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Family Health Version

Immune thrombocytopenia (PTI)

(Idiopathic thrombocytopenic purpura; immune thrombocytopenic
purpura)

By David J. Kuter , MD, DPhil, Harvard Medical School

Revised/Correct: Jun 2022

Immune thrombocytopenia (immune thrombocytopenic purpura, PTI) is a hemorrhagic disorder caused

by the decrease in the number of platelets (thrombocytes) that occurs in a person who has no other
disorder that affects platelets. In INP, the immune system produces antibodies against the person's
own platelets and destroys them.

People may have tiny purple spots on the skin (petechiae) and bleed easily.

The diagnosis is made by blood tests to measure the number of platelets.

Corticosteroids or other drugs are administered to block the destruction of platelets.

Some people benefit from medicines that increase the production of platelets.

In adults, doctors sometimes remove the person's spleen.

(See also General considerations on platelet disorders and General considerations on

thrombocytopenia).

Platelets are cells that are manufactured in the bone marrow and circulate in the bloodstream and
help the blood to clot. Blood usually contains between 140,000 and 440,000 platelets per microliter
(140 to 440 × 109 per liter). When the platelet count drops to less than 50,000 platelets per microliter
of blood (less than 50 × 10 9 per liter), bleeding may occur even after relatively minor lesions. The
most serious risk of bleeding, however, usually does not occur until the platelet count drops to less
than 10,000 to 20,000 platelets per microliter of blood (10 to 20 × 109 per liter). At these low levels,
hemorrhages can occur without any recognized injury.

Immune thrombocytopenia is a disorder in which antibodies form and destroy the body's platelets. It
is not known why antibodies are formed; however, in children, itP often occurs after a viral infection.
Although bone marrow can increase the production of platelets to compensate for their destruction,
supply usually cannot compensate for demand. Sometimes, antibodies that are destroying platelets
also attack the bone marrow and reduce platelet production.

In adults, PTI is usually prolonged (chronic). In children, the PTI often solves itself.

Symptoms of PTI
Symptoms of PTI
It is possible that some do not have the symptoms of immune thrombocytopenia. In other people,
the symptoms of bleeding can appear suddenly or gradually. In chronic ICU, fatigue is common.

Skin hemorrhage may be the first sign of a low platelet count. Many tiny red dots (petechiae) often
appear on the skin of the lower legs and small lesions can cause black and bluish bruises (echymosis
or purple). The gums may bleed and blood may appear in the stool or urine. Menstrual periods or
nosebleeds can be unusually intense. It can be difficult to stop the bleeding.

Hemorrhage on the skin

Ecchymoses (bruises)

Ecchymoses are large purple spots seen on this leg.

DR P. MARAZZI/SCIENCE PHOTO LIBRARY

 Petechiae (skin)

Petechiae are small red dots as seen here on the skin.

With permission from the editor. From Deitcher S. in

Atlas of Clinical Hematology. Edited by JO Armitage.
Philadelphia, Current Medicine, 2004.

Petechiae (mouth)

Petechiae are small red dots as seen here in the

mouth.

DR P. MARAZZI/SCIENCE PHOTO LIBRARY

Bleeding worsens as the number of platelets decreases. People with a shortage of platelets may lose
large amounts of blood in the digestive tract or, in rare cases, suffer life-threatening brain
hemorrhages despite not having suffered any injury. Headache and other nervous system symptoms
can occur with bleeding in the brain.

ICU Diagnosis

Blood tests to measure platelet count and coagulation

 Blood tests to measure platelet count and coagulation

Tests to rule out other disorders that cause low platelet count and bleeding

Doctors make the diagnosis of immune thrombocytopenia (ITP) when the platelet count is less than
100,000 per microliter of blood (less than 100 × 109 per liter) without a similar decrease in the
number of red blood cells or white blood cells and when there is no other clear explanation for
thrombocytopenia, such as an infection or the use of certain medications (see table Causes of
thrombocytopenia ). There is no well-established test to confirm that a person has itP.

Platelet count can be measured with an automated counter to determine the severity of
thrombocytopenia and a blood sample should be examined under a microscope to provide clues to
its cause. Doctors need to examine the blood microscopic to distinguish ITP from thrombotic
thrombocytopenic purpura (PTT) and hemolytic-uremic syndrome (HUS). PTT and HUS are other
disorders that can cause thrombocytopenia by the destruction of platelets.

Rarely, a bone marrow sample is taken and examined under a microscope (bone marrow aspiration
and biopsy) to provide information about platelet production.

Treatment of PTI

Corticosteroids

Intravenous immunoglobulin, thrombopoietin receptor agonists, or immunosuppressants (e.g.,

rituximab, azathioprine or mycophenolate)

Sometimes, removal of the spleen

Rarely, platelet transfusions

In theITP, platelet-destroying antibodies can be temporarily blocked with a corticosteroid

(prednisone, for example) or intravenous immunoglobulin, which allows the number of platelets to
increase. Children usually recover within a few weeks or months after this treatment.

Approximately 30% of adults recover during the first year, but most do not. It is possible that adults
who do not respond adequately to corticosteroids or are dependent on corticosteroids will need
other drugs that increase the production of platelets (thrombopoietin receptor agonists) or that
suppress the immune system, including rituximab, azathioprine, Cytoxan, cyclosporine or
mycophenolate. Fostamatinib is another medicine that can be used if other medicines do not help.

Thrombopoietin receptor agonists (such as romiplostim, eltrombopague and avatrombopague)

increase the rate of platelet production and can be effective for years. These drugs are especially
useful for people who cannot (or do not want to) undergo splenectomy.

Some adults (but usually not children) with GTI benefit from surgical removal of the spleen
(splenectomy). Since the spleen removes anomalous platelets from the bloodstream, the removal of
the spleen sometimes causes an increase in the number of platelets. The drawbacks of splenectomy
include an increased risk of having blood clots, an increased risk of having cancer and an increased
risk of having certain life-threatening infections, such as pneumococcal infections. People undergoing
splenectomy can take certain antibiotics or vaccines that reduce (but do not completely eliminate)
the risk of infection. Pharmacological treatment is increasingly being used to replace splenectomy.

People with life-threatening bleeding may receive platelet transfusion (in addition to intravenous
People with life-threatening bleeding may receive platelet transfusion (in addition to intravenous
corticosteroids and/or immunoglobulin).

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