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CPD I > Hematology II > Flashcards

Hematology II Flashcards

1
Q

Normal platelet count:

A

140-440K/uL

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2
Q

Normal platelet lifespan:

A

7-10 days

destroyed by spleen

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3
Q

What might cause low platelets (thrombocytopenia):

A

sequestration, used up in clotting, destruction, failed production, dilution (pregnancy increased blood volume), drugs, liver dz, DIC from cancer or sepsis, HIV, ITP, quinine (tonic water)

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4
Q

If platelet labs don’t make sense clinically, you should:

A

retest! platelets are easier to mess up in the lab.

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5
Q

Initial finding in 10% of HIV diagnosis:

A

thrombocytopenia

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6
Q

Most common cause of platelet dysfunction:

A

aspirin, platelet aggregation inhibitor

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7
Q

Normal platelet function - 3 A’s:

A

Adherance
Activation
Aggregation

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8
Q

What dx do you suspect in pts with lifelong bleeding disorders but normal platelet counts and coag studies?

A

Hereditary intrinsic platelet disorders

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9
Q

Autoimmune disorder with increased platelet destruction:

A

Idiopathic thrombocytopenic purpura (ITP)

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10
Q

ITP tends to affect what age(s)?

A

Children and >60yrs

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11
Q

ITP etiology:

A

HIV! HEP C!

CMV, drugs, H. Pylori, thyroid disorder, B12/Folate deficiency, autoimmune - SLE

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12
Q

ITP in children presentation:

A

acute
self limiting
often triggered by viral illness

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13
Q

S/sx of ITP:

A 
may be asx
petechiae on [gravity] dependent areas (LE, feet, ankles)
mucosal bleeding
conjunctival hemorrhages
splenomegaly
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14
Q

Suspected Dx for unexplained low platelet count, otherwise normal CBC?

A

Idiopathic thrombocytopenia purpura

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15
Q

Compare vasculitic purpura with hemophilia:

A

In hemophilia, tend to get deep hematoma and ecchymosis.

ITP has petechiae, particularly in dependent areas

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16
Q

Thrombocytopenia secondary to splenomegaly seen in:

A

advanced cirrhosis
myelofibrosis
myeloid metaplasia

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17
Q

Deficiency in enzyme ADAMTS13 (can’t break down VWF, platelets destroyed by fibrin strands in small vessels):

A

Thrombotic thrombocytopenic purpura (TTP)

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18
Q

Etiology of TTP:

A

children: severe diarrhea with E. Coli 0157:H7
adults: many idiopathic! drug toxicity, pregnancy, autoimmune dz (SLE, scleroderma), AIDS, E. coli, Shiga toxin

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19
Q

Classic pentad of TTP:

[board exam question, all 5 seen in 5% of pts]

A 
Thrombocytopenia
red cell fragmentation
fever
transient neurologic deficits
kidney failure
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20
Q

S/sx of TTP:

A

fever

ischemia: confusion, coma, seizures, headaches, abd pain, arrhythmia, chest pain

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21
Q

Lab picture of TTP-HUS:

A

Normal PT, PTT, fibrinogen, D dimer, Coombs
Increase LDH, bilirubin, creatinine
Decreased/absent haptoglobin

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22
Q

TTP in children with E. Coli 0157:H7:

A

HUS - hemolytic uremic syndrome

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23
Q

Hereditary bleeding disorder resulting in impaired synthesis of VWF, prolonged bleeding time, factor 8 deficiency:

A

Von Willebrand’s Disease

24
Q

S/sx of VWD:

A

easy bruising, skin bleeding, prolonged bleeding from mucosal surfaces (oropharyngeal, GI, uterine), menorrhagia, prolonged bleeding after surgery

25
Q

VWD exam:

A

look for bruising
may be asx
ask about family hx

26
Q

Three tests recommended as VWD screening:

A

Plasma VWF antigen
Plasma VWF activity
Factor VIII activity

27
Q

Arrest of bleeding from an injured vessel:

A

hemostasis
requires vascular, platelet and plasma factors
abnormalities result in excessive bleeding or thrombosis

28
Q

T/F - PT and PTT are assessing platelets in the clotting cascade.

A

FALSE! these tests examine fibrin and thrombin, but tell you nothing about platelets.

