thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare and serious blood disorder that affects the small blood vessels throughout the body. It is characterized by the formation of blood clots (thrombi) in these small vessels, which can lead to a low platelet count (thrombocytopenia) and the appearance of purple bruises or spots on the skin (purpura).

Key Features:

• Blood clots form in small blood vessels throughout the body [4]
• Low platelet count (thrombocytopenia) [3, 5, 7, 8]
• Purple bruises or spots on the skin (purpura) [1, 2, 6]
• Can cause serious medical problems if blood clots block vessels and restrict blood flow to organs such as the brain, kidneys, and heart [4]

Description:

TTP is a rare disorder that causes blood clots to form in small blood vessels throughout the body. These clots can cause serious medical problems if they block vessels and restrict blood flow to organs such as the brain, kidneys, and heart. The symptoms of TTP are related to where in the body blood clots form [3]. Diagnosis is based on the person's symptoms and blood test results.

Symptoms:

• Severe decrease in the number of blood platelets [5]
• Clotting in small blood vessels throughout the body [4]
• Low platelet count (thrombocytopenia) [3, 5, 7, 8]
• Purple bruises or spots on the skin (purpura) [1, 2, 6]

References:

[1] Context result 1 [2] Context result 2 [3] Context result 3 [4] Context result 4 [5] Context result 5 [6] Context result 6 [7] Context result 7 [8] Context result 8

Thrombotic thrombocytopenic purpura (TTP) is a rare condition characterized by the formation of blood clots in small blood vessels throughout the body. The signs and symptoms of TTP can vary in severity and may include:

• Purplish bruises on the skin or mucous membranes: These bruises, called purpura, are caused by bleeding under the skin.
• Pinpoint-sized red or purple dots on the skin: These dots, called petechiae, often appear on the skin as a result of blood leaking from small blood vessels.
• Abdominal pain: This is one of the most common symptoms, with or without nausea and vomiting.
• Bleeding into the skin or mucus membranes: This can cause red, purple, or black spots to appear on the skin.
• Confusion
• Fatigue, weakness
• Fever
• Headache
• Pale skin color or yellowish skin color

It's worth noting that TTP can also cause more severe symptoms, such as:

• Renal dysfunction: This can lead to kidney failure if left untreated.
• Neurologic dysfunction: This can cause a range of symptoms, including confusion, seizures, and coma.

If you or someone you know is experiencing any of these symptoms, it's essential to seek medical attention immediately. Without treatment, TTP has a high mortality rate, but with proper care, the condition can be managed and treated effectively.

References:

• [1] Thrombotic thrombocytopenic purpura is a rare condition that causes blood clots to develop in small blood vessels throughout your body.
• [2] Blood clots, a low platelet count, and damaged red blood cells cause the signs and symptoms of thrombotic thrombocytopenic purpura (TTP).
• [3] The single most common symptom was abdominal pain (23.5%), with or without nausea/vomiting.
• [4] Purpura is the term for the bruises that appear on the skin as a result of bleeding under the skin.
• [10] Thrombotic means that blood clots form, thrombocytopenic means the platelet count is low, and purpura means that spots or bruises (usually red, purple, or black) appear on the skin.

Thrombotic thrombocytopenic purpura (TTP) can be confirmed through various diagnostic tests, which are crucial for accurate diagnosis and treatment.

Common Diagnostic Tests:

• Complete Blood Count (CBC): A CBC measures the number of platelets, red blood cells, and white blood cells. People with TTP typically have a lower number of platelets [1].
• Kidney Function Tests and Urine Tests: These tests assess kidney function and may reveal protein or blood cells in the urine, indicating potential kidney damage [3].
• Blood Smear: A blood smear examines the shape and size of red blood cells, which can be affected in TTP [6].
• ADAMTS 13 Activity Level: This test measures the level of ADAMTS 13, a protein that helps regulate platelet formation. Low levels are associated with TTP [8].

Additional Tests:

• Bilirubin and Lactate Dehydrogenase (LDH) tests can indicate red blood cell destruction [8].
• Creatinine Level and Reticulocyte Count may also be performed to assess kidney function and red blood cell production, respectively [9].
• Serum Haptoglobin and Peripheral Blood Smear can provide further evidence of hemolytic anemia [9].

Guidelines and Recommendations:

The International Society on Thrombosis and Haemostasis (ISTH) has published guidelines for the diagnosis of TTP, which include measuring plasma ADAMTS13 activity and identifying or quantifying other relevant biomarkers [11]. A study by Grall et al. highlights the importance of accurate diagnosis and management of TTP, emphasizing the need for standardized approaches [12].

Key Points:

• A combination of diagnostic tests is often necessary to confirm TTP.
• Accurate diagnosis is critical for effective treatment and improved outcomes.

References:

[1] Context reference 1 [3] Context reference 3 [6] Context reference 6 [8] Context reference 8 [9] Context reference 9 [11] Zheng et al. (2020) [12] Grall et al.

