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clin med > blood > Flashcards

blood Flashcards

1
Q

thalassemia

A

AR

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2
Q

G6PD

A

XR

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3
Q

SSA

A

AR

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4
Q

hemophilia A and B

A

XR

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5
Q

hemophilia C

A

AR

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6
Q

von willebrand disease

A

AD

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7
Q

factor 5 leiden

A

AD

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8
Q

factor 2 mutation

A

AD

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9
Q

protein C and S deficiency

A

AD

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10
Q

American black men

A

G6PD

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11
Q

High reticulocyte count
High LDH
Bite cells, blister cells, heinz bodies

A

G6PD

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12
Q

Healthy without chronic hemolytic anemia or splenomegaly

A

G6PD

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13
Q

Avoid oxidant drugs: nitrofurantoin, bactrim, quinolones, chloroquine, quinine

A

G6PD

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14
Q

Episodic hemolytic anemia under oxidative stress

A

G6PD

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15
Q

Pain, jaundice, gallstones, decreased oxygen saturation, chronically ill, early splenomegaly then hyposplenism
Vascular occlusion, stroke, retinopathy, neurocognitive dec, damage to all organs especially the lungs

A

SSD

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16
Q

Onset during 1st year of life

A

SSD

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17
Q

Acute pain crisis from vascular occlusive cluster of sickle cells usually in back & long bones that last about 2-7 days

A

SSD

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18
Q

Acute chest syndrome- leading cause of death; pulmonary infiltrates on chest x ray, distinguish from pneumonia

A

SSD

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19
Q

Multisystem organ failure

A

SSD

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20
Q 
High wbc
Reticulocytosis/high reticulocytes
Sickled on smear
Nucleated RBC
Howell jolly bodies
Target cells
High LDH
Confirm with hemoglobin electrophoresis
A

SSD

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21
Q

Hydroxyurea- reduces pain crisis
Folic acid- 1 mg/day
L glutamine- (voxelotor and crizanlizumab) meds to reduce pain crisi

A

SSD

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22
Q

BM failure

A

aplastic anemia

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23
Q 
Causes:
 autoimmune suppression MC is idiopathic
Stem cell injury: radiation, chemo, toxins, drugs
SLE
Post viral
A

aplastic anemia

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24
Q

Labs:
Pancytopenia (low RMC, WBC, platelets)
NO abnormal cells in blood or BM
Low reticulocytes- bc BM isn’t doing its job

A

aplastic anemia

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25
Q

Dx:

must get BM biopsy and see hypocellular bone marrow

A

aplastic anemia

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26
Q

Increase in RBC, WBC, and platelets

Risk of thrombosis