Thrombocytopenia/Thrombotic Microangiopathies/Coagulation Disorders (48)
About this set
Created by:
stevenypark Plus on September 30, 2011
Subjects:
Log in to favorite or report as inappropriate.
Order by
93 terms
Terms | Definitions |
|---|---|
 List 4 general causes of thrombocytopenia. | Decreased production Increased consumption/destruction Sequestration (hypersplenism) Massive transfusion (dilution): transfused blood does not have too many platelets |
| Spontaneous bleeding platelet count | < 20,000/microliter |
| Post-traumatic bleeding platelet count | < 50,000/micro liters |
| Describe platelet count, bleeding time, prothrombin time, and partial thromboplastin time in thrombocytopenia. | decreased platelet count increased bleeding time normal prothrombin time normal partial thromboplastin time |
| Patient presents with petechiae, ecchymoses, epistaxis, mucosal bleeding and CNS hemorrhages | thrombocytopenia *CNS hemorrhages are the most severe complication of low platelet counts. |
| List 2 general causes of decreased platelet production. | bone marrow failure decreased megakaryocytic in bone marrow |
| List 3 causes of bone marrow failure. | aplastic anemia myelophthisic anemia myelodysplastic disorders chemotherapy (bone marrow transplant) drug reactions infections |
| List 2 autoimmune disorders that lead to destruction of platelets. | Idiopathic thrombocytopenic purpura Systemic lupus erythematosus: thrombocytopenia is the most common bleeding disorder in SLE. |
| List 3 drugs that can cause autoimmune destruction of platelets. | quinidine heparin sulfa |
| List 3 viruses that cause infection-associated autoimmune destruction of platelets. | EBV HIV CMV *may also decrease platelet production |
| What is the usual cause of idiopathic thrombocytopenic purpura in children? | viral infection |
| Idiopathic thrombocytopenic purpura adult sex and age range | females 20-40 years of age |
| List 2 autoimmune antibodies created in adults afflicted with idiopathic thrombcytopenic purpura. | anti-platelet membrane Gp 2b/3a anti-platelet membrane 1b/1x |
| Widespread formation of thrombi in microcirculation due to platelet and fibrin deposition. | thrombotic microangiopathies |
| List 2 components of thrombi in thrombotic microangiopathies. | platelets fibrin |
| Thrombotic microangiopathies RBC morphology | schistocytes |
| List 2 end organ dysfunctions that occur in thrombotic microangiopathies. | renal failure mental status changes |
| Review platelet clotting. |  |
| List 2 types of thrombotic microangiopathies. | thrombotic thrombocytopenic purpura hemolytic uremic syndrome |
| Adult patient presents with fever, CNS changes, schistocytes, and thrombotic microangiopathy | thrombotic thrombocytopenic purpura (classic pentad) |
| What causes abnormal platelet activation in thrombotic thrombocytopenic purpura? | metalloprotease deficiency (ADAMTS13) <-- either inherited deficiency or autoantibody (most common) *ADAMTS13 keeps Von-Willebrand factor at a certain size. Absence of the metalloprotease allows VWF to get very large and cause platelet aggregation. |
| Thrombotic microangiopathy that affects adults | thrombotic thrombocytopenic purpura |
| Thrombotic microangiopathy that affects children | hemolytic uremic syndrome |
| Platelet dysfunction clinical features (3) | petechiae ecchymosis excessive bleeding |
| List 3 causes of platelet dysfunction. | NSAIDs Uremia Vonn Willebrand disease |
| Child presents with renal failure, and lack of CNS changes | hemolytic uremic syndrome |
| Thombotic microangiopathy associated with endothelial cell injury due to gastroenteritis caused by E. coli toxin | hemolytic uremic syndrome |
| Platelet dysfunction bleeding time, platelet count, prothrombin time, partial thrombplastin time | increased bleeding time, the rest are normal |
| What is the most common cause of acquired coagulation disorder? | vitamin K deficiency |
| What 4 coagulation factors are decreased due to vitamin K deficiency? | factor 2 factor 7 factor 9 factor 10 |
| Which coagulation has the shortest biological half-life? | factor 7 |
| List 4 causes of acquired coagulation disorders. | Vitamin K deficiency Liver disease: most coagulation factors are made in the liver Massive transfusion DIC |
| Where is factor 8 synthesized? | in the liver |
| Factor 8 fx | activates factor 10 (intrinsic pathway) |
| Patient presents with normal PTT and prolonged PT. | factor 7 deficiency |
| Disorder characterized by deficiency of coagulation factor 8 | hemophilia A |
| Hemophilia A pattern of inheritance | X-linked recessive |
| List 2 binding targets for Von-Willebrand factor. | collagen platelet membrane GP1b |
| Where is Von-Willebrand factor synthesized? | endothelial cells |
