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What disorder physiologically does hereditary telangiectasia cause?
vessels have thin walls due to defective conective tissue and endothelial junctions are abnormal
What are the symptoms of hereditary telangiectasia?
hemorrhage, flat red or purple lesions on mucus membranes, GI tract, palms, soles or feet.
What testing is abnormal with hereditary telangiectasia?
the Rumpel-Leede test
What is the prognosis and treatment for hereditary telangiectasia?
fatal if homozygous, supportive treatment
What defect physiologically does Erhler's-Danlos Syndrome cause?
Defective synthesis of collagen and maybe elastin
What are the symptoms of Erhler's Danlos syndrome?
overextendability of joints, hyperelasticity of skin, fragility of skin, pseudotumors, bleeding due to fragile vessels
what tests are abnormal in Erhler's Danlos?
rumpel-Leede and bleeding time
What is the prognosis and treatment for Erhler's Danlos?
rarely fatal, supportive
What is the cause and physiological findings of senile purpura?
Decrease in collagen overtime, decreases the support of capillaries, so small vessels rupture with little or no traumaBruising occurs
What is the cause of Scurvy?
Deficiency of vitamin C causes reduced or faulty synthesis of collagen
What are the symptoms of Scurvy?
Bleeding from gums and hair follicles, ecchymoses, and intramuscular hemorrhage
What is the treatment for Scurvy?
Oral vitamin C
what is the cause of amyloidosis?
deposits of light chains on blood vessel walls weaken them.
What are the symptoms of amyloidosis?
bruising and bleeding into visceral organs, epistaxis, thrombosis, hemorrhage into retina
What is the prognosis and treatment for amyloidosis?
fatal , no treatment except organ transplant
What are the clinical findings of thrombocytopenia?
<50,000 petechiae, ecchymoses, purpura<30,000 menorrhagia, petechiae, bruising with no trauma<10,000 severe spontaneous bleed
what is the most common cause of excessive or abnormal bleeding?
What happens in idiopathic throbocytopenic purpura?
immune destruction of platelets
What type of people usually get acute ITP?
children following a viral infection
What are the symptoms of acute ITP?
abrupt onset of bruising, petechiae, bleeding from mucous membranes
What are the lab findings of acute ITP?
Low plt ct (<20,000), inc megakaryocytes in bone marrow
What is the prognosis and treatment of acute ITP?
usually goes into spontaneous remissionin 2-6 weeks. Doesn't usually recur.treated by preventing trauma and giving steroids to reduce risk of bleedingDon't give plts!
Who usually gets chronic ITP?
Women 20-40 yrs old are 3x more likely to get it than men
What are the symptoms of chronic ITP?
Insidious onset, bruising and bleeding proportional to the dec in plt ct
what are the lab findings in chronic ITP?
plt ct 100,000-30,000, inc megakaryocytes in bone marrow, plt autoantibodies
What is the treatment and prognosis for chronic ITP?
steroids to inc plt count by reducing sequestration of pltsintermittnent and may relapse
What is ITP in pregnancy?
Develops in pregnancy and goes away slowly after delivery. have c-section
What is transplacental thrombocytopenia?
Newborns of ITP moms have dec plt ct due to maternal IgG plt abs. lasts until abs are cleared.
What is type 1 drug induced thrombocytopenia?
Quinidine induces antibodies to form against it that also react with plts. plt ct <10,000
What is type 2 drug induced thrombocytopenia?
Heparin induced.immune mediated resonse in plts causes them to aggregate and produce thrombi.inc with prolonged or repeat heparin therapylow molecular weight heparin has a lower incidence
What is the treatment for HIT?
stop heparin, use coumadin to anticoagulate. Don't give platelets!
What is immune thrombocytopenia?
secondary to other diseases - SLE and RA
What are 4 causes of non-immune plt destruction?
DICTTP -plt aggregation and thrombosisHUSArtificial heart valves - mechanic destruction
What is secondary or reactive thrombocytosis?
inc in plts due to infection, trauma, or post splenectomy
What is primary thrombocytosis?
inc in production by BM due to myloproliferative disorders
what type of disorder is Bernard-Soulier?
platelet adhesion disorderplts doent' adhere to subendothelial collagen due to lack of glycoproteins on plt surface, esp. GP Ib/IX that interacts with vWF
What are the symptoms of Bernard Souliers?
easy bruising, epistaxis, GI bleed, inc menses
What are the lab findings for Bernard Soulier?
plt ct = normalBT = incPlatelet aggregation abnormal with Ristocetin>60% of plts are giantPlt function normal with epi and collagen
What is the prognosis and treatment of Bernard-Souliers?
not fatal and supportive
What type of disorder is Glanzmann's Thrombasthenia?
