SIGNS AND SYMPTOMS 
History
- Bleeding is the most common complaint:
- Common:
- Mucous membrane: Gingiva, epistaxis, conjunctival, menorrhagia
- Rare (more common in coagulopathies):
- GI bleeding, hemarthrosis, hematuria, and hematomas
- 84% of pediatric cases are 2–3 wk following a viral illness:
- Small percentage found following vaccinations
- Most adult cases have an insidious onset:
- Up to 28% of patients are asymptomatic and diagnosed on routine CBC
- < 5% present with life-threatening bleeding
Physical Exam
- Mucosal bleeding often apparent in symptomatic cases
- Commonly may follow dental procedures, extraction or trauma
- Petechiae (nonblanching)
- Nonpalpable purpura
- Distinguishes ITP from Henoch–Schönlein purpura (HSP)
- Melena, bright red blood per rectum, or hemoccult positive stools
- Spleen normal size in ITP:
- Enlarged spleen may be found in leukemia or other platelet sequestration syndromes
- Neurologic deficits from intracranial hemorrhage (ICH):
- ICH is the most common cause of death in ITP.
- Risk of ICH in ITP increases with age:
- Age < 40 yr: 2%
- Age > 60 yr: 48%
ESSENTIAL WORKUP
- Diagnosis of exclusion—other causes of thrombocytopenia must be ruled out.
- CBC with differential
- PT and PTT if actively bleeding to exclude other forms of coagulopathy
- BUN and creatinine to evaluate renal function
- Liver function to exclude liver disease
- Type and screen if actively bleeding
- Pregnancy test if of childbearing age
- HIV testing should be considered
DIAGNOSIS TESTS & INTERPRETATION
Lab
CBC with differential and peripheral smear:
- Thrombocytopenia
- Increased mean platelet volume
- Normal WBC and RBC morphology and size
- Liver function, coagulation studies, and kidney function should all be within normal limits
Imaging
CT head without contrast to evaluate for ICH if clinically indicated by focal findings, headache, or head trauma.
Diagnostic Procedures/Surgery
Bone marrow biopsy:
- All adults > 60 yr to evaluate for malignancy
- Atypical symptoms and cases refractory to treatment
- Patients considering splenectomy
- Children with persistent thrombocytopenia > 6 mo
- Children unresponsive to intravenous immunoglobulin (IVIG)
- Antibody testing is of no clinical value
DIFFERENTIAL DIAGNOSIS
- Impaired bone marrow production:
- Bone marrow fibrosis
- Bone marrow infiltration owing to malignancy
- Cytotoxic drugs used in chemotherapy
- Congenital/acquired bone marrow abnormalities
- Splenic sequestration:
- Accelerated destruction of platelets:
- Drug induced:
- Decreased platelet production:
- Chemotherapy
- Thiazide diuretics
- Ethanol
- Estrogen
- Increased destruction of platelets:
[Outline]
PRE-HOSPITAL
Stabilize ABCs
- Significant oral or laryngeal bleeding may affect airway.
- CNS event may affect ability to control airway
- Establish IV access for cases of significant bleeding
- Control any bleeding with direct pressure
INITIAL STABILIZATION/THERAPY
- ABCs
- Stabilize life-threatening bleeding:
- ICH:
- Airway control
- Neurosurgery consultation: Craniotomy not typically possible until platelet counts > 75 K
- Hemorrhagic shock:
- 2 large-bore IVs
- Direct pressure for hemostasis
- Resuscitation with blood transfusions and isotonic crystalloid
- Medications and platelets for acute life-threatening episodes of bleeding:
- IV high-dose dexamethasone or methylprednisolone
- IVIG infusion
- Platelet transfusions: 2–3 times the normal amount
- Platelets typically infused after steroids and/or IVIG
- Mucous membrane bleeding:
- Topical agents
- Other:
- IV Aminocaproic acid may be considered
- In rare cases, plasmapheresis has been proven helpful
ED TREATMENT/PROCEDURES
- Initial treatment options for ITP are based on:
- Degree of thrombocytopenia
- Severity of illness
- Duration of symptoms
- Age
- Risk factors for bleeding (hypertension, peptic ulcer disease, vigorous lifestyle)
- Hematologist preference
- Efficacy of treatment is demonstrated in terms of platelet recovery time and not morbidity or mortality.
