Thrombotic thrombocytopenic purpura
3,960views
00:00 / 00:00
Videos
Notes
Thrombotic thrombocytopenic purpura
Pathology
Anemias
Iron deficiency anemia
Beta-thalassemia
Alpha-thalassemia
Sideroblastic anemia
Anemia of chronic disease
Lead poisoning
Hemolytic disease of the newborn
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Autoimmune hemolytic anemia
Pyruvate kinase deficiency
Paroxysmal nocturnal hemoglobinuria
Sickle cell disease (NORD)
Hereditary spherocytosis
Anemia of chronic disease
Aplastic anemia
Fanconi anemia
Megaloblastic anemia
Folate (Vitamin B9) deficiency
Vitamin B12 deficiency
Fanconi anemia
Diamond-Blackfan anemia
Heme synthesis disorders
Acute intermittent porphyria
Porphyria cutanea tarda
Lead poisoning
Coagulation disorders
Hemophilia
Vitamin K deficiency
Platelet disorders
Bernard-Soulier syndrome
Glanzmann's thrombasthenia
Hemolytic-uremic syndrome
Immune thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Mixed platelet and coagulation disorders
Von Willebrand disease
Disseminated intravascular coagulation
Heparin-induced thrombocytopenia
Thrombosis syndromes (hypercoagulability)
Antithrombin III deficiency
Factor V Leiden
Protein C deficiency
Protein S deficiency
Antiphospholipid syndrome
Lymphomas
Hodgkin lymphoma
Non-Hodgkin lymphoma
Leukemias
Chronic leukemia
Acute leukemia
Leukemoid reaction
Leukemoid reaction
Dysplastic and proliferative disorders
Myelodysplastic syndromes
Polycythemia vera (NORD)
Myelofibrosis (NORD)
Essential thrombocythemia (NORD)
Langerhans cell histiocytosis
Mastocytosis (NORD)
Plasma cell dyscrasias
Multiple myeloma
Monoclonal gammopathy of undetermined significance
Waldenstrom macroglobulinemia
Hematological system pathology review
Microcytic anemia: Pathology review
Non-hemolytic normocytic anemia: Pathology review
Intrinsic hemolytic normocytic anemia: Pathology review
Extrinsic hemolytic normocytic anemia: Pathology review
Macrocytic anemia: Pathology review
Heme synthesis disorders: Pathology review
Coagulation disorders: Pathology review
Platelet disorders: Pathology review
Mixed platelet and coagulation disorders: Pathology review
Thrombosis syndromes (hypercoagulability): Pathology review
Lymphomas: Pathology review
Leukemias: Pathology review
Plasma cell disorders: Pathology review
Myeloproliferative disorders: Pathology review
Assessments
Thrombotic thrombocytopenic purpura
Flashcards
0 / 8 complete
USMLE® Step 1 questions
0 / 2 complete
High Yield Notes
6 pages
Flashcards
Thrombotic thrombocytopenic purpura
of complete
Questions
USMLE® Step 1 style questions USMLE
of complete
| Laboratory value | Result |
| Hematologic | |
| Hemoglobin | 12 g/dL |
| Hematocrit | 40% |
| Platelet count | 95,000/mm3 |
| Leukocyte count | 9,000/mm3 |
| Coagulation studies | |
| Prothrombin time (PT) | 12 seconds |
| Activated partial thromboplastin time (aPTT) | 29 seconds |
| Bleeding time* | 15 minutes |
Which of the following conditions is the patient at greatest risk of developing?
Memory Anchors and Partner Content
External References
First Aid
2022
2021
2020
2019
2018
2017
2016
Thrombotic thrombocytopenic purpura (TTP) p. 424, 434
External Links
Transcript
Content Reviewers
Kelly Johnson, MS
Contributors
Jerry Ferro
Alaina Mueller
Megan Gullotto, MSMI
Thrombotic Thrombocytopenic Purpura, often referred to as TTP, is a rare, serious blood disease that results in many small blood clots forming throughout the body.
Typically, blood clot formation starts the tissue injury. Right away, some cells begin to release a protein called von Willebrand factor. This von Willebrand factor sticks to exposed fibers in the torn tissue and becomes like glue, allowing platelets to stick to the site.
As the platelets pile on top of one another, they link together into a mesh, which we call a clot. In TTP, there is often a severe deficiency of the enzyme ADAMTS13 which breaks down von Willebrand factor when it’s no longer needed.
Without this von Willebrand factor regulation by ADAMTS13, small blood clots can form more frequently throughout the body.
The small blood clots formed in a person with TTP can block normal blood flow through arteries and veins, leading to clinical findings that include decreased platelets, increased red blood cell destruction, and neurological issues such as headaches, mental changes, confusion, speech abnormalities, partial paralysis, seizures, or coma.
TTP can cause a wide range of other symptoms. Due to the low number of platelets, small areas of abnormal bleeding in the skin may occur which can be seen as a rash-like appearance or a purplish discoloration.
Other symptoms may affect the entire body including fever, weakness, fatigue, and extreme paleness. There can also be episodes of unusually heavy bleeding or abdominal pain accompanied by nausea and vomiting.
Acute kidney failure occurs in less than 10% of people with TTP and requires dialysis. Due to the lack of blood and urine filtration when the kidneys stop working properly, increased water, salt, and protein retention can occur causing an onset of symptoms within days including foot swelling, shortness of breath, headaches, fever, and an irregular heartbeat.
Summary
Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder that is characterized by the formation of small blood clots in the small blood vessels throughout the body. These clots can limit or block blood flow to vital organs, leading to tissue damage and organ dysfunction. TTP can also cause low levels of platelets (thrombocytopenia), which are important for blood clotting.
Although the exact cause of TTP is unknown, it's associated with a deficiency of ADAMTS13, an enzyme that breaks down the clotting protein called von Willebrand factor. Without sufficient levels of ADAMTS13 in the blood, large pieces of von Willebrand factor can move around the blood and increase platelet clotting and red blood cell destruction. TTP can be either congenital or acquired. Congenital TTP is caused by a genetic mutation that affects the production or function of ADAMTS13, while acquired TTP is often caused by the development of autoantibodies that target and destroy ADAMTS13.
TTP can cause a wide range of other symptoms such as easy bruising and bleeding, fever, weakness, fatigue, and extreme paleness. There can also be episodes of unusually heavy bleeding or abdominal pain accompanied by nausea and vomiting. Treatment typically involves plasmapheresis, steroids, and other drugs such as rituximab. Plasmapheresis is the process of exchanging plasma through a machine.
Copyright © 2023 Elsevier, except certain content provided by third parties
Cookies are used by this site.
USMLE® is a joint program of the Federation of State Medical Boards (FSMB) and the National Board of Medical Examiners (NBME). COMLEX-USA® is a registered trademark of The National Board of Osteopathic Medical Examiners, Inc. NCLEX-RN® is a registered trademark of the National Council of State Boards of Nursing, Inc. Test names and other trademarks are the property of the respective trademark holders. None of the trademark holders are endorsed by nor affiliated with Osmosis or this website.