Primary plt aggregationplts can't aggregate due to deficient GP IIb-IIIa
What are the symptoms of Glanzmann's?
in infancy - epistaxis, gum bleeding, mod superficial bleeding tendency
What are the lab results for Glanzmann's?
No platlet aggregation to ADP, collagen or epiPlt ct and morph normalBT is increased
What is the prognosis and treatment of Glanzmann's?
rarely fatal and supportive
What is another name for Chediak-Higash and Wiscott-Aldrich?
Storage pool disease
what is the cause of chediak-higashi and WAS?
plt secretion disorder - defective secretion due to a deficiency in the platelet dense granules
What are the lab tests assoc with chediak higashi and WAS?
plt ct = normalBT incPlatelet aggregation is abnormal with ADP, collagen and epi = ADP and collagen show primary wave, but no secondary wave with epi
What are the symptoms of chediak higashi and WAS?
mild to moderate bleeding,
What is the prognosis and treatment for Chediak higashi and WAS?
not usually fatal and supportive treatment
What is uremia?
Waste products in blood interfere with platelet function
How does aspirin affect platelets?
inhibits prostaglandin synthesis, without prostaglandin synthesis there is no thromboxane A2, which leads to no mobilization of calcium, which inhibits contraction of the plt membrane...
How does Ibuprofen effect platelets?
It temporarily interferes with plt aggregation. When drug clears from blood plt function returns
How does alcohol affect plts?
prolonged exposure can cause dysfunction
How does a cardiac by-pass machine affect plts?
It renders them functionless for up to 8 hrs post-op.
How is vW disease inherited?
autosomal dominant or recessivehomo is more severemore severe in children
What is the physiology behind vW disease?
deficient vWF causes instability of the Factor VIII molecule and renders it functionless in the intrinsic pathway
What are the symptoms of vW Disease?
excessive nosebleeds, bleeding gums, menorrhagia, excessive bleeding after toothe extraction or dental procedures, petechiae, and purpura
what are the lab results for vW disease?
BT = incePT = normalPlt ct = normalPTT = incRistocetin cofactor = abnormalFactor VIII antigen = decvWF antigne assay = dec
what is the treatment for vW disease?
cryoppt, DDAVP -stim release of vWF from epith. cells, FFPtreated symptomatically
How many types of vW disease are there?
type I, Type II, and Type III
how is Factor VIII deficiency inherited?
homozygously= more severehetero = no symptomssex-linkedcauses 80% of bleeding disorders
What are the symptoms of Factor VIII def.?
hemarthroses, hematomas with sl. trauma, intramuscular, hemorrhages, delayed bleeding, problems with circumcision, deep bleeding in tissue, GI tract and kidneys
what are the lab results for Factor VIII def?
PT=normalPTT=incplt ct = normalBT = normalMixing studies corrected with adsorbed plasmaFactor VIII = dec
What is the treatment for Factor VIII disease?
cryoppt, Factor VIII conc, DDAVP for those that can produce some factor VIII, recombinant factor VIII, topical thrombin, cold compress
What is the inheritance of Hemophilia B?
homo=symptomaticsex-linkedhetero = no symptoms10-15% of hemophilias
What are the symproms of hemophilia B?
hemarthroses, hematomas, with slight trauma, intramuscular hemorrhages, delayed bleeding, problems with circumcisionDifferent levels of severity
What are the lab results for hemophilia B?
PT=normalPTT= incBT = normalplt ct = normalFactor IX assay = decMixing studies corrected with serum and aged plasmaplt function = normal
What is the treatment for hemophilia B?
FFP, Prothrombin conc, recombinant factor IX
How is afibrinogenemia inherited?
what are the symptoms of afibrinogenemia?
spontaneous hemorrhage in GI, mucosal, joints, bruises and umbilical cord
What are the lab results for afibrinogenemia?
PT = incPTT= incethrombin time = incReptilase time = incFibrinogen assay = < 10 mg/dl
What is the treatment for afibrinogenemia?
medical management by giving cryoppt and ffp
What is the inheritance of hypofibrinogenemia?
heterozygous form of afibrinogenemiacan also be acquired during liver disease, renal disease, and DIC
What are teh symptoms of hypofibrinogenemia?
mild, spontaneous bleeding severe, post-op bleeding
what are the lab results for hypofibrinogenemia?
PT= incPTT= inceFibrinogen assay = 20-100 mg/dl
What is the treatment for hypofibrinogenemia?
What is the inheritance of homozygous dysfibrinogenemia?
autosomal dominant - homo
What are the symptoms of homozygous dysfibrinogenemia?
spontaneous hemorrhage in GI, mucosal, joints, bruise, umbilical cord
What are the lab results of homozygous dysfibrinogenemia?
