Disorders of Coagulation

188 cards

causes, symptoms, tests and treatments of coagulation disorders.


 
  
Created Apr 3, 2011
by
biogirl10

 

 
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1
What disorder physiologically does hereditary telangiectasia cause?
 
vessels have thin walls due to defective conective tissue and endothelial junctions are abnormal
2
What are the symptoms of hereditary telangiectasia?
 
hemorrhage, flat red or purple lesions on mucus membranes, GI tract, palms, soles or feet.
3
What testing is abnormal with hereditary telangiectasia?
 
the Rumpel-Leede test
4
What is the prognosis and treatment for hereditary telangiectasia?
 
fatal if homozygous, supportive treatment
5
What defect physiologically does Erhler's-Danlos Syndrome cause?
 
Defective synthesis of collagen and maybe elastin
6
What are the symptoms of Erhler's Danlos syndrome?
 
overextendability of joints, hyperelasticity of skin, fragility of skin, pseudotumors, bleeding...
7
what tests are abnormal in Erhler's Danlos?
 
rumpel-Leede and bleeding time
8
What is the prognosis and treatment for Erhler's Danlos?
 
rarely fatal, supportive
9
What is the cause and physiological findings of senile purpura?
 
Decrease in collagen overtime, decreases the support of capillaries, so small vessels rupture...
10
What is the cause of Scurvy?
 
Deficiency of vitamin C causes reduced or faulty synthesis of collagen
11
What are the symptoms of Scurvy?
 
Bleeding from gums and hair follicles, ecchymoses, and intramuscular hemorrhage
12
What is the treatment for Scurvy?
 
Oral vitamin C
13
what is the cause of amyloidosis?
 
deposits of light chains on blood vessel walls weaken them.
14
What are the symptoms of amyloidosis?
 
bruising and bleeding into visceral organs, epistaxis, thrombosis, hemorrhage into retina
15
What is the prognosis and treatment for amyloidosis?
 
fatal , no treatment except organ transplant
16
What are the clinical findings of thrombocytopenia?
 
<50,000 petechiae, ecchymoses, purpura<30,000 menorrhagia, petechiae, bruising with no...
17
what is the most common cause of excessive or abnormal bleeding?
 
thrombocytopenia
18
What happens in idiopathic throbocytopenic purpura?
 
immune destruction of platelets
19
What type of people usually get acute ITP?
 
children following a viral infection
20
What are the symptoms of acute ITP?
 
abrupt onset of bruising, petechiae, bleeding from mucous membranes
21
What are the lab findings of acute ITP?
 
Low plt ct (<20,000), inc megakaryocytes in bone marrow
22
What is the prognosis and treatment of acute ITP?
 
usually goes into spontaneous remissionin 2-6 weeks. Doesn't usually recur.treated by preventing...
23
Who usually gets chronic ITP?
 
Women 20-40 yrs old are 3x more likely to get it than men
24
What are the symptoms of chronic ITP?
 
Insidious onset, bruising and bleeding proportional to the dec in plt ct
25
what are the lab findings in chronic ITP?
 
plt ct 100,000-30,000, inc megakaryocytes in bone marrow, plt autoantibodies
26
What is the treatment and prognosis for chronic ITP?
 
steroids to inc plt count by reducing sequestration of pltsintermittnent and may relapse
27
What is ITP in pregnancy?
 
Develops in pregnancy and goes away slowly after delivery. have c-section
28
What is transplacental thrombocytopenia?
 
Newborns of ITP moms have dec plt ct due to maternal IgG plt abs. lasts until abs are cleared.
29
What is type 1 drug induced thrombocytopenia?
 
Quinidine induces antibodies to form against it that also react with plts. plt ct <10,000
30
What is type 2 drug induced thrombocytopenia?
 
Heparin induced.immune mediated resonse in plts causes them to aggregate and produce thrombi.inc...
31
What is the treatment for HIT?
 
stop heparin, use coumadin to anticoagulate. Don't give platelets!
32
What is immune thrombocytopenia?
 
secondary to other diseases - SLE and RA
33
What are 4 causes of non-immune plt destruction?
 
