Overview
Definition:
Fever and petechiae in children are alarming signs requiring urgent differentiation between life-threatening meningococcemia (a form of bacterial sepsis) and a more benign, though still significant, hematological disorder, Idiopathic Thrombocytopenic Purpura (ITP)
Meningococcemia is caused by Neisseria meningitidis and can rapidly progress to disseminated intravascular coagulation (DIC) and shock
ITP is an autoimmune disorder characterized by isolated thrombocytopenia and a tendency for bleeding, typically without fever or signs of systemic infection unless a co-existing viral illness is present.
Epidemiology:
Meningococcemia is rare but has a high mortality rate
incidence varies geographically but is more common in unvaccinated populations and during outbreaks
Peak incidence is in infants and young children
ITP is the most common acquired bleeding disorder in children, with an incidence of 2-8 cases per 100,000 children per year
It can occur at any age but is most common between 2-6 years, with a bimodal distribution (post-viral in younger children, bimodal again in adolescents/adults).
Clinical Significance:
Prompt and accurate diagnosis is paramount
Delay in recognizing meningococcemia can lead to rapid deterioration and death
Conversely, misdiagnosing ITP as meningococcemia can lead to unnecessary aggressive antibiotic therapy and inpatient admission
Understanding the distinct clinical features, diagnostic workup, and management strategies is crucial for pediatric residents preparing for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
Fever is common in both
Meningococcemia: sudden onset of high fever, headache, stiff neck, vomiting, photophobia, lethargy, irritability, myalgias
Petechiae often appear rapidly and may coalesce into purpura
ITP: bleeding manifestations are primary – epistaxis, gum bleeding, petechiae, purpura, menorrhagia, hematuria, melena
Fever is usually absent or low-grade, often preceding rash by days and related to a preceding viral illness.
Signs:
Meningococcemia: ill-appearing child, signs of meningitis or sepsis, characteristic petechial or purpuric rash (can be widespread, non-blanching)
Tachycardia, hypotension
Signs of shock or DIC (e.g., organ dysfunction)
ITP: normal or mildly febrile child, isolated petechiae and purpura, normal vital signs
Examination for signs of systemic infection should be negative
Lymphadenopathy and splenomegaly are typically absent in acute ITP.
Diagnostic Criteria:
Meningococcemia: Clinical suspicion confirmed by positive blood culture for Neisseria meningitidis
Presence of fever, petechial/purpuric rash, and meningeal signs or sepsis are highly suggestive
ITP: Diagnosis of exclusion based on isolated thrombocytopenia (platelet count < 100,000/µL) in an otherwise healthy child with normal coagulation studies and no other identifiable cause of thrombocytopenia
Absence of fever and signs of systemic infection is key.
Diagnostic Approach
History Taking:
Recent travel or exposure to known cases of meningococcal disease
Vaccination status (Hib, MenACWY, MenB)
Prodromal viral illness (URI, gastroenteritis) preceding rash by days to weeks
History of recent blood transfusions or medications
Family history of bleeding disorders or autoimmune conditions
Trauma or recent procedures
Use of NSAIDs or aspirin
Red flags: rapid onset of rash, high fever, lethargy, purpura fulminans.
Physical Examination:
Thorough assessment for signs of sepsis (toxic appearance, hypotension, tachycardia)
Careful examination of the skin for blanching vs non-blanching lesions
distribution and morphology of petechiae/purpura
Neurological examination for meningeal signs
Examination for source of infection (e.g., otitis, pneumonia, cellulitis)
Assess for bleeding from other sites.
Investigations:
Meningococcemia: Blood culture (essential, before antibiotics if possible)
Complete blood count (CBC) with differential (may show leukocytosis, but can be normal or low in overwhelming sepsis)
Coagulation profile (PT, aPTT, INR, fibrinogen, D-dimer) to assess for DIC
Lumbar puncture if meningitis is suspected (CSF analysis for pleocytosis, Gram stain, culture)
Polymerase chain reaction (PCR) for Neisseria meningitidis in blood or CSF
ITP: CBC with differential (platelet count < 100,000/µL, often < 20,000/µL
WBC and Hgb typically normal)
Peripheral blood smear (confirm thrombocytopenia, exclude pseudo-thrombocytopenia or other red cell abnormalities)
Coagulation profile (PT, aPTT, INR, fibrinogen, D-dimer) to rule out DIC
Liver function tests (LFTs) and renal function tests (RFTs)
Serology for viral causes of thrombocytopenia (e.g., EBV, CMV, parvovirus B19) if indicated
Bone marrow aspirate and biopsy typically not needed in acute childhood ITP but may be considered in chronic or atypical cases.