Thrombotic Thrombocytopenic Purpura (TTP) is a rare blood disorder characterized by the formation of blood clots in small blood vessels throughout the body. The treatment for TTP typically involves a combination of medications and therapies to manage symptoms, prevent further clotting, and improve platelet count.

Medications Used to Treat TTP:

• Corticosteroids: Corticosteroids are commonly given to patients with TTP to reduce inflammation and suppress the immune system. [5]
• Glucocorticoids: Glucocorticoids, such as prednisone, are also used to treat TTP by reducing inflammation and improving platelet count. [2], [8]
• Rituximab: Rituximab is a monoclonal antibody that targets the CD20 protein on B cells, which can contribute to the development of TTP. It has been shown to be effective in treating refractory TTP cases. [2], [7]
• Vincristine: Vincristine is a chemotherapy medication that can help reduce platelet destruction and improve platelet count in patients with TTP. [8]
• Cyclosporine A: Cyclosporine A is an immunosuppressive medication that can be used to treat refractory TTP cases by reducing the immune system's attack on platelets. [8]

Other Therapies:

• Plasma Exchange: Plasma exchange involves removing plasma from the blood and replacing it with fresh frozen plasma (FFP) or albumin to help remove antibodies and other substances that contribute to clotting. This therapy is often used in conjunction with corticosteroids and immunosuppressive medications. [1], [11]
• Splenectomy: In severe cases of TTP, a splenectomy may be necessary to remove the spleen, which can help reduce platelet destruction and improve platelet count. [2]

Emerging Treatments:

• Caplacizumab: Caplacizumab is a monoclonal antibody that targets von Willebrand factor (VWF) and has been shown to be effective in reducing the frequency of major thromboembolic events, exacerbations, and death in patients with acquired TTP. [6], [9]

It's essential to note that treatment for TTP should only be administered under the guidance of a qualified healthcare professional, as each patient's condition is unique and requires individualized care.

References:

[1] Nov 15, 2023 — The therapy of choice is plasma exchange with fresh frozen plasma and immunosuppression. [2] Feb 9, 2022 — Common medications used to treat TTP include rituximab and glucocorticoids. [3] by C Masias · 2018 · Cited by 30 — Caplacizumab reduces the time to platelet recovery and the exacerbation rate in acute TTP. [4] by A Gómez-De León · 2020 · Cited by 16 — Plasma exchange and corticosteroids continue to be the mainstay of treatment; however, repurposed drugs and novel agents are emerging as efficient treatment options for TTP. [5] Nov 15, 2023 — Corticosteroids are commonly given to patients with TTP. [6] by M Scully · 2019 · Cited by 883 — Caplacizumab reduces the frequency of major thromboembolic events, exacerbations and death in patients with acquired thrombotic thrombocytopenic purpura (TTP). [7] by FA Sayani · 2015 · Cited by 193 — Upfront treatment of acute TTP includes plasma exchange and corticosteroids. [8] Medicines used to treat TTP include glucocorticoids, vincristine, rituximab, and cyclosporine A. [9] by XL Zheng · 2020 · Cited by 324 — Drug acceptability would likely be reasonable because caplacizumab can be administered subcutaneously in an outpatient or home setting via self-administration. [10] Result: (no data available) [11] by ML Sennett · 1986 · Cited by 35 — Plasmapheresis with fresh frozen plasma is often used to treat TTP.

Differential Diagnosis of Thrombotic Thrombocytopenic Purpura (TTP)

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening disorder characterized by the formation of blood clots in small blood vessels throughout the body. The differential diagnosis for TTP includes several conditions that can present with similar symptoms, making accurate diagnosis crucial.

Conditions to Consider:

• Hemolytic-Uremic Syndrome (HUS): A condition that affects the kidneys and is often associated with thrombocytopenia and microangiopathic hemolytic anemia.
• Immune Thrombocytopenic Purpura (ITP): An autoimmune disorder characterized by low platelet count, but without the presence of blood clots in small vessels.
• Disseminated Intravascular Coagulation (DIC): A condition that involves widespread clotting and bleeding throughout the body.

Key Diagnostic Features:

• ADAMTS13 Activity: Levels of ADAMTS13 activity can help differentiate TTP from other conditions. A deficiency of <10% defines TTP.
• Microangiopathic Hemolytic Anemia (MAHA): The presence of schistocytes and fragmented red blood cells is a hallmark of MAHA, which is characteristic of TTP.

Other Considerations:

• TMA Syndromes: Other thrombotic microangiopathies (TMAs) can present with similar symptoms to TTP.
• Pregnancy/Postpartum: TTP can occur in pregnant or postpartum women, and the differential diagnosis should include conditions such as eclampsia and HELLP syndrome.

References:

• [1] Thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia (MAHA), severe thrombocytopenia, and ischemic end-organ damage resulting from formation of platelet-rich thrombi in the microvasculature. [Source: 14]
• [2] The differential diagnosis for TTP includes HUS, ITP, DIC, and other TMAs. [Source: 9]
• [3] ADAMTS13 activity is a key diagnostic feature that can help differentiate TTP from other conditions. [Source: 1]

Note: The references provided are based on the search results within the context.