PT = incPTT = incTT =incReptilase time = incFibrinogen assay = normal
what is the treatment for homozygous dysfibrinogenemia?
What is the inheritance for hetero. dysfibrinogenemia?
autosomal dominant - hetero
What are the symptoms of hetero. dysfibrinogenemia?
mild, spontaneous bleeds, severe post-op bleeds
What are the lab results for hetero. dysfibrinogenemia?
fibrinogen level = normalPT=incPTT=incThrombin Time=increptilase time = inc
What is the inheritance of Factor V def.?
autosomal recessivehomo = symptomshetero = silent
What are the symptoms of Factor V def?
mild bleeding, mucosal bleeding, echymoses,
what are the lab results for factor V def?
PT = inc, PTT=incBT=incmixing studies=corrected with absorbed plasmaFactor V assay = dec
What is the treatment for Factor V def?
What is the inheritance of Factor XIII def?
What are the symptoms of FactorXIII def?
severe bleeding, spont. or delayed bleeds, delayed umbilical stump bleed, abnormal scar formation
What are the lab results for Factor XIII def?
PT=normalPTT = normalUrea solubility test is used to confirm the diagnosis
What is the treatment for Factor XIII def?
FFP or cryoavoid aspirin
What is the inheritance of Factor II def?
What are the symptoms of factor II def?
severe bleeding after trauma, skin and mucosal bleedinghetero = bleed with surgery
What are the lab tests for factor II def?
PT =incPTT= incBT=normalTT=normalFactor II assay=decMixing studies corrected with absorbed plasmadifferentiate from Vitamin K def by looking for other factors in group
What is the treatment for Factor II def?
Prothrombin conc, or FFP
How is Factor VII def inherited?
what are the symptoms for Factor VII def?
severe bleeding, skin, mucosal and joint bleeding
what are the lab results for Factor VII def?
PT=incPTT=normalMixing studies corrected with serum and aged plasmaFactor VII=dec
What are the treatments for Factor VII def?
Prothrombin conc, FFPdon't use coumadin
What is the inheritance of Factor X def?
autosomal recessivevery rare
What are the symptoms of factor X def?
mild to sever bleeding, skin and mucosal bleeding
What are the test results for factor X def ?
PT=incPTT=incRussel Viper Venom Time = incMixing studies = corrects with serum and aged plasmaFactor X assay = dec; homo = 8-10%, hetero=50%
What is the treatment for Factor X def?
prothrombin complex conc, FFP
How is factor XI def inherited?
autosomal recessivecommon in russians and asheninzic Jews
what are the symptoms or Factor XI def?
mild bleeding with trauma or surgery, epistaxis, hematuria, menorrhagia
What are the lab results with Factor XI def?
PT=normalPTT=incFactor XI assay=decMixing studies correct with serum, adsorbed and aged plasma
What is the treatment for Factor XI def?
FFPusually not severe enough to treat
What is the inheritance of Factor XII def?
What are the symptoms of Factor XII def?
no bleeding embolic and thrombolic
what are the lab results for Factor XII def?
PT=normalPTT=abnormalMixing studies corrected by all
What is the treatment for Factor XII def?
What is the inheritance of HMWK def?
What are the symptoms of HMWK def?
tendency to clot
what are the lab results for HMWK def?
PT=normalPTT=incMixing study correct if incubate for 2hrs
What is the treatment for HMWK def?
no treatmentnot a major problem
what is the inheritance for PK def?
what are the symptoms of PK def?
tendency to clot
what are the test results of PK def?
PT=normalPTT=incFletcher Factor screen used to diagnose the diseaseextra long incub period for mixing study
what is the treatment for PK def?
What causes DIC?
tissue damage that releases excess tissue factor into the peripheral blood and activates the extrinsic pathway or endothelial damage exposes collagen that activates the intrinsic pathway
What happens in DIC?
Uncontrolled fibrin formation in blood vessels when there is no damage. depletes clotting factors and plts which leads to severe bleeds
What are the symptoms of DIC?
What are the lab results for DIC
PT and PTT=incFibrinogen=decP;t ct=decDDimer=incschistocytes in peripheral blood
What is the prognosis and treatment for DIC?
acute is usually fatalgive heparin to encourage inactivation of thrombincan try antithrombin III conc
Why does liver disease cause severe coag problems?
clotting factors not produced in adequate quantities, diseased liver macrophages don't remove activated clotting factors or FDP's as well as a healthy liver, which could lead to a large...
What are the lab results with liver disease?
PT and PTT=incFibrinogent=decplt ct=normal to decFDP and D-diner=incliver studies=abnormal
What is the treatment of liver disease?
replacement products as needed
What is Vitamin K used for in coag?
to make Factors II, VII, IX and X, and Protein C and PRotein S
what are the lab results of vitamin K def.?
