.2012Weight : 18.5kgHeight : 105cm

Current ComplaintsShe came to the hospital due to

planning of trepanobiopsy (bone marrow aspiration and biopsy)

Previously she was diagnosed with chronic ITP

She has weak cold and afebrile

Personal HistoryLabor was spontaneous in 42nd weekShe has postpartum complication due

to knotted umbilical cord around her neck

She was in neonate ICU for 14 days with ventilatory support

She was breastfed for 8 months and vaccinated according to schedule

She had varicella infection at 8 months

From September 2013 onwards her mother noticed some bruising on her body.

In March 2014, she fell down and there was a significant hematoma on her head

She was diagnosed with ITP in July 2014 and acute bronchitis on October 2014

She received IVIG against ITPIn 26th January 2015 she was included in

the study of Romiplostim (protein analogue of thrombopoietin)

Last application was on 16th February 2016

Allergy : NoPharmacologic History : No

Social History: Sometimes she goes to kindergarten otherwise at home.

Live with her family and has pet Father is a smoker

Family HistoryHer grandmother had valvular

heart diseaseHer mother has varicose veinHer father healthyHer older sister has migraine

Stomatic Status She was afebrile, with runny noseNormal heart sound, skin without

pathology, normal peristalsis, no edema, no bruise

She has slight splenomegaly

Laboratory FindingLeucocytes: 9.47 10^9/lErythrocytes: 4.91 10^12/lHemoglobin: 111g/LHematocrit: 0.33MCV: 68.0 fLMCH: 22.6 pgRDW: 15.8%Platelets: 275 10^9

Differential AnalysisLymphocytes: 35.6%Monocytes: 9.3%Neutrophils: 52.6%Eosinophils: 2.3%Basophils: 0.2%

Theory Part

Immune Thrombocytopenic Purpura(ITP)The most common cause of

acute onset of thrombocytopenia in an otherwise well child

Estimated about 1 in 20,000 children

A recent history of viral illness is described in 50-65% of cases of childhood ITP

One - 4 wk after exposure to a common viral infection (eg EBV, CMV, rhinitis, parvovirus B19)

The peak age is 1-4 yr.ITP seems to occur more often in

late winter and spring after the peak season of viral respiratory illness.

Pathophysiology2 possible mechanisms :

1. An autoantibody bind to the platelet surface, circulating antibody-coated platelets are recognized by the Fc receptor on splenic macrophages, ingested, and destroyed.

2. Impaired production of the glycoprotein hormone thrombopoietin, which is the stimulant for platelet production. (reduction in circulating platelets)

Source = https://undertheguiseofglitter.wordpress.com/2015/07/14/all-my-issues/

https://quizlet.com/95852614/pathophysiology-chapter-03-hematopoietic-function-flash-cards/

Clinical ManifestationThe classic presentation of ITP is a

previously healthy 1-4 yr old child who has sudden onset of generalized petechiae and purpura

Often there is bleeding from the gums and mucous membranes, particularly with profound thrombocytopenia (platelet count <10 × 10^9/L).

The presence of abnormal findings such as hepatosplenomegaly, bone or joint pain, or remarkable lymphadenopathy suggests other diagnoses

Classification SystemITP is classified as: Class 1: No symptomes Class 2. Mild symptoms: Bruising and petechiae Occasional minor epistaxis Very little interference with daily living Class 3. Moderate: More severe skin and mucosal lesions More troublesome epistaxis and menorrhagia Class 4. Severe: Bleeding episodes—menorrhagia, epistaxis, melena—requiring transfusion or hospitalization - Symptoms interfering seriously with the quality of life

Prognosis Severe bleeding is rare (<3% of

cases)In 70-80% of children who

present with acute ITP, spontaneous resolution occurs within 6 mo

Fewer than 1% of patients develop an intracranial hemorrhage.

Approximately 20% of children who present with acute ITP go on to have chronic ITP

The outcome/prognosis may be related more to age, as:

ITP in younger children is more likely to resolve

The development of chronic ITP in adolescents approaches 50%.

Differential DiagnosisAutoimmune thrombocytopenia may be an

initial manifestation of : (Because ITP is a diagnosis of exclusion, it is important to rule out other causes of low platelets)

1. SLE2. Infections (HIV, EBV, CMV, varicella, parvovirus B19)3. Common variable immunodeficiency4. Hodgkin Lymphoma(rarely)5. Vaccinations (eg, MMR )6. Medications (anti-epilepsy)

7. Congenital Amegakaryocytic Thrombocytopenia (CAMT)8. Bernard-Soulier Syndrome = (deficiency of glycoprotein Ib (GpIb), the receptor for von Willebrand factor)9. Wiskott-Aldrich Syndrome (WAS)= (characterized by eczema, thrombocytopenia (low platelet count), immune deficiency)10. Glanzmann's thrombasthenia = (platelets contain defective or low levels of glycoprotein IIb/IIIa (GpIIb/IIIa) which is a receptor for fibrinogen. As a result, no fibrinogen bridging of platelets to other platelets can occur, and the bleeding time is significantly prolonged)

Management Initial approaches to the

management of ITP include the following:

1.No therapy other than education and counseling of the family and patient for patients with minimal, mild, and moderate symptoms, as defined earlier.2.Intravenous immunoglobulin (IVIG).3.Intravenous anti-D therapy (for Rh positive patients)

4. Prednisone (continued for 2-3 wk or until a rise in platelet count to >20 × 10^9/L achieved)5. TPO mimetics (Romiplostim)6. Mycophenolate mofetil (MMF)= for those unresponsive to corticosteroids and/or splenectomy7. Rituximab (monoclonal antibody against protein CD20)8. Immunosuppressant (azathioprine, cyclosporin A)9. Platelet transfusion in ITP is usually contraindicated unless life-threatening bleeding is present 10. Splenectomy in severe condition(age above 6 y/old and other treatments are not effective – before surgery need to give vaccination for pneumococcal, meningococcal and h.influenza B)

Reference (Immune Thrombocytopenic Purpura (ITP): A

New Look at an Old Disorder, 2010), Published by Indiana Hemophilia and Thrombosis Center

http://www.itpsupport.org.uk/childhooditp.htm

www.pdsa.org/about-itp.htmlhttp://

www.bloodjournal.org/content/106/7/2244?sso-checked=true