Overview
Definition:
Idiopathic Thrombocytopenic Purpura (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia (low platelet count) in the absence of any other identifiable cause, leading to increased bleeding risk
In children, it is often acute and self-limiting, typically occurring after a viral infection.
Epidemiology:
ITP is the most common acquired bleeding disorder in childhood, with an incidence of approximately 3-8 per 100,000 children per year
It can occur at any age, but peaks are seen between 2-6 years and in adolescence
There is no significant sex predilection in younger children, but adolescent girls may be more commonly affected.
Clinical Significance:
Understanding activity restrictions and bleeding precautions is crucial for preventing serious hemorrhagic complications in children with ITP
These measures are vital for guiding parents and caregivers, ensuring safe participation in school and daily activities, and minimizing the risk of trauma-induced bleeding
For DNB and NEET SS preparation, this topic frequently appears in clinical scenarios requiring practical management advice.
Clinical Presentation
Symptoms:
Easy bruising (ecchymoses)
Petechiae, often on lower extremities
Nosebleeds (epistaxis) that are difficult to control
Bleeding gums
Blood in urine (hematuria) or stool (melena)
Heavy menstrual bleeding in adolescent females
In severe cases, intracranial hemorrhage (rare).
Signs:
Isolated thrombocytopenia (<100 x 10^9/L) on peripheral blood smear
Normal or slightly enlarged spleen
Absence of fever, lymphadenopathy, or hepatosplenomegaly suggesting other causes of thrombocytopenia
Petechiae and purpura visible on skin and mucous membranes
Vital signs may be normal unless significant blood loss is present.
Diagnostic Criteria:
Diagnosis is primarily based on clinical presentation and laboratory findings
Criteria include: isolated thrombocytopenia (<100 x 10^9/L) with normal white blood cell and red blood cell counts
absence of fever, lymphadenopathy, hepatosplenomegaly, or congenital anomalies
normal coagulation profile (PT, aPTT)
and absence of another identifiable cause of thrombocytopenia (e.g., infections, toxins, other autoimmune diseases)
Presence of antiplatelet antibodies can support the diagnosis but is not routinely required.
Management Activity Restrictions
Platelet Count Thresholds:
Activity restrictions are guided by the platelet count and bleeding severity
Generally: Platelet count >50 x 10^9/L: No restrictions, normal activity allowed
Platelet count 20-50 x 10^9/L: Avoid contact sports, high-risk activities, and unsupervised play
Encourage less vigorous activities
Platelet count <20 x 10^9/L: Strict avoidance of contact sports, rough play, and any activity with a risk of head injury or significant trauma
Supervised activities only
Platelet count <10 x 10^9/L: Consider hospitalization and stricter precautions
Avoid all contact sports and activities with even minor risk of injury.
School And Extracurricular Activities:
When platelet counts are acceptable, children can attend school and participate in most activities
However, coaches, teachers, and parents must be informed
Contact sports (e.g., football, rugby, martial arts) are generally contraindicated until platelet counts are consistently above 50 x 10^9/L
Activities like swimming, cycling (with helmet), and team sports with less physical contact may be permissible with lower counts, depending on individual assessment.
Head Injury Prevention:
This is paramount
Children with ITP, especially those with platelet counts <20 x 10^9/L, must wear helmets for cycling, skateboarding, and other activities that pose a risk of head trauma
Parents should be educated on recognizing signs of intracranial hemorrhage (severe headache, vomiting, altered mental status, focal neurological deficits) and seek immediate medical attention.
Travel And Environmental Considerations:
Travel to remote areas or places with limited access to medical care may require careful planning, especially if the child has a lower platelet count
Advise parents to carry relevant medical information and a supply of any prescribed medications
Avoid activities that increase the risk of injury in potentially hazardous environments.
Management Bleeding Precautions
Home Safety Measures:
Childproof the home to minimize accidental injuries
Remove sharp objects, sharp corners on furniture, and tripping hazards
Ensure adequate lighting
Supervise young children closely
Use soft bedding and appropriate safety gates.
Medication Avoidance:
Strict avoidance of aspirin, NSAIDs (like ibuprofen), and other antiplatelet or anticoagulant medications is essential, as they can exacerbate bleeding
Acetaminophen (paracetamol) is the preferred analgesic for pain management.
Oral Hygiene:
Gentle oral hygiene practices are recommended
Use a soft-bristled toothbrush and avoid vigorous brushing that can lead to gum bleeding
If mouth sores or bleeding gums are significant, consult the hematologist.
Managing Minor Bleeding:
For minor cuts and abrasions, apply direct pressure for 5-10 minutes
For nosebleeds, have the child sit upright, lean forward, and pinch the soft part of the nose for 10-15 minutes
If bleeding is prolonged or severe, seek medical advice
Apply ice packs to bruises to help reduce swelling and pain.