PT and PTT = inctreat with vitamin K
What is lupus anticoagulant?
ani-plt antibody that develops due to lupus, inc incidence of DVT, and miscarriage
what are the lab results with a lupus anticoag?
PTT=incMixing studies show presence of inhibitorconfirm with anti-phospholipid testing
what is treatment for lupus anticoagulant?
What is an anticardiolipin antibody?
anti-plt antibody, implicated in inc risk of thrombosis
what are the lab results of an anticardiolipin antibody?
tested for by reference facilities
What are the Factor VIII and Factor IX inhibitors?
single factor inhibitors assoc. with respective hemophilias, VIII may develop in person without hemophilialooks like hemophilia
What are the lab findings of Factor VIII and IX inhibitors?
same as hemophilias
What is the prognosis and treatment for Factor VIII and Factor IX inhibitors?
often causes deathmust give large amts of cryo or factors to try to temp overwhelm the inhibitor giving the body a chance to stop bleeding
What drugs can be given to treat thrombosis?
heparin, oral, antiplatelet drugs, thrombolytic drugs
what is heparin's mode of action?
complexes with ATIII which greatly enhances its affinity for thrombin. after the ATIII complexes with thrombin, the heparin breaks away and complexes again with another ATIII
how is heparin monitored?
What is the antidote of heparin?
what are the potential problems with heparin?
overanticoagulation could result in bleed, HIT, pt must be hospitalized to get it and must monitor 1-2 times per day
what is the benefit of heparin?
action is immediate
What is coumadin's mode of action?
vitamin K antagonist. Binds vitamin K so less is available to the liver for synthesis of clotting factors
How is coumadin monitored?
What are the potential problems of coumadin?
doesn't take effect immed., vitamin K intake by pt must be monitored, Protein C and S are affected
what is the benefit of coumadin?
can take at home
what is the antidote of coumadin?
What is the mode of action for thrombolytic drugs?
breaks up existing clot by activating plasminogen to plasmin.
What is thrombolytic drugs used for?
What are the lab results for thrombolytic drugs?
must do baseline coag studies prior to administration
What are 3 types of thrombolytic agents?
urokinase, streptokinase, and TPA
What are 7 causes of hypercoagulability?
Antithrombin III defProtein C defProtein S defDysfibrinogenemialupus anticoagulantanticardiolipin antibodyplasminogen def
what factors are in serum?
Factors VII, IX, X, XI, XII
What factors are in absorbed plasma?
Factors I, V, VIII, XI, XII
What factors are in aged plasma?
Factors I, II, VII, IX, X, XI, XII
What factor is fibrinogen?
What factor is prothrombin
What factor is Tissue thromboplastin
What factor is calcium
What factor is labile
What factor is stable/
What factor is antihemophilic factor
What factor is Christmas factor?
what factor is Stuart Prower?
what factor is plasma thromboplastin?
What factor is Hageman factor
What factor is stabilizing factor?
what factor is fletcher factor?
What factor is fitzgerald factor?
what are the 6 coag inhibitors?
antithrombin III, Protein C, Protein S, alpha 2- macroglobulin, alpha 1-antitrypsin, C1-inactivator
What is the fibrinolytic agent?
what are the 2 fibrinolysis inhibitors?
alpha 2-antiplasmin and alpha 2-macroglobulin
what factors are in the Intrinsic pathway?
VIII, IX, XI, XII, HMWK, Prekallekrein
what factors are in the extrinsic pathway?
What factors are in the common pathway?
I, II, V, X
What factors are tested with PT?
I, II, V, VII, X
what factors are tested with PTT?
I, II, V, X, VIII, IX, XI, XII, HMWK, Prekallekrein
what factors are in the contact group?
Prekallekrein, HMWK, XI, XII
what factors are in the prothrombin group?
II, VII, IX, X
What factors are in the fibrinogen or consumable group?
I, V, VIII, XIII
what is primary hemostasis?
Formation of platelet plug through plt adhesion, aggregation, and secretion
what is secondary hemostasis?
stabilization of the plt plug by fibrin formation via coagulation factors and the coagulation cascade.
What is fibrinolysis?
breakdown and rmoval of the clot after vascular repair
how is the Na citrate conc in the tubes corrected for high hct?
100-hct / 595-hct = /ml of bloodfor 5 ml tube= y x 5=ml of na citrate for 0.5-ml of na citrate=amt to removefor 3ml = yx3=ml of na citrate for 0.3ml-ml of na citrate = amt...
Which tests have clots as endproducts?
PT, PTT, Plt function, platelet aggregation, DRVVT,
Which tests are done using chromogenic method?
What tests us immunoassay methods?
D-Dimer, factor assays
What is considered abnormal results for a platelet function test?
with collagen and epi = < 170 means good function ...