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Case study (28) – Immune Thrombocytopenic Purpura


Case study (28) – Immune Thrombocytopenic Purpura 


A 4-year-old child has a history of bruising on the face, neck, and lower limbs for 3 to 4 days. 

She has just recovered from a viral infection. 

On Examination: 


- Purpura on the legs and face 


- The spleen is not palpable. 

Laboratory Investigation: 


1. CBC: 


Hemoglobin (Hb) 119 g/L 

White blood cells (WBC) 9.3 X 109/L (neutrophils 37%, lymphocytes 61%) 

Platelets 9 X 109/L 

2. Coagulation study: 

- (PT) Prothrombin time 12 seconds (control 10−12 seconds) 

- (APTT) Activated partial thromboplastin time 35 seconds (control 30−40 seconds) 

- Fibrinogen 2.3 g/L (NR 2−4 g/L) 

3. Other investigation: 


Creatinine 72 mmol/L 


4. Peripheral blood smear: 



Questions: 

Q1. What is the likeliest diagnosis? 

Q2. What further tests are required? 

Q3. What treatment would you recommend? 

Answers: 

A1. The blood film shows thrombocytopenia, with no polychromasia, no red cell fragmentation and there is a normal neutrophil. 

The coagulation tests are normal, effectively excluding disseminated intravascular coagulation, and the creatinine is normal, excluding hemolytic uremic syndrome. 

Immune thrombocytopenia (ITP) is the likeliest diagnosis. 

A2. ITP in childhood is a self-limiting condition. 

If the child is in good condition, invasive examinations should be avoided, and parents should be reminded of the importance of observing signs of mucosal bleeding, such as from the nose and mouth. 

If there is mucosal hemorrhage, treatment should be considered, and other causes of thrombocytopenia should be reasonably excluded (drugs, including rubella infection, infectious mononucleosis, hepatitis C, bacterial infection). 

Hematological malignancy can manifest as thrombocytopenia, If the platelet count does not improve spontaneously within a few days and before treatment of ITP, hematological malignancies should be excluded. 

Bone marrow aspiration validated the presence of megakaryocytes (large, multinucleated cells), thus suggesting that platelets were destroyed.
 


The value of platelet antibody detection is far less than that of red blood cell detection. 

An anti-nuclear factor assay should be performed. The previous history of viral infection is consistent with ITP, although this is not universally visible. 

A3. Avoidance of injury and anti-platelet drugs, for example, aspirin. 

Most children recover spontaneously and do not need treatment. 

If the count does not recover spontaneously (for example, within 2 weeks), or if symptomatic bruising occurs, especially affecting the mucous membranes of the nose and mouth, treatment is required. 

The first-line treatment is prednisolone, which starts at 0.5 mg/kg/day and decreases based on response. 

Intravenous immunoglobulin (0.4 mg/kg/day) for 3-5 days is equally effective but is most suitable for non-responders. 

Thrombopoietin receptor analogs are a new class of drugs, used when steroids or intravenous immunoglobulin (IVIG) are ineffective. 

Romiplostim must be administered by parenteral injection, while Eltrombopag can be taken orally. 

Other possible immunosuppressive drugs (such as cyclophosphamide, azathioprine, mycophenolate mofetil, and anti-CD20 monoclonal antibody rituximab) are other possible methods but are resistant to ITP or relapse but usually in children Prohibited adults. 

It is best to avoid splenectomy in children under 5 years old. 

In any case, pneumococcal and Hemophilus influenzae type B vaccine should be vaccinated first, and then penicillin V should be taken orally for a long time as a preventive treatment to prevent infection.
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