- Specific treatment options:
- Observation is recommended for children without bleeding complications or profound thrombocytopenia (< 20–30 K)
- Profound thrombocytopenia (< 20 K):
- High-dose corticosteroids: 75% response
- IVIG: 80% response, but costly, so reserved in time critical emergencies
- Anti-D IG: 70% response (used only in Rh+ patients)
- Splenectomy:
- 2nd-line therapy if inadequate response to a course of glucocorticoid therapy
- Two-thirds of adult patients respond, three-fourths of pediatric patients respond.
- No specific indications for emergent splenectomy
Pregnancy Considerations
- Differential diagnosis of thrombocytopenia during pregnancy:
- Gestational thrombocytopenia (75%):
- Usually of no clinical significance
- Does not cause neonatal thrombocytopenia
- Remits 1–2 wk after delivery
- Platelets typically > 50 K
- HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets)
- ITP (15%):
- Platelets typically < 50 K
- Maternal platelet count does not correlate to neonatal thrombocytopenia.
- Treatment with steroids or IVIG does not alter incidence of neonatal thrombocytopenia.
- Degree of thrombocytopenia should not alter decision of vaginal birth vs. C-section.
- Measure neonatal CBC and check brain ultrasound for ICH.
- Treat neonatal thrombocytopenia with IVIG and steroids
MEDICATION
First Line
- Glucocorticoids (high dose over 2–3 wk):
- Dexamethasone: 40 mg PO daily
- Prednisone: 1–2 mg/kg/24 hr PO/day
- Methylprednisolone: 1 g IV q8h (30 mg/kg/24 hr pediatric dose)
- IVIG: 1–2 g/kg IV × 1 dose and possibly repeated in 24 hr:
- Used only for critical bleeding or when the need to acutely raise platelets is required such as emergency surgery
- Anti-D immunoglobulin: 50 µg/kg/24 hr IV:
- Used only presplenectomy and in Rh+ patients
Second Line
- Chronic long-term suppression and steroid bolus therapy
- Immunosuppressive agents:
- Other:
- Rituximab: Monoclonal antibody directed against B cell antigens
- Danazol: Antiandrogen
- Experimental:
- Other monoclonal antibodies directed at B cells
- Stem cell transplants
- Thrombopoietin and thrombopoietin-like agonists
[Outline]
DISPOSITION
Admission Criteria
- Life-threatening bleeding regardless of platelet count
- Any bleeding with platelet count < 20 K
- Asymptomatic patient with platelet count < 20 K with issues of noncompliance or poor follow-up
Discharge Criteria
- Asymptomatic patients
- Patients with minor bleeding and platelets > 30 K
FOLLOW-UP RECOMMENDATIONS
Hematology referral is indicated in all cases (either outpatient or inpatient consultation)
[Outline]
ICD9
287.31 Immune thrombocytopenic purpura
ICD10
D69.3 Immune thrombocytopenic purpura
[Outline]
- British Committee for Standards in Haematology General Haematology Task Force. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. Br J Haematol. 2003;120:574–596.
- Fogarty PF, Segal JB. The epidemiology of immune thrombocytopenic purpura. Curr Opin Hematol. 2007;14:515–519.
- Godeau B, Provan D, Bussel J. Immune thrombocytopenic pupura in adults. Curr Opin Hematol. 2007;14:535–556.
- Neunert C, Lim W, Crowther M, et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011;117(16):4190–4207.
- Schipperus M, Fijnheer R. New therapeutic options for immune thrombocytopenia. Neth J Med. 2011;69(11):480–485.
See Also (Topic, Algorithm, Electronic Media Element)
HELLP Syndrome; Thrombotic Thrombocytopenic Purpura