Practice Essentials
Immune thrombocytopenic purpura (ITP) is a clinical syndrome in which a decreased number
of circulating platelets (thrombocytopenia) (see the image below) manifests as a bleeding
tendency, easy bruising (purpura), or extravasation of blood from capillaries into skin and
mucous membranes (petechiae). Although most cases of acute ITP, particularly in children,
are mild and self-limited, intracranial hemorrhage may occur when the platelet count drops
below 10 109/L (<10 103/L); [1] this occurs in 0.5-1% of children, and half of these cases
are fatal. [2]
Peripheral blood smear from a
patient with immune thrombocytopenic purpura (ITP) shows a decreased number of platelets,
a normal-appearing neutrophil, and normal-appearing erythrocytes. ITP is diagnosed by
excluding other diseases; therefore, the absence of other findings from the peripheral smear is
at least as important as the observed findings. This smear demonstrates the absence of
immature leukocytes (as in leukemia) and fragmented erythrocytes (as in thrombotic
thrombocytopenic purpura) and no clumps of platelets (as in pseudothrombocytopenia).
Signs and Symptoms
ITP is a primary illness occurring in an otherwise healthy person. Signs of chronic disease,
infection, wasting, or poor nutrition indicate that the patient has another illness.
Splenomegaly excludes the diagnosis of ITP.
An initial impression of the severity of ITP is formed by examining the skin and mucous
membranes, as follows:
Widespread petechiae and ecchymoses, oozing from a venipuncture site, gingival
bleeding, and hemorrhagic bullae indicate that the patient is at risk for a serious
bleeding complication
If the patient's blood pressure was taken recently, petechiae may be observed under
and distal to the area where the cuff was placed and inflated
Suction-type electrocardiograph (ECG) leads may induce petechiae
Petechiae over the ankles in ambulatory patients or on the back in bedridden ones
suggest mild thrombocytopenia and a relatively low risk for a serious bleeding
complication
Findings suggestive of intracranial hemorrhage include the following:
Headache, blurred vision, somnolence, or loss of consciousness
Hypertension and bradycardia, which may be signs of increased intracranial pressure
On neurologic examination, any asymmetrical finding of recent onset
On fundoscopic examination, blurring of the optic disc margins or retinal hemorrhage
See Clinical Presentation for more detail.
Diagnosis
On complete blood cell count, isolated thrombocytopenia is the hallmark of ITP. Anemia
and/or neutropenia may indicate other diseases. Findings on peripheral blood smear are as
follows:
The morphology of red blood cells (RBCs) and leukocytes is normal
The morphology of platelets is typically normal, with varying numbers of large
platelets
If most of the platelets are large, approximating the diameter of red blood cells, or if
they lack granules or have an abnormal color, consider an inherited platelet disorder
Many children with acute ITP have an increased number of normal or atypical lymphocytes
on the peripheral smear, reflecting a recent viral illness. Clumps of platelets on a peripheral
smear prepared from ethylenediaminetetraacetic acid (EDTA)anticoagulated blood are
evidence of pseudothrombocytopenia. [3] This diagnosis is established if the platelet count is
normal when repeated on a sample from heparin-anticoagulated or citrate-anticoagulated
blood.
Aspects of bone marrow aspiration and biopsy are as follows:
The value of bone marrow evaluation for a diagnosis of ITP is unresolved [4]
Biopsy in patients with ITP shows a normal-to-increased number of megakaryocytes
in the absence of other significant abnormalities
In children, bone marrow examination is not required except in patients with atypical
hematologic findings, such as immature cells on the peripheral smear or persistent
neutropenia. [5]
In adults older than 60 years, biopsy is used to exclude myelodysplastic syndrome or
leukemia
In adults whose treatment includes corticosteroids, a baseline pretreatment biopsy
may prove useful for future reference, as corticosteroids can change marrow
morphology
Biopsy is performed before splenectomy to evaluate for possible hypoplasia or
fibrosis
Unresponsiveness to standard treatment after 6 months is an indication for bone
marrow aspiration
See Workup for more detail.
Management
ITP has no cure, and relapses may occur years after seemingly successful medical or surgical
management. [6] Most children with acute ITP do not require treatment, and the condition
resolves spontaneously. [7, 8]
Treatment is as follows:
Corticosteroids remain the drugs of choice for the initial management of acute ITP
Oral prednisone, IV methylprednisolone, or high-dose dexamethasone may be used [9,
10, 11]
IV immunoglobulin (IVIG) has been the drug of second choice for many years [12, 13]
For Rh(D)-positive patients with intact spleens, IV Rho immunoglobulin (RhIG)
offers comparable efficacy, less toxicity, greater ease of administration, and a lower
cost than IVIG [14, 15]
RhIG can induce immune hemolysis (immune hemolytic anemia) in Rh(D)-positive
persons and should not be used when the hemoglobin concentration is less than 8 g/dL
Sporadic cases of massive intravascular hemolysis, [16] disseminated intravascular
coagulation (particularly in elderly individuals), and renal failure [17] have been
reported with RhIG
Rituximab is third-line therapy
Platelet transfusions may be required to control clinically significant bleeding but are
not recommended for prophylaxis
If 6 months of medical management fails to increase the platelet count to a safe range
(about 30,000/L), splenectomy becomes an option
Thrombopoietin receptor agonists (ie, eltrombopag, romiplostim) may maintain
platelet counts at safe levels in adults with chronic ITP refractory to conventional
medical management or splenectomy
Pregnant women require special consideration for delivery, as follows [18] :
If the platelet count is greater than 50 10 9/L (>50 10 3/L), the risk of serious
hemorrhage is low, but beginning oral prednisone a week before delivery is a
reasonable precaution
If the platelet count is less than 50 10 9/L (50 10 3/L) before delivery, treatment
with oral prednisone and IVIG is recommended
Avoiding the use of IV RhIG in this situation until safety data are available is
advisable
Rarely, splenectomy may be required to manage acute hemorrhage [19]
See Treatment and Medication for more detail.