DICTTP -plt aggregation and thrombosisHUSArtificial heart valves - mechanic destruction
34
What is secondary or reactive thrombocytosis?
 
inc in plts due to infection, trauma, or post splenectomy
35
What is primary thrombocytosis?
 
inc in production by BM due to myloproliferative disorders
36
what type of disorder is Bernard-Soulier?
 
platelet adhesion disorderplts doent' adhere to subendothelial collagen due to lack of glycoproteins...
37
What are the symptoms of Bernard Souliers?
 
easy bruising, epistaxis, GI bleed, inc menses
38
What are the lab findings for Bernard Soulier?
 
plt ct = normalBT = incPlatelet aggregation abnormal with Ristocetin>60% of plts are giantPlt...
39
What is the prognosis and treatment of Bernard-Souliers?
 
not fatal and supportive
40
What type of disorder is Glanzmann's Thrombasthenia?
 
Primary plt aggregationplts can't aggregate due to deficient GP IIb-IIIa
41
What are the symptoms of Glanzmann's?
 
in infancy - epistaxis, gum bleeding, mod superficial bleeding tendency
42
What are the lab results for Glanzmann's?
 
No platlet aggregation to ADP, collagen or epiPlt ct and morph normalBT is increased
43
What is the prognosis and treatment of Glanzmann's?
 
rarely fatal and supportive
44
What is another name for Chediak-Higash and Wiscott-Aldrich?
 
Storage pool disease
45
what is the cause of chediak-higashi and WAS?
 
plt secretion disorder - defective secretion due to a deficiency in the platelet dense granules
46
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...
47
What are the symptoms of chediak higashi and WAS?
 
mild to moderate bleeding, 
48
What is the prognosis and treatment for Chediak higashi and WAS?
 
not usually fatal and supportive treatment
49
What is uremia?
 
Waste products in blood interfere with platelet function
50
How does aspirin affect platelets?
 
inhibits prostaglandin synthesis, without prostaglandin synthesis there is no thromboxane A2,...
51
How does Ibuprofen effect platelets?
 
It temporarily interferes with plt aggregation. When drug clears from blood plt function returns
52
How does alcohol affect plts?
 
prolonged exposure can cause dysfunction
53
How does a cardiac by-pass machine affect plts?
 
It renders them functionless for up to 8 hrs post-op.
54
How is vW disease inherited?
 
autosomal dominant or recessivehomo is more severemore severe in children
55
What is the physiology behind vW disease?
 
deficient vWF causes instability of the Factor VIII molecule and renders it functionless in...
56
What are the symptoms of vW Disease?
 
excessive nosebleeds, bleeding gums, menorrhagia, excessive bleeding after toothe extraction...
57
what are the lab results for vW disease?
 
BT = incePT = normalPlt ct = normalPTT = incRistocetin cofactor = abnormalFactor VIII antigen...
58
what is the treatment for vW disease?
 
cryoppt, DDAVP -stim release of vWF from epith. cells, FFPtreated symptomatically
59
How many types of vW disease are there?
 
type I, Type II, and Type III
60
how is Factor VIII deficiency inherited?
 
homozygously= more severehetero = no symptomssex-linkedcauses 80% of bleeding disorders
61
What are the symptoms of Factor VIII def.?
 
hemarthroses, hematomas with sl. trauma, intramuscular, hemorrhages, delayed bleeding, problems...
62
what are the lab results for Factor VIII def?
 
PT=normalPTT=incplt ct = normalBT = normalMixing studies corrected with adsorbed plasmaFactor...
63
What is the treatment for Factor VIII disease?
 
cryoppt, Factor VIII conc, DDAVP for those that can produce some factor VIII, recombinant factor...
64
What is the inheritance of Hemophilia B?
 
homo=symptomaticsex-linkedhetero = no symptoms10-15% of hemophilias
65
What are the symproms of hemophilia B?
 
hemarthroses, hematomas, with slight trauma, intramuscular hemorrhages, delayed bleeding, problems...
66
What are the lab results for hemophilia B?
 
