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TTP is a life-threatening thrombotic microangiopathy causing low platelets, haemolytic anaemia and organ ischaemia.
Quick Answer
TTP is a life-threatening thrombotic microangiopathy causing low platelets, haemolytic anaemia and organ ischaemia. Dr. Sachita Baniya (NMC 20158, 8+ years of clinical experience in haematology and haemato-oncology) evaluates thrombotic thrombocytopenic purpura (ttp) at Kathmandu Neurology Clinic & Cognitive Center, 117 Lal Durbar Marg, Kathmandu, Bagmati Province 44600, Nepal — evidence-based, medically reviewed.
TTP is caused by severe ADAMTS13 deficiency, leading to widespread microthrombi. It can affect the brain, kidneys, heart and gut. Urgent plasma exchange is the cornerstone of treatment and should not wait for confirmatory testing.
TTP is suspected with thrombocytopenia and microangiopathic haemolysis. Schistocytes, raised LDH, low haptoglobin and elevated bilirubin support the diagnosis. ADAMTS13 testing confirms but treatment must start urgently.
Urgent plasma exchange, corticosteroids and supportive care are standard. Caplacizumab may be used in selected settings. Platelet transfusion is avoided unless there is life-threatening bleeding.
Management is individualized and discussed with benefits, limitations, and follow-up.
There is no proven prevention. Patients with prior TTP need monitoring for relapse, especially during pregnancy or illness.
Red flags — seek urgent care
Yes. Without urgent treatment, TTP can be fatal. Plasma exchange should begin as soon as the diagnosis is suspected.
Platelet transfusion can worsen microthrombi in TTP. It is reserved for life-threatening bleeding or before urgent surgery.
It is an enzyme that cleaves von Willebrand factor. Severe deficiency leads to abnormal platelet clumping and microthrombi.
Yes, relapse can occur. Long-term ADAMTS13 monitoring helps detect recurrence, especially after immune-mediated TTP.
Pregnancy carries a risk of relapse and requires specialist planning with haematology and obstetrics.
It blocks von Willebrand factor-mediated platelet adhesion and may be used with plasma exchange in immune-mediated TTP.
TTP is usually ADAMTS13-mediated, while HUS is often complement-mediated or associated with Shiga toxin. They can look similar and need specialist evaluation.
ADAMTS13 monitoring, blood counts, renal and neurological review, and relapse surveillance.
Specialist assessment for abnormal blood counts, anaemia, low platelets, bleeding, clotting and suspected blood disorders.
Assessment of low platelets including ITP, TTP, drug causes and marrow disorders.
Red cell and platelet transfusion support for haematological disease and treatment.
Immune thrombocytopenia is an acquired immune-mediated fall in platelets causing bruising and bleeding.
Pancytopenia is a reduction in all three blood cell lines, caused by marrow failure, destruction or sequestration.
Cancer-associated thrombosis is venous thromboembolism occurring in people with cancer, driven by the cancer and its treatment.
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Reviewed by Dr. Jitendra Prasad Yadav • Last reviewed: 2026-11-10
Content is educational and aligns with standard medical references; individual evaluation may vary. External links provide context and do not imply endorsement.
Doctor — medically reviewed
Dr. Sachita Baniya
Consultant Haemato-Oncologist & Clinical Hematologist • MBBS, FCPS (Clinical Haematology) • NMC 20158
This condition guide was medically reviewed by Dr. Sachita Baniya.
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