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Lecture 32
Platelet Disorders
| Question | Answer |
|---|---|
| What is the life span of platelets? | 7-10 days |
| What is contained in alpha granules of platelets? | 1)von Willebrand Factor 2)Fibrinogen 3)Chemokines 4)Thrombospondin 5)P-selectin |
| What is contained in dense granules of platelets? | 1)ADP/ATP 2)Calcium 3)Serotonin |
| A X-linked congenital thrombocytopenia syndrome characterized by the presence of small platelets, immunodeficiency, and eczema. | Wiskott-Aldrich Syndrome (ACS) |
| A autosomal dominant congential thrombocytopenia syndrome characterized by large/pale platelets which lack alpha granules. | Gray platelet syndrome |
| A congential thrombocytopenia syndrome characterized by neutrophil inclusions and giant platelets. Other findings include hearing loss, cataract, and renal defects. | MYH9-related (May-Hegglin, Sebastian, Fechtner, Epstein) |
| What is pseudothrombocytopenia? | A result of platelet clumping in EDTA tube. Also large and giant platlets are miscounted by automated instruments. |
| What is the criteria for diagnosis of Immune Thrombocytopenic Purpura (ITP)? | It is a diagnosis of exlusion. 1)Plt count<150,000 2)Decreased plt survivial 3)Increased megakaryocytes in marrow 4)Normal WBC number and morphology 5)Normal spleen size |
| Age of onset of Acute vs. Chronic Immune Thrombocytopenia Purpura | Acute ITP occurs in preadolescent population. Chronic ITP occurs in post-adolescent population. |
| Sex predilection of Acute vs. Chronic Immune Thrombocytopenic Purpura (ITP) | Acute ITP: 1:1 (M:F) and Chronic ITP 1:3 (M:F) |
| Typical platelet count in Acute vs. Chronic Immune Thrombocytopenic Purpura (ITP) | Acute ITP has platelet count <20,000. Chronic ITP has platelet counts between 20-80,000 |
| (T or F) Acute Immune Thrombocytopenia Purpura typically resolves on its own. | True. |
| (T or F) Chronic Immune Thrombocytopenic Purpura is self-limited. | False. Chronic ITP does not resolve without treatment. |
| What lymphoproliferative disorder is associated with ITP? | CLL |
| What autoimmune disorder is commonly associated with ITP? | SLE |
| What infections are commonly associated with ITP? | CMV, HIV |
| What is the treatment for ITP with platelet counts <20,000? | Steroids, IVIg, anti-D |
| What is the treatment for ITP with platelet counts 20-30,000? | If asymptomatic, treat with prednisone. If symptomatic, treat with steroids, IVIg, and anti-D |
| What is the treatment for asymtomatic ITP with platelet counts 30-100,000? | No treatment is required |
| What is the treatment for ITP with platelet counts 30-100,000 and minor bleeding? | Prednisone |
| What is the treatment for ITP with platelet counts 30-100,000 and major bleeding? | Treat with Prednisone, IVIg, and anti-D |
| What are the available second line treatments for ITP? | Cyclophosphamide, Vinca alkaloids, Danazol, Vit C, Protein A immunoadsorption/Plasma exchange, Interferon alpha, Azathioprine, Amicar |
| (T or F) Relapse of ITP can occur after splenectomy. | True. Relapse typically occurs in first 6 months. |
| Describe the immune complex deposition of drug-induced thrombocytopenia | The inciting drug and antibody attach to the platelet surface and is an "innocent bystander". The platelets are removed by the reticuloendothelial system. |
| What are common drugs that incite immune complex deposition drug-induced thrombocytopenia? | Quinidine, NSAIDs, Gold, Sulfa, Dilantin |
| Describe the Hapten type drug-induced thrombocytopenia | The inciting drug attaches to the platelet surface, forming a hapten. Antibodies are formed aganist the hapten and as a result the platelet/drug complex is removed by the recticuloendothelial system. |
| Pathogenesis of Heparin-Induced Thrombocytopenia Type II | It is an immune-mediated condition. IgG directed aganist PF4-Hep complex on platelets. The IgG binds to platelet Fc(gamma)II receptors, promoting platlet activation. Platelet activation induces platelet clumping and fibrin formation. |
| What is the treatment of Heparin-Induced Thrombocytopenia Type II | Discontinue heparin therapy |
| When is the typical onset of HITT? | 1)5-10 days after heparin start 2)Within 24 hours if recent exposure to heparin (<3 months) |
| What is the typical change in platelet count in HITT? | 50% decrease from baseline platelet count or platelet count <100,000 |
| What are the clinical manifestations of Thrombotic Thrombocytopenic Purpura? | 1)Low platelet count 2)Fever 3)Renal Failure 4)Neurologic findings 5)Microangiopathic hemolytic anemia |
| Pathogenesis of TTP | Either congential lack of ADAMSTS13 enzyme or acquired antibodies to ADAMSTS13 enzyme |
| What is the treatment for TTP? | Plasma exchange |
| An obstetric complication characterized by low platelets, hypertension, and proteinuria. | Preeclampsia |
| An obstetric complication characterized by elevated liver enzymes, low platelets, and hemolytic anemia. | HELLP (H)emolytic anemia, (E)levated liver enzymes, (L)ow (P)latelet count |
| A common cause of renal failure in children associated with E. coli. Clinical manifestations include low platelet counts, fever, and microangiopathic hemolytic anemia. | Hemolytic Uremic Syndrome |
| What is the inheritance pattern of Bernard Soulier Disease? | Autosomal recessive |
| Pathogensis of Bernard Soulier Disease | It is a disorder of platelet adhesion due to the absence of gycoprotein Ib receptor on the platelet |
| Pathogenesis of Glanzmann Thrombasthenia | An intrinsic platelet defect associated with defect in Glanzmann IIbIIIa receptors on platelets resulting in defect in platelet aggregation. |
| (T or F) Each unit of platelet concentrate is from an individual donor. | True. |
| How long are platelets viable at room temperature on a rocker? | 5 days |
| Definition of Thrombocytosis | Platelet count>450,000/uL |
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