PT=normalPTT= incBT = normalplt ct = normalFactor IX assay = decMixing studies corrected with...
67
What is the treatment for hemophilia B?
 
FFP, Prothrombin conc, recombinant factor IX
68
How is afibrinogenemia inherited?
 
autosomal recessive
69
what are the symptoms of afibrinogenemia? 
 
spontaneous hemorrhage in GI, mucosal, joints, bruises and umbilical cord
70
What are the lab results for afibrinogenemia?
 
PT = incPTT= incethrombin time = incReptilase time = incFibrinogen assay = < 10 mg/dl
71
What is the treatment for afibrinogenemia?
 
medical management by giving cryoppt and ffp
72
What is the inheritance of hypofibrinogenemia?
 
heterozygous form of afibrinogenemia
can also be acquired during liver disease, renal disease,...
73
What are teh symptoms of hypofibrinogenemia?
 
mild, spontaneous bleeding severe, post-op bleeding
74
what are the lab results for hypofibrinogenemia?
 
PT= incPTT= inceFibrinogen assay = 20-100 mg/dl
75
What is the treatment for hypofibrinogenemia?
 
cryoppt
76
What is the inheritance of homozygous dysfibrinogenemia?
 
autosomal dominant - homo
77
What are the symptoms of homozygous dysfibrinogenemia?
 
spontaneous hemorrhage in GI, mucosal, joints, bruise, umbilical cord
78
What are the lab results of homozygous dysfibrinogenemia?
 
PT = incPTT = incTT =incReptilase time = incFibrinogen assay = normal
79
what is the treatment for homozygous dysfibrinogenemia?
 
cryoppt
80
What is the inheritance for hetero. dysfibrinogenemia?
 
autosomal dominant - hetero
81
What are the symptoms of hetero. dysfibrinogenemia?
 
mild, spontaneous bleeds, severe post-op bleeds
82
What are the lab results for hetero. dysfibrinogenemia?
 
fibrinogen level = normalPT=incPTT=incThrombin Time=increptilase time = inc
83
What is the inheritance of Factor V def.?
 
autosomal recessivehomo = symptomshetero = silent
84
What are the symptoms of Factor V def?
 
mild bleeding, mucosal bleeding, echymoses, 
85
what are the lab results for factor V def?
 
PT = inc, PTT=incBT=incmixing studies=corrected with absorbed plasmaFactor V assay = dec
86
What is the treatment for Factor V def?
 
FFPavoid aspirin
87
What is the inheritance of Factor XIII def?
 
autosomal recessive
88
What are the symptoms of FactorXIII def?
 
severe bleeding, spont. or delayed bleeds, delayed umbilical stump bleed, abnormal scar formation
89
What are the lab results for Factor XIII def?
 
PT=normalPTT = normalUrea solubility test is used to confirm the diagnosis
90
What is the treatment for Factor XIII def?
 
FFP or cryoavoid aspirin
91
What is the inheritance of Factor II def?
 
autosomal recessive
92
What are the symptoms of factor II def?
 
severe bleeding after trauma, skin and mucosal bleedinghetero = bleed with surgery
93
What are the lab tests for factor II def?
 
PT =incPTT= incBT=normalTT=normalFactor II assay=decMixing studies corrected with absorbed...
94
What is the treatment for Factor II def?
 
Prothrombin conc, or FFP
95
How is Factor VII def inherited?
 
autosomal recessiverare
96
what are the symptoms for Factor VII def?
 
severe bleeding, skin, mucosal and joint bleeding
97
what are the lab results for Factor VII def?
 
PT=incPTT=normalMixing studies corrected with serum and aged plasmaFactor VII=dec
98
What are the treatments for Factor VII def?
 