Diagnostic Approach
History Taking:
Key history points include the onset and duration of symptoms, presence and pattern of bleeding (nosebleeds, gum bleeding, bruising, hematuria, melena), recent infections (viral prodrome), immunization history, family history of bleeding disorders, and any medications taken
Red flags include severe headache, vomiting, altered consciousness, focal neurological signs, abdominal pain, or significant blood loss.
Physical Examination:
Focus on a thorough skin and mucous membrane examination for petechiae, purpura, and ecchymoses
Assess for signs of active bleeding
Palpate the abdomen for splenomegaly or hepatomegaly
Perform a complete neurological exam, especially if there are concerns for intracranial bleeding
Check vital signs, including heart rate and blood pressure, to assess for hypovolemia.
Investigations:
Complete Blood Count (CBC) with differential: Essential to confirm isolated thrombocytopenia and rule out other cytopenias
Platelet count is critical
Peripheral Blood Smear: To assess platelet morphology and rule out pseudothrombocytopenia or other causes of microangiopathic hemolytic anemia
Coagulation Profile (PT, aPTT, INR): To ensure normal coagulation and rule out disseminated intravascular coagulation (DIC)
Bleeding Time: Historically used, but less common now due to variability
platelet function analyzers can assess platelet aggregation
Bone Marrow Aspiration (optional): Reserved for cases where diagnosis is uncertain, to rule out other bone marrow disorders, or in children not responding to standard therapy
It typically shows normal or increased megakaryocytes.
Differential Diagnosis:
Conditions to consider include viral-induced thrombocytopenia, drug-induced thrombocytopenia, immune thrombocytopenias secondary to other autoimmune diseases (e.g., SLE), thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), leukemia, and aplastic anemia
Distinguishing features include the presence of other cytopenias, fever, organomegaly, neurological symptoms (TTP/HUS), or a clear history of drug exposure.
Management Medical
Observation Vs Treatment:
For children with mild ITP (platelet count >20-30 x 10^9/L) and no significant bleeding, observation may be appropriate, as spontaneous remission is common
Treatment is indicated for platelet counts <20-30 x 10^9/L, severe bleeding, or impending risk of severe hemorrhage
The goal is to rapidly increase platelet counts to a safe level.
Pharmacological Treatment:
First-line therapy typically includes: Corticosteroids: Prednisolone 1-2 mg/kg/day orally for 2-4 weeks, followed by a tapering dose
Intravenous Immunoglobulin (IVIG): 1 g/kg/day for 1-2 days or 0.8-1 g/kg as a single dose
This provides a rapid, albeit transient, increase in platelet count, useful for severe bleeding or before procedures
Platelet Transfusions: Reserved for life-threatening bleeding or active intracranial hemorrhage, as they are transient and may induce alloimmunization.
Second Line Therapies:
If first-line therapies fail or for chronic ITP, second-line options include: Romiplostim or Eltrombopag (thrombopoietin receptor agonists) - used in refractory or chronic ITP in older children, carefully monitored
Rituximab - an anti-CD20 monoclonal antibody
Immunosuppressants like Azathioprine or Cyclosporine.
Splenectomy:
Rarely considered in children with chronic, refractory ITP that has failed multiple medical treatments
It is usually a last resort due to increased risk of infection.
Prognosis
Factors Affecting Prognosis:
Most children (80-85%) achieve remission within weeks to months
Chronic ITP (persisting beyond 12 months) is less common, occurring in about 15-20% of cases
Factors associated with chronic ITP include older age at diagnosis, severe bleeding at presentation, and low initial platelet counts
The risk of severe bleeding, particularly intracranial hemorrhage, is low but significant.
Outcomes:
The vast majority of children with acute ITP recover fully with no long-term sequelae
In chronic ITP, management focuses on maintaining a safe platelet count to prevent bleeding, and the quality of life can be affected
Recurrence of ITP is possible but uncommon.
Follow Up:
Children with acute ITP should have regular follow-up with their pediatrician or hematologist to monitor platelet counts and assess for resolution of bleeding
For those with chronic ITP, long-term follow-up is necessary to manage the condition, monitor for treatment side effects, and assess for potential complications like infections.
Key Points
Exam Focus:
DNB/NEET SS exam questions often focus on differentiating ITP from other causes of thrombocytopenia, immediate management of bleeding, appropriate activity restrictions based on platelet count, and the role of steroids vs
IVIG
Understanding the safe analgesic choice (paracetamol over NSAIDs) is also key.
Clinical Pearls:
Always ask about recent viral illnesses
Educate parents thoroughly on activity restrictions and when to seek urgent care
Remember that platelet transfusions are generally reserved for life-threatening bleeding, not for raising platelet counts to facilitate procedures unless absolutely necessary and closely monitored.
Common Mistakes:
Over-treating mild ITP without significant bleeding
failing to adequately educate parents on precautions
prescribing NSAIDs or aspirin for pain management
missing red flags for intracranial hemorrhage
or not considering bone marrow biopsy in atypical presentations.