Term
| hereditary hemmorhagic telnectasia |
|
Definition
| small clusers of fragile blodo vessels, immune defect and mucosal bleeding .fairly common. |
|
|
Term
|
Definition
| defect of collagen, platelets are fine but are unable to bind dto collagen- bleeding , hyperelasticskin. |
|
|
Term
| thrombocytopenia absent radii |
|
Definition
| autosomal recessive, no radii, low platelets. |
|
|
Term
|
Definition
| x linkeed, defect in WAS gene (11:22), tiny platelets. Tcell deficiency, problaby autoimmune. |
|
|
Term
|
Definition
| dohle bodies- big platelets, and dohle bodies. Abnormality of granulocytes and platelets. |
|
|
Term
|
Definition
| plalelt (anti gp2b3a) auto-antibodies, megakaryocyte auto-antibodies , T celldefects. , relative tpo deficiency |
|
|
Term
|
Definition
| Viral infections, vaccines, known immundisordersandautoimmunediseases |
|
|
Term
| microagiopahic hemolyticanemia. |
|
Definition
| thrombocytopenia and anemia , withschistocytes in blod smear. |
|
|
Term
|
Definition
| pathological activatio fo coagulation due to severeunderelyingpathology. Severe clottign of al lsmall vessels, clots consume resources, when resources runotu leadstoabnormal bleding. Causes: serious illnesses, damage to endo suracess, stagnation ofblodo flow, ifnections ( stop making new thrombi- sepsis,trauma, obstetric emergency, pancreatisis, nephrotic syndrome, transfusion). Lab: decrease of all plottin factos/ proteins, increase in fibrin split products. Fragmentederythrocytes. Treat udnerlyign disease. Supportive care, replacement of factors . heaparin and antithromin in some indicatoins |
|
|
Term
| Thrombotic Thrombocytopenia purpura |
|
Definition
| amadts13 defiency- platelets clump to uncleaved VWF and agglutinate. Adamts13 deficiency (inherited , rare), or antibodies (acquired). Pentad: thrombocytopenia, micoangiopathic hemolytic anemia, neuro abnormalities, renal failure, fever. Treatment: plasmapheresis or plasma infusion. Diagnosis: shicstocytes, thrombocytopenia, elevated ldh, eelvated adamst13 AB |
|
|
Term
| hemolytic uremic syndrome |
|
Definition
| Caused by E. Coli fecal contaminationSimliar to pentad of TTP- hemolytic anemia, renal failure, NO NUERO PROBLEMS, GI PROBLEMS INSTEAD, thrombocytopenia, . Treatment:supportive care, renal dialysis if needed. NO antiobitics, plasmapheresis wont work |
|
|
Term
| differentiation btween ttp and DIC |
|
Definition
| bothpresentwith low platelets and schistocytes. Coag screens- normal in TTP, abnormal in DIC. Fibrinogen low in DIC normal in TTP. FSP- high inDIC, minmal in TTP. |
|
|
Term
| Bernard- SOuliderSyndrome |
|
Definition
| Autosomaal recessvie. Defieicny of GP Ib. hallmark: large plateletes (like myh-9). Diagnosis- look at aggregation studies- put in a variety of activators find that aggregation isormal. Howver when put in just ristocetin , orristocen and von willebrand factor, finding no aggregation whatsoever. |
|
|
Term
|
Definition
| becausebinds factor VIII, will see low levels of factor VIII (half life is very short when free). Type I don’t make enough VWF- type 3- complete absence. Type 2- present but screwed up . Diagnosis; PTT may be long, (PT normal), PFA is prolonged and low VWF antigen. |
|
|
Term
| congenital afibrinogenmia |
|
Definition
| absence of plasma fibrinogen |
|
|
Term
| Glanzmann thrombatshenemia |
|
Definition
| Autosomal recessive: severe mucutaneous bleeding. Defiiency of GP Iib/IIIa. Diagnosis:this time only thign normal is ristocetin binding (this is dependant on VFW). All other activators will cause no aggregation. |
|
|
Term
|
Definition
| absence of dense bodies- Oocultocutaenous albinism. |
|
|
Term
| idiopathic dense granueldisorder |
|
Definition
| most common platelet defect. Unkonwn defect in densegranules. Treatment for dense granule disorders: avoid giving platelets, this may cause Ab development. Instead avoid thigns that may decrease paltelet functions e.g. alcool and and NSAIDs. |
|
|
Term
|
Definition
| Factor VIII deficieny. Hallmark: bleeding into muscles and joints. Risk is intracranial hemmorhage classficaition: 1 percent = severe, joint disease probable, moderate: 1-5 percent , mild: 5-50 percent- occurs only with real injury. X linked recessive but women may exhibit some deficienies as carriers treatment: factor replacement concentrates. |
|
|
Term
|
Definition
|
|
Term
|
Definition
| Factor 11 deficiency- clincially mild, no joint bleeding. |
|
|
Term
|
Definition
| lack o fibrin cross linking. Delayed bleeding PT and PTT appear normal. Clincial test: urea clot lysis time. |
|
|
Term
|
Definition
| Sevre protein Ca defieincy, rapidly progressing to DIC. Usually happens shortly after birth |
|
|
Term
|
Definition
| usually benign, may resutl in prolonged PTT (there is phospholipid in assay). But also pathological system which results in actiation of aogulation (due to interaction with endothelial cells) |
|
|
Term
|
Definition
| petechialrash. Purpura- bruising. . Intracranial hemmorhage risk. Trnasfused platelets destroyed rapidly, |
|
|
Term
|
Definition
| Tons of mega on bm , paucity of platelets on smear |
|
|
Term
|
Definition
| Treatmetn: increasepaltelet count, decrase bleeding.: immunosupressants, tpo agonist. Generally goes away on its own. Treatment not curative. |
|
|
Term
| primary cauases for lwo platelte ocunti n patient |
|
Definition
| decreased prodcution, increased destrucitn or sequestration. |
|
|
Term
| most common form of platelet decreased |
|
Definition
| acquired decerasedp roduction |
|
|
Term
| cuases of platelet decrese |
|
Definition
| infection drugs alcohol, bm fialure |
|
|
Term
| MPV as clue for decreased platelets |
|
Definition
| larger MPV means moren ew platelets churned out which proablyb means destruction. |
|
|
Term
|
Definition
| acute- children, equal malesto fmeals, seasonal onset : viral ifnections. preceedingienfceiton all most allthtiem, acute in onset, plaetlet cound very low lesthan 20. Bleeding risk is low- brand ne w pllateltes thatworkvery well. Duration- can get better on its own (supportivecare) |
|
|
Term
| mechanisms of antiplatelet antibody formation |
|
Definition
| plateletm embrane autoantigen, hapten, adsobred immune compelxes for other antigens. |
|
|
Term
|
Definition
| liver disease, portal hypertension, infection, storage disease |
|
|
Term
| Hemophelia comlication to treatment |
|
Definition
| INHIBITORS; binding to factor 8 and 9 antibodies, less common for factor 9 |
|
|
Term
|
Definition
| may cause splenomegaly= sequestration, also decrease of vitamin k depepdnant clotting factors in lvier disease |
|
|
Term
| acquried factor defificeines |
|
Definition
| immune mediated. Factor 8: cancer autoimmune disease pregnancy; factor Vf; bovine thrombine, thrombin: antiphosopholipid luekemia |
|
|
Term
| prolonged ptt, deficiency or inhibitor |
|
Definition
| if put in 100 percent ptt and it corrects, means its deficiency, if doesntt, inhibitor. Things that don’t correct: factor antibodies, factors to ptt reagants, anticoagulants |
|
|
