Steroids, IVIG key in successful management of severe immune TTP
Case report IDs what clinicians say is best approach for emergency patients
Adults with severe immune thrombotic thrombocytopenic purpura (TTP) who are at a high risk of bleeding may be managed in a hospital’s emergency department (ED) with corticosteroids and intravenous immunoglobulin (IVIG), but should be admitted after stabilization until their platelet levels increase.
That’s according to a report detailing the case of a woman in her 30s, which a team of researchers used to address the question: “What are the emergency management considerations in immune thrombocytopenic purpura?”
Their study, “Just the facts: approach to adult patients with immune thrombocytopenic purpura in the emergency department,” characterizes what the team believes to be the best approach for the diagnosis and management of the rare blood disease in an ED. It was published in the Canadian Journal of Emergency Medicine.
“There is no diagnostic laboratory test for ITP,” the researchers wrote, noting that a diagnosis is based on platelet counts — small cell fragments that help blood to clot — in the body “while excluding other conditions.” In the study, set up in a Q&A format, the team detailed “essential laboratory testing in the ED setting,” and discussed “key disposition and follow-up considerations.”
TTP is characterized by the formation of blood clots inside small blood vessels, leading to organ damage. It is caused by a deficiency of the ADAMTS13 enzyme, which is normally responsible for preventing platelets from forming clots when they aren’t needed.
In immune (acquired) TTP, low ADAMTS13 activity is due to self-reactive antibodies that target the enzyme. The disease may also be identified as primary TTP if it occurs in isolation, or as secondary TTP if it is associated with an obvious underlying cause or disease. In most adults, the condition becomes chronic.
Woman, 32, diagnosed with immune TTP after seeking emergency treatment
Here, researchers discussed the case of a 32-year-old woman who sought emergency department care following two days of small, flat spots of bleeding under the skin, known as petechiae, on her legs, and easy bruising. She denied any recent illnesses or medication use. Upon examination, the woman was also found to have blood-filled vesicles in her right cheek, without active bleeding.
These and other symptoms, such as nose or gum bleeding, are common in people with immune TTP. Female patients often experience heavy menstrual bleeding or hemorrhage after delivery if they’ve had a baby.
Diagnosis commonly involves assessing the levels of platelets and blood cells, while excluding other conditions, such as infections, cancer, and liver disease.
According to the report, at the emergency department, clinicians should also assess for bleeding, particularly in the mucous membranes, such as in the mouth, and at other critical sites. Such sites include the brain, spinal cord, eyes, and around the heart; the team noted this recommendation is specifically intended for patients with severe disease, defined as platelet levels lower than 20-30 x109(billion)/L.
In this case, the woman’s platelet levels were low, at 8×109/L, and her platelets were large, but she had no signs of red blood cell destruction, the team noted. The woman received treatment in the emergency room with dexamethasone, a corticosteroid, and IVIG, which is an antibody-based therapy intended to help prevent platelet destruction and reduce the risk of bleeding. She was then admitted into the hospital.
Woman given corticosteroids, IVIG for TTP management
In their report, the investigators recommended treatment with corticosteroids plus IVIG in patients with severe immune TTP and no active bleeding. The team also urged admission to the hospital for observation, and to ensure that a patient’s platelet counts get higher than 30 × 10⁹/L.
“Up to 80% of patients respond to corticosteroids, typically within 2-3 weeks, and [30% to] 50% of adults will demonstrate a sustained response,” the researchers wrote. Typically used corticosteroids are dexamethasone or prednisone.
The team recommended IVIG for patients who have active bleeding or are at high risk of bleeding, and for those who do not respond to or cannot receive corticosteroids. About 80% of patients respond to one dose of IVIG, the researchers noted.
“The primary goals of [TTP] management include stopping active bleeding and reducing the risk of future bleeding,” the researchers wrote.
If corticosteroids and IVIG are ineffective, clinicians may turn to other treatments, such as rituximab to reduce self-reactive antibody production, or Tavalisse (fostamatinib disodium hexahydrate) to increase platelet levels, according to the team.
The primary goals of [TTP] management include stopping active bleeding and reducing the risk of future bleeding.
Discharge should only occur when patients are in stable condition, with no bleeding, with close outpatient follow-up, the team noted.
Further, patients should avoid taking medications that prevent platelets from clumping, as well as nonsteroid anti-inflammatory therapies. The team also cautioned against injections into the muscle and high-risk physical activities.
To end their report, the clinicians noted in a “case resolution” section that the woman in this case saw her platelet counts return to acceptable levels, and that she remains stable. She was admitted to the hospital, the team reported.
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