| Von-Willebrand disease pattern of inheritance | autosomal dominant |
| Type 1 Von-Willebrand disease bleeding time, platelet count, prothrombin time, partial thromboplastin time | increased bleeding time normal platelet count normal prothrombin time increased partial thromboplastin time (decreased factor 8) |
| What type of Von-Willebrand disease is associated with decreased high molecular weight VWF multimers? | type 2 *opposite of thrombotic thrombocytopenic purpura |
| What type of Von-Willebrand disease is associated with decreased circulating Von-Willebrand factor? | type 1 --> secondary decrease in factor 8 |
| Factor 8 deficiency with moderate symptoms | 1-5% |
| Factor 8 deficiency with severe symptoms | <1% |
| Disorder characterized by decreased high molecular weight Von-Willebrand multimers | Type 2 Von-Willebrand disease |
| What percent of people with hemophilia A have antibodies against factor 8? | 15% |
| What is the most common hereditary deficiency associated with serious bleeding? | hemophilia A (factor 8 deficiency) |
| Which sex is more often afflicted with hemophilia A? | males (X-linked) |
| Hemophilia A bleeding time, platelet count, prothrombin time, partial thrombplastin time | increased bleeding time normal platelet count normal prothrombin time increased partial thromboplastin time |
| Patient complains of easy bruising and massive bleeding after trauma (hemorrhage into joints as well). | hemophilia A |
| Hemophilia A Rx | purified or recombinant factor 8 |
| Factor associated with hemophilia B | factor 9 deficiency |
| Hemophilia B pattern of inheritance | X-linked recessive |
| Disseminated intravascular coagulation bleeding time, platelet count, prothrombin time, partial thromboplastin time | all abnormal |
| List 3 general causes of DIC. | obstetric complications infections neoplasia (M3) massive tissue injury miscellaneous (acute IV hemolysis, snake bites, shock, heat stroke, vasculitis, aortic aneurysm, liver disease) |
| Review pathophysiology of DIC. |  *In thrombotic thrombocytopenic purpura, only platelets are consumed; DIC consumes both platelets and coagulation factors. 12 |
| Review morphology of DIC. |  |
| Acute DIC clinical presentation | bleeding predominates |
| Chronic DIC clinical presentation | thrombosis predominates |
| Review clinical course of DIC. |  |
| Mild splenomegaly weight | < 500 g |
| Moderate splenomegaly weight | 500-1000 g |
| Severe splenomegaly weight | > 1000 g |
| List 2 consequences of splenomegaly. | hypersplenism rupture |
| Acute splenitis (blood-borne infection) splenomegaly | mild splenomegaly |
| Acute splenic congestion (central venous congestion) splenomegaly | mild splenomegaly |
| Infectious mononucleosis splenomegaly | mild splenomegaly |
| Chronic congestion (portal hypertension, splenic vein obstruction) splenomegaly | moderate splenomegaly |
| Acute leukemias splenomegaly | moderate splenomegaly |
| Hereditary spherocytosis splenomegaly | moderate splenomegaly |
| Thalassemia major splenomegaly | moderate splenomegaly |
| Autoimmune hemolytic anemia splenomegaly | moderate splenomegaly |
| Amyloidosis splenomegaly | moderate splenomegaly |
| Langerhans histiocytosis splenomegaly | moderate splenomegaly |
| Chronic splenitis due to infective endocarditis splenomegaly | moderate splenomegaly |
| Granulomatous diseases splenomegaly | moderate splenomegaly |
| Metastatic neoplasm splenomegaly | moderate splenomegaly |
| Long-standing congestion splenomegaly | severe splenomegaly |
| Chronic myeloid leukemia splenomegaly | severe splenomegaly |
| MMMF splenomegaly | severe splenomegaly |
| Chronic lymphocytic leukemia splenomegaly | severe splenomegaly |
| Hairy cell leukemia splenomegaly | severe splenomegaly |
| Lymphomas splenomegaly | severe splenomegaly |
| Malaria splenomegaly | severe splenomegaly |
| List 3 autoimmune disorders that present with lymphoid follicles in the thymus (thymic hyperplasia). | Myasthenia gravis: present in 65-75% of cases Systemic lupus erythematosus Rheumatoid arthritis |
| In thymoma, what component of the thymus undergoes neoplastic proliferation? | epithelial component |
| Type 1 thymoma morphology and behavior |  |
| Type 2 thymoma morphology and behavior | |
| Thymoma age group | middle age |
| List a common location of thymomas. | anterior superior mediastinal mass (30-40%) |
| Patient presents with a cough, shortness of breath, and superior vena cava syndrome | thymoma in anterior superior mediastinal compartment |
| What systemic disease is associated with thymomas? | myasthenia gravis (30-45%) *15-20% of myasthenia gravis patients have thymoma |
First Time Here?
Welcome to Quizlet, a fun, free place to study. Try these flashcards, find others to study, or make your own.