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
Q

Thrombotic thrombocytopenic purpura (TTP)

A

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 (!)

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26
Q

TTP lab results

A

neurological manifestation
PT normal
APTT normal
fibrinogen, FDP, D-dimer normal

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27
Q

Disseminated intravascular coagulation (DIC)

A

body’s blood clotting mechanism are activated throughout the body
fibrin formation w/in the blood vessels leads to plt activation & consumption!

28
Q

DIC lab results

A

Prolonged PT, APTT, TT
increased FDPs & D-dimer
decrease in fibrinogen

29
Q

Primary thrombocytosisor Essential Thrombocytosis

A

neoplastic stem cell disorder causing dyregulated production of large #s of abnormal plts

30
Q

Primary thrombocytosis or Essential Thrombocytosis lab values

A

increased plts
abnormal plt shapes
hemorrhages

31
Q

Secondary thrombocytosis

A

caused by another disease or condition
normal plt morphology
elevated thrombocytosis

32
Q

Bernard-Soulier Syndrome (BSS)

A

rare hereditary disorder of platelet adhesion
‘giant plt syndrome’
decrease in amount or abnormal function of the GPlb/IX complex
prevents adhesion!

33
Q

Bernard-Soulier disease lab results

A

normal or decreased plt count
platelet aggregation test is abnormal with RISTOCETIN! (normal w/ others)
giant plts

34
Q

Glanzmann’s thrombasthenia

A

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
Q

Signs/symptoms of coag pathway factor deficiency

A

excessive bleeding following trauma, uncontrolled menstrual bleeding, vomiting blood, nosebleeds, unexplained hematomas

36
Q

Clotting factor disorders grouped by (3)

A

autosomal recessive disorders
sex-linked disorders
autosomal dominant disorders

37
Q

Autosomal Recessive disorders

A
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
Q

Factor I (fibrinogen) deficiency

A

autosomal recessive

2 forms: Afibrinogenemia & hypofibrinogenemia

39
Q

Afibrinogenemia

A

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
Q

Hypofibrinogenemia

A

few bleeding symptoms
normal PT, APTT
abnormal TT
normal fibrinogen

41
Q

Factor II (prothrombin) deficiency

A
mild hemorrhaging
rarest bleeding disorder
increased PT, PTT
normal TT, BT
2 types: congenital & acquired is more common
42
Q

Factor V (proaccelerin) deficiency

A

mild to moderate bleeding w/ bruising
increased PT, PTT
normal TT
diagnosis made w/ factor V assay

43
Q

Factor VII (proconvertin) deficiency

A

mild to moderate bleeding
increased PT * only coag factor deficieny in which PT alone is prolonged!***
diagnosis made w/ factor VII assay

44
Q

Factor X (stuart) deficiency

A
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
Q

Factor XI (plasma thromboplastin antecedent) deficiency

A
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
Q

Factor XII (Hageman) deficiency

A

asymptomatic NO BLEEDING
normal PT, TT
increased PTT

47
Q

Factor XIII (Fibrin stabilizing) deficiency

A

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
Q

Prekallikrein (fletcher) deficiency

A

asymptomatic!
normal PT, TT
increased PTT

49
Q

HMWK (Fitzgerald) deficiency

A

asymptomatic!
normal PT, TT
Increased PTT

50
Q

Sex-Linked Disorders

A

Factor VIII deficiency (hemophilia A)

Factor IX deficiency (hemophilia B)

51
Q

Factor VIII deficiency

A
sex-linked hemophilia A
effects males
normal PT
increased PTT
musculoskeletal lesions, neurological deficiencies
52
Q

Factor IX deficiency

A

sex linked hemophilia B

less common than hemophilia A but presents the same

53
Q

Autosomal Dominant Disorders

A

von Willebrand Disease

54
Q

Von Willebrand Disease

A

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
Q

Type 1 vWF disease

A

autsomal dominant
70% of all cases
quantitative decrease in vWF
mild bleedign

56
Q

Type 2 vWF disease

A

variable inheritance

abnormality in structure of vWF

57
Q

type 3 vWF disease

A

rare autosomal recessive absent levels of vWF

severe bleeding - similar to hemophilia A

58
Q

vWF disease screening test

A
  1. bleeding time
  2. platelet function analyzer (replacing BT)
  3. APTT -typically increased
  4. plt count -normal
  5. PT- normal
59
Q

vWF specific tests

A
  1. measurements of plasma vWF antigen
  2. factor VIII activity
  3. assays of vWF plasma activity
    all are decreased!!
60
Q

vWF disease treatment

A

cryoprecipitate or DDAVP

61
Q

Acquired disorders of secondary hemostasis

A

DIC
acquired pathologic inhibitors
liver disease
vit K deficiency

62
Q

If every test (primary & secondary hemostasis) are abnormal what should you suspect first?

A

DIC

63
Q

Pathological inhibitors

A
single coagulation factor inhibitor
lupus anticoagulant (LA)
64
Q

Inhibitors of single factors

A

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
Q

Diagnosis of inhibitors

A

APTT markedly prolonged
mixing study
assays for specific inhibitors

66
Q

Lupus Anticoagulant

A

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