Prothrombin conc, FFPdon't use coumadin
99
What is the inheritance of Factor X def?
 
autosomal recessivevery rare
100
What are the symptoms of factor X def?
 
mild to sever bleeding, skin and mucosal bleeding
101
What are the test results for factor X def ?
 
PT=incPTT=incRussel Viper Venom Time = incMixing studies = corrects with serum and aged plasmaFactor...
102
What is the treatment for Factor X def?
 
prothrombin complex conc, FFP
103
How is factor XI def inherited?
 
autosomal recessivecommon in russians and asheninzic Jews
104
what are the symptoms or Factor XI def?
 
mild bleeding with trauma or surgery, epistaxis, hematuria, menorrhagia
105
What are the lab results with Factor XI def?
 
PT=normalPTT=incFactor XI assay=decMixing studies correct with serum, adsorbed and aged plasma
106
What is the treatment for Factor XI def?
 
FFPusually not severe enough to treat
107
What is the inheritance of Factor XII def?
 
Autosomal recessive
108
What are the symptoms of Factor XII def?
 
no bleeding embolic and thrombolic
109
what are the lab results for Factor XII def?
 
PT=normalPTT=abnormalMixing studies corrected by all
110
What is the treatment for Factor XII def?
 
anticoagulant
111
What is the inheritance of HMWK def?
 
autosomal recessive
112
What are the symptoms of HMWK def?
 
tendency to clot
113
what are the lab results for HMWK def?
 
PT=normalPTT=inc
Mixing study correct if incubate for 2hrs
114
What is the treatment for HMWK def?
 
no treatmentnot a major problem
115
what is the inheritance for PK def?
 
autosomal recessive
116
what are the symptoms of PK def?
 
tendency to clot
117
what are the test results of PK def?
 
PT=normalPTT=incFletcher Factor screen used to diagnose the diseaseextra long incub period...
118
what is the treatment for PK def?
 
anticoagulant therapy
119
What causes DIC?
 
tissue damage that releases excess tissue factor into the peripheral blood and activates the...
120
What happens in DIC?
 
Uncontrolled fibrin formation in blood vessels when there is no damage. depletes clotting factors...
121
What are the symptoms of DIC?
 
Uncontrolled bleeding 
122
What are the lab results for DIC
 
PT and PTT=incFibrinogen=decP;t ct=decDDimer=incschistocytes in peripheral blood
123
What is the prognosis and treatment for DIC?
 
acute is usually fatalgive heparin to encourage inactivation of thrombincan try antithrombin...
124
Why does liver disease cause severe coag problems?
 
clotting factors not produced in adequate quantities, diseased liver macrophages don't remove...
125
What are the lab results with liver disease?
 
PT and PTT=incFibrinogent=decplt ct=normal to decFDP and D-diner=incliver studies=abnormal
126
What is the treatment of liver disease?
 
replacement products as needed
127
What is Vitamin K used for in coag?
 
to make Factors II, VII, IX and X, and Protein C and PRotein S
128
what are the lab results of vitamin K def.?
 
PT and PTT = inctreat with vitamin K
129
What is lupus anticoagulant?
 
ani-plt antibody that develops due to lupus, inc incidence of DVT, and miscarriage
130
what are the lab results with a lupus anticoag?
 
PTT=incMixing studies show presence of inhibitorconfirm with anti-phospholipid testing
131
what is treatment for lupus anticoagulant?
 
aspirin
132
What is an anticardiolipin antibody?
 
anti-plt antibody, implicated in inc risk of thrombosis
133
what are the lab results of an anticardiolipin antibody?
 
tested for by reference facilities
134
What are the Factor VIII and Factor IX inhibitors?
 
single factor inhibitors assoc. with respective hemophilias, VIII may develop in person without...
135
What are the lab findings of Factor VIII and IX inhibitors?
 
same as hemophilias
136
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...
137
What drugs can be given to treat thrombosis?
 
heparin, oral, antiplatelet drugs, thrombolytic drugs
138
what is heparin's mode of action?
 
complexes with ATIII which greatly enhances its affinity for thrombin. after the ATIII complexes...
139
how is heparin monitored?
 