Differential Diagnosis:
Other causes of fever and petechiae/purpura: Viral exanthems with thrombocytopenia (e.g., dengue fever, enteroviruses)
Rocky Mountain spotted fever (RMSF) and other tick-borne illnesses
Hemolytic Uremic Syndrome (HUS)
Henoch-Schönlein Purpura (HSP) – typically rash is palpable and urticarial/purpuric, often associated with abdominal pain and arthritis
Leukemia or other hematological malignancies
Drug-induced thrombocytopenia
Disseminated intravascular coagulation (DIC) from any cause
Other bacterial sepsis with rash (e.g., Staphylococcus aureus, Streptococcus pyogenes).
Management
Initial Management:
Meningococcemia: IMMEDIATE broad-spectrum IV antibiotics (e.g., Ceftriaxone 100 mg/kg/dose IV q12h, or Cefotaxime) should be started empirically if meningococcemia is suspected, even before culture results or definitive diagnosis, after blood cultures are obtained
Fluid resuscitation for shock
Close monitoring in an intensive care setting
ITP: If platelet count > 30,000/µL and no significant bleeding, observe
If platelet count < 30,000/µL or bleeding, consider treatment
Steroids (e.g., Prednisolone 1-2 mg/kg/day orally or IV) or IV Immunoglobulin (IVIG) (1 g/kg/day IV for 1-2 days).
Medical Management:
Meningococcemia: Continue IV antibiotics for at least 7-10 days, tailored to culture and sensitivity results
Prophylaxis for close contacts with Rifampin, Ciprofloxacin, or Ceftriaxone
ITP: Steroids (prednisolone, dexamethasone) or IVIG are first-line
Platelet transfusions are reserved for active, severe bleeding or very low platelet counts (< 10,000/µL) in conjunction with other therapies.
Surgical Management:
Not typically indicated for primary meningococcemia or ITP
Surgery may be required for complications like limb ischemia or tissue necrosis due to purpura fulminans in meningococcemia, or in rare cases of splenectomy for refractory ITP.
Supportive Care:
Meningococcemia: Aggressive supportive care including fluid management, vasopressors if needed, mechanical ventilation, management of DIC and organ failure
Close monitoring of vital signs, urine output, and neurological status
ITP: Strict avoidance of aspirin and NSAIDs
Education on signs of bleeding
Monitoring for recurrence.
Complications
Early Complications:
Meningococcemia: Purpura fulminans, DIC, shock, adrenal hemorrhage (Waterhouse-Friderichsen syndrome), myocarditis, arthritis, meningitis, encephalitis, limb loss, death
ITP: Significant bleeding (intracranial hemorrhage is rare but serious), prolonged disease course, anemia due to chronic blood loss.
Late Complications:
Meningococcemia: Neurological deficits, hearing loss, limb deformities, scarring
ITP: Chronic ITP (persists > 12 months), increased risk of bleeding with minor trauma or surgery.
Prevention Strategies:
Meningococcemia: Vaccination against Neisseria meningitidis (especially serogroups A, C, Y, W-135, and B)
Prompt diagnosis and treatment of cases
Chemoprophylaxis for close contacts
ITP: No specific prevention
Focus on early recognition and management to prevent severe bleeding.
Prognosis
Factors Affecting Prognosis:
Meningococcemia: Rapid diagnosis and initiation of antibiotics are the most critical factors
Severity of illness at presentation (shock, DIC), age, and virulence of the infecting strain
ITP: Most children (80-85%) achieve spontaneous remission within 6-12 months
Factors associated with chronic ITP include older age at diagnosis and certain viral infections
Severe bleeding events significantly impact short-term outcome.
Outcomes:
Meningococcemia: Mortality rates have decreased with prompt treatment but remain significant (5-15% in developed countries)
Survivors may have long-term sequelae
ITP: Excellent prognosis for spontaneous recovery in most children
Chronic ITP requires long-term management.
Follow Up:
Meningococcemia survivors: Regular follow-up for neurological, audiological, and dermatological assessment
ITP: Regular monitoring of platelet counts, especially during the first year
Education for parents regarding signs of bleeding and when to seek medical attention
Annual review for chronic ITP.
Key Points
Exam Focus:
Distinguishing fever/petechiae in pediatric emergencies
Meningococcemia = SEPSIS + RASH
ITP = THROMBOCYTOPENIA + BLEEDING (usually NO fever/sepsis)
Always consider meningococcemia in febrile child with petechiae
start antibiotics empirically AFTER blood culture
ITP management is based on platelet count and bleeding severity, not solely platelet count.
Clinical Pearls:
A non-blanching petechial or purpuric rash in a febrile child is a medical emergency until proven otherwise
NEVER delay antibiotics for suspected meningococcemia while waiting for investigations
In ITP, look for bleeding, not infection
Lumbar puncture is contraindicated if signs of increased intracranial pressure or signs of DIC with purpura fulminans.
Common Mistakes:
Delaying empirical antibiotics in suspected meningococcemia due to fear of masking fever
Misdiagnosing meningococcemia as viral exanthem or ITP
Aggressively treating ITP with steroids/IVIG when observation is sufficient (platelets > 30k and no bleeding)
Failing to perform blood cultures before starting antibiotics for suspected meningococcemia.