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Coagulation, Urinalysis & body fluids > Disorders of Hemostasis > Flashcards

Disorders of Hemostasis Flashcards

1
Q

Infusion of platelets

A

used to treat bleeding caused by thrombocytopenia or dysfunctional platelets

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2
Q

Fresh Frozen plasma

A

provides replacement coagulation factors & contains all coag factors
used for patients w/ multiple factor deficiencies

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3
Q

Cryoprecipitate

A

concentrate of fibrinogen, vWF, factor VIII, factor XIII
used for the replacement of fibrinogen & factor XIII
historically used as treatment of von willebrand disease & FVIII deficiency
* blood borne viruses cannot be inactivated

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4
Q

Factor concentrates

A

from either human plasma or genetically engineered cell line

individual concentrates are not availalbe for all factors

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5
Q

Prothrombin complex concentrates (PCC)

A

made from human plasma & includes factors: II, VII, IX, X (vit K def)
suitable for individual deficiencies of factor II, IX, X

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6
Q

Desmopressin (DDAVP)

A

synthetic hormone used to promote the release of vWF in patients w/ von willebrand disease, mild hemophilia A (FVIII) & thrombocytopenia

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7
Q

Petechiae

A

pinpoint bruising, small red to purple spots in the skin

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8
Q

Ecchymoses

A

bruises larger than 3 mm red to purple when first formed & become yellowish green as they heal

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9
Q

Hematoma

A

bruise that occurs when blood leaks from an opening in a vessel & collects beneath intact skin, blue or purple & slightly raised

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10
Q

Acquired Disorders of the Vascular System

A

purpura due to decreased connective tissue: scurvy, excess glucocorticoids
purpura due to vasculitis: drugs/infection

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11
Q

Quantitative platelet disorders

A

thrombocytopenia: primary & secondary

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12
Q

Functional Platelet disorders

A
  1. bernard-soulier disease

2. Glanzmann’s thrombasthenia

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13
Q

Thrombocytopenia: Decreased platelet production

A 
bone marrow function is abnormal 
3 causes:
megakaryocyte hypoplasia (aquired)
ineffective thromopoiesis (inherited)
hereditary condition affects ability of bm to support megakaryocyte growth
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14
Q

May Hegglin anomaly

A

inherited cause of thrombocytopenia- lack of adequate bm megakaryocytes
large plts
dohle bodies present

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15
Q

Thrombocytopenia: accelerated or increased plt destruction

A

most common cause of thrombocytopenia

destruction can be immune (ITP, NAIT, HIT) mediated & nonimmune (TTP, DIC, HUS)

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16
Q

ITP (immune thrombocytopenic purpura)

A

common disorder causing severe thrombocytopenia

an autoimmune disorder in which autoreactive antibodies bind to platelets

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17
Q

Acute ITP

A

disease of children!
usually follows viral infection
spontaneous remission
lab <20x10^9/L

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18
Q

Chronic ITP

A 
disease of young adults
female > male
plt count: 30-80x109/L
mucosal bleeding 
duration of month or years
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19
Q

NAIT

A

immune plt destruction by alloantibodies that are stimulated by foreign antigen during pregnancy
similar to HDN except antibodies are directed towards plt antigens

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20
Q

Heparin Therapy thrombocytopenia

A

decrease in plt count is either:

  1. heparin associated thrombocytopenia (HAT)
  2. heparin induced thrombocytopenia (HIT)
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21
Q

Heparin associated thrombocytopenia (HAT)

A

non-immune, benign & limited thrombocytopenia

not associated w/ bleeding

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22
Q

Heparin-induced thrombocytopenia (HIT)

A

immune mediated adverse effect of heparin that increases risk of thrombosis
develop an IgG antibody to heparin-plt 4 complexes
associated w/ bleeding

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23
Q

Hemolytic-uremic syndrome (HUS)

A

more commonly found in young children
90% of cases caused by Shigella dysenteriae or E. coli
toxins from bacteria attach themselves to glomerulus cells, damages the cells, leads to formation of thrombi in renal vasculature

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24
Q

HUS lab results

A

hemolytic anemia!
schistocytes
renal failure
thrombocytopenia

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25
Thrombotic thrombocytopenic purpura (TTP)
rare but often fatal; women > men; 30-40 yrs 50% have a history of a virus prior to onset thrombotic lesions from arterioles & capillaries using up available plts results in thrombus in organs (!)
26
TTP lab results
neurological manifestation PT normal APTT normal fibrinogen, FDP, D-dimer normal
27
Disseminated intravascular coagulation (DIC)
body's blood clotting mechanism are activated throughout the body fibrin formation w/in the blood vessels leads to plt activation & consumption!
28
DIC lab results
Prolonged PT, APTT, TT increased FDPs & D-dimer decrease in fibrinogen
29
Primary thrombocytosisor Essential Thrombocytosis
neoplastic stem cell disorder causing dyregulated production of large #s of abnormal plts
30
Primary thrombocytosis or Essential Thrombocytosis lab values
increased plts abnormal plt shapes hemorrhages
31
Secondary thrombocytosis
caused by another disease or condition normal plt morphology elevated thrombocytosis
32
Bernard-Soulier Syndrome (BSS)
rare hereditary disorder of platelet adhesion 'giant plt syndrome' decrease in amount or abnormal function of the GPlb/IX complex prevents adhesion!
33
Bernard-Soulier disease lab results
normal or decreased plt count platelet aggregation test is abnormal with RISTOCETIN! (normal w/ others) giant plts
34
Glanzmann's thrombasthenia
rare hereditary disorder aggregation does not occur! def. of GPlb/IIIa complex - site of attachment of fibrinogen to plt surface plt aggregation test is abnormal w/ ADP, collagen & epinephrine!!
35
Signs/symptoms of coag pathway factor deficiency
excessive bleeding following trauma, uncontrolled menstrual bleeding, vomiting blood, nosebleeds, unexplained hematomas
36
Clotting factor disorders grouped by (3)
autosomal recessive disorders sex-linked disorders autosomal dominant disorders
37
Autosomal Recessive disorders
``` Factor I (fibrinogen) deficiency Factor II (prothrombin) deficiency Factor V (proaccelerin) deficiency Factor VII (proconvertin) deficiency Factor X (Stuart) deficiency Factor XI (plasma thromboplastin antecedent) deficiency Factor XII (hageman) deficiency Factor XIII (fibrin stabilizing) deficiency Prekallikrein (fletcher) deficiency HMWK (Fitzgerald) deficiency ```
38
Factor I (fibrinogen) deficiency
autosomal recessive | 2 forms: Afibrinogenemia & hypofibrinogenemia
39
Afibrinogenemia
no functionally detectable fibrinogen found more severe @ birth & can lead to death (bleeding from umbilical cord etc) increased PT, PTT, & TT decreased fibrinogen would be corrected w/ mixing study
40
Hypofibrinogenemia
few bleeding symptoms normal PT, APTT abnormal TT normal fibrinogen
41
Factor II (prothrombin) deficiency
``` mild hemorrhaging rarest bleeding disorder increased PT, PTT normal TT, BT 2 types: congenital & acquired is more common ```
42
Factor V (proaccelerin) deficiency
mild to moderate bleeding w/ bruising increased PT, PTT normal TT diagnosis made w/ factor V assay
43
Factor VII (proconvertin) deficiency
mild to moderate bleeding increased PT * only coag factor deficieny in which PT alone is prolonged!*** diagnosis made w/ factor VII assay
44
Factor X (stuart) deficiency
``` mild to severe bleeding increased PT, PTT normal TT diagnosis made w/ factor X assay *** may want to exclude vit K deficiency before diagnosis ```
45
Factor XI (plasma thromboplastin antecedent) deficiency
``` Hemophilia C mild bleeding after trauma normal PT, TT increased PTT! diagnosis made w/ factor XI assay 2nd most common bleeding disorder affecting females! ```
46
Factor XII (Hageman) deficiency
asymptomatic NO BLEEDING normal PT, TT increased PTT
47
Factor XIII (Fibrin stabilizing) deficiency
umbilical cord bleeding & delayed healing normal PT, PTT, TT ***lab diagnosis relies on dissolution of the fibrin clot in 1% monochloroacetic acid or 5 M urea***
48
Prekallikrein (fletcher) deficiency
asymptomatic! normal PT, TT increased PTT
49
HMWK (Fitzgerald) deficiency
asymptomatic! normal PT, TT Increased PTT
50
Sex-Linked Disorders
Factor VIII deficiency (hemophilia A) | Factor IX deficiency (hemophilia B)
51
Factor VIII deficiency
``` sex-linked hemophilia A effects males normal PT increased PTT musculoskeletal lesions, neurological deficiencies ```
52
Factor IX deficiency
sex linked hemophilia B | less common than hemophilia A but presents the same
53
Autosomal Dominant Disorders
von Willebrand Disease
54
Von Willebrand Disease
plts are intrinsically normal but exhibit abnormal adhesion bc of the absence or dysfunction of vWF & results in decreases in factor VIII & abnormal secondary hemostasis very variable 3 types
55
Type 1 vWF disease
autsomal dominant 70% of all cases quantitative decrease in vWF mild bleedign
56
Type 2 vWF disease
variable inheritance | abnormality in structure of vWF
57
type 3 vWF disease
rare autosomal recessive absent levels of vWF | severe bleeding - similar to hemophilia A
58
vWF disease screening test
1. bleeding time 2. platelet function analyzer (replacing BT) 3. APTT -typically increased 4. plt count -normal 5. PT- normal
59
vWF specific tests
1. measurements of plasma vWF antigen 2. factor VIII activity 3. assays of vWF plasma activity all are decreased!!
60
vWF disease treatment
cryoprecipitate or DDAVP
61
Acquired disorders of secondary hemostasis
DIC acquired pathologic inhibitors liver disease vit K deficiency
62
If every test (primary & secondary hemostasis) are abnormal what should you suspect first?
DIC
63
Pathological inhibitors
``` single coagulation factor inhibitor lupus anticoagulant (LA) ```
64
Inhibitors of single factors
usually seen in patients w/ inherited factor deficiencies neutralizing antibody directed against specific coag factors leading to a loss of activity common inhibitors are VIII & IX
65
Diagnosis of inhibitors
APTT markedly prolonged mixing study assays for specific inhibitors
66
Lupus Anticoagulant
nonspecific, usually does not cause bleeding problems associated w/ autoimmune diseases, neoplasms etc react w/ phospholipid surfaces of test reagents used in the APTT - prolongs test results

Coagulation, Urinalysis & body fluids (4 decks)

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