PTT
140
What is the antidote of heparin?
 
protamine sulfate
141
what are the potential problems with heparin?
 
overanticoagulation could result in bleed, HIT, pt must be hospitalized to get it and must...
142
what is the benefit of heparin?
 
action is immediate
143
What is coumadin's mode of action?
 
vitamin K antagonist. Binds vitamin K so less is available to the liver for synthesis of clotting...
144
How is coumadin monitored?
 
monthly PT
145
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
146
what is the benefit of coumadin?
 
can take at home
147
what is the antidote of coumadin?
 
vitamin K
148
What is the mode of action for thrombolytic drugs?
 
breaks up existing clot by activating plasminogen to plasmin.
149
What is thrombolytic drugs used for?
 
MI patients
150
What are the lab results for thrombolytic drugs?
 
must do baseline coag studies prior to administration
151
What are 3 types of thrombolytic agents?
 
urokinase, streptokinase, and TPA
152
What are 7 causes of hypercoagulability?
 
Antithrombin III defProtein C defProtein S defDysfibrinogenemialupus anticoagulantanticardiolipin...
153
what factors are in serum?
 
Factors VII, IX, X, XI, XII
154
What factors are in absorbed plasma?
 
Factors I, V, VIII, XI, XII
155
What factors are in aged plasma?
 
Factors I, II, VII, IX, X, XI, XII
156
What factor is fibrinogen?
 
I
157
What factor is prothrombin
 
II
158
What factor is Tissue thromboplastin
 
III
159
What factor is calcium
 
IV
160
What factor is labile
 
V
161
What factor is stable/
 
VII
162
What factor is antihemophilic factor
 
VIII
163
What factor is Christmas factor?
 
IX
164
what factor is Stuart Prower?
 
X
165
what factor is plasma thromboplastin?
 
XI
166
What factor is Hageman factor
 
XII
167
What factor is stabilizing factor?
 
XIII
168
what factor is fletcher factor?
 
Pre-Kallikrein
169
What factor is fitzgerald factor?
 
HMWK
170
what are the 6 coag inhibitors?
 
antithrombin III, Protein C, Protein S, alpha 2- macroglobulin, alpha 1-antitrypsin, C1-inactivator
171
What is the fibrinolytic agent?
 
plasmin
172
what are the 2 fibrinolysis inhibitors?
 
alpha 2-antiplasmin and alpha 2-macroglobulin
173
what factors are in the Intrinsic pathway?
 
VIII, IX, XI, XII, HMWK, Prekallekrein
174
what factors are in the extrinsic pathway?
 
III, VII
175
What factors are in the common pathway?
 
I, II, V, X
176
What factors are tested with PT?
 
I, II, V, VII, X
177
what factors are tested with PTT?
 
I, II, V, X, VIII, IX, XI, XII, HMWK, Prekallekrein
178
what factors are in the contact group?
 
Prekallekrein, HMWK, XI, XII
179
what factors are in the prothrombin group?
 
II, VII, IX, X
180
What factors are in the fibrinogen or consumable group?
 
I, V, VIII, XIII
181
what is primary hemostasis?
 
Formation of platelet plug through plt adhesion, aggregation, and secretion
182
what is secondary hemostasis?
 
stabilization of the plt plug by fibrin formation via coagulation factors and the coagulation...
183
What is fibrinolysis?
 
breakdown and rmoval of the clot after vascular repair
184
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...
185
Which tests have clots as endproducts?
 
PT, PTT, Plt function, platelet aggregation, DRVVT, 
186
Which tests are done using chromogenic method?
 
ATIII
187
What tests us immunoassay methods?
 
D-Dimer, factor assays
188
What is considered abnormal results for a platelet function test?
 
with collagen and epi = < 170 means good function          ...

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