29
Q

Why might thrombosis occur:

A 
Turbulence in a blood vessel
Venous stasis - prolonged immobility, bedrest
Clotting disorders - factor deficiencies
Trauma
Medication - oral contraceptives
Pregnancy
heart failure
renal dz
cardiac risk factors - smoking, obesity, air pollution, hyperhomocysteinemia
30
Q

What is Virchow’s triad:

A

vascular endothelial injury
alterations in blood flow (stasis)
alterations in blood constituents

31
Q

5 most common thrombophilia:

A 
prothrombin gene mutation
factor V leiden mutation
Protein S deficiency
Protein C deficiency
Antithrombin deficiency
32
Q

DVT &/or PE with no provocation. you suspect:

increased risk with:

A

Factor V Leiden

oral contraceptives, HRT, pregnancy

33
Q

Which protein inactivates coat factors Va and VIIIa?

A

Protein C

34
Q

Protein C synthesis in the liver is ______ dependent.

A

Vitamin K

35
Q

S/sx of protein C deficiency:

A

venous thromboembolism
neonatal purpura fulminans in homozygous or doubly heterozygous newborns
warfarin-induced skin necrosis

36
Q

Cofactor in the protein C system:

A

Protein S

37
Q

S/sx of protein S deficiency:

A

arterial thrombosis

and similar to protein C deficiency

38
Q

Antithrombin deficiency:

A

can be fatal to fetus, genetic deficiency,

39
Q

Hyperhomocysteinemia may be due to:

A

B12, folate, or B6 deficiency

think about MTHFR mutations

40
Q

Antiphospholipid antibody syndrom (lupus anticoag):

A 
predisposed arterial/venous thrombosis, stroke, DVT, PE
prolonged PTT (paradoxical)
41
Q

Widespread activation of the clotting system and consumption of all clotting factors resulting in bleeding out:

A

Disseminated intravascular coagulation (DIC)

42
Q

DIC etiology:

A

septicemia, crush injury, severe head injury, cancer, shock from any cause, heat stroke, burn, snake venom, pregnancy complications, amphetamine OD, ABO incompatibility, liver dz/failure

43
Q

S/sx of DIC:

A 
rapid or slow-evolving
persistent bleeding at puncture sites
ecchymosis at injection site
serious GI bleeding
intravascular hemolysis
44
Q

Dx for - central! acute renal/hepatic dysfunction, respiratory dysfunction, shock, thromboembolism, CNS involvement:, petechiae, ecchymoses, blood oozing from mucosa and wound sites (around IV), prolonged PT/PTT/TT, reduced factors

A

Rapid DIC - decompensated

45
Q

Dx for - peripheral petechiae, ecchymoses, venous thrombosis, pulmonary emboli, elevated D dimer and fibrin degradation products, normal PT/PTT/TT and factors.

A

Chronic DIC - compensated

46
Q

Inherited bleeding disorders with clotting factor deficiencies:

A

hemophilias

47
Q

Most common hemophilia:

A

Hemophilia A - 80%

factor VIII deficient

48
Q

S/sx of hemophilia:

A

increased bleeding after circumcision, intracranial hemorrhage in the perinatal period, serious hemorrhage from minor injury, excessive bruising, hematuria, hematoma, hemarthroses - almost pathognomic
Most commonly dx >1 yr with learning to walk (falls)

49
Q

Dx for hemophilia:

A

PT/PTT, platelet count, assays for factors VIII/IX
prolonged PTT
normal PT and platelets

50
Q

too much amyloid, laid down in vessels causing fragility:

A

amyloidosis

51
Q

small vessel vasculitis and purpura:

A

cryoglobulinemia

52
Q

too much IgG, usually related to immunologic disorder like SLE, vascular purpura, residual brown spots:

A

hypergammaglobulinemic purpura

53
Q

elevated plasma IgM, seen in Waldenstrom’s macroglobulinemia:

A

hyperviscosity syndrome

54
Q

arteriovenous malformations, multiple hemorrhagic lesions on face/lips/oral/nasal muscosas/fingers/toes:

A

Hereditary hemorrhagic telangiectasia (HHT)

55
Q

common easy bruising with normal labs, vascular fragility, without significant blood loss:

A

purpura simples

[responds well to flavonoids!]

56
Q

persistent dark ecchymoses in elderly patients, usu forearms and hands, without known trauma, resolve leaving brown spot

A

senile purpura

CPD I (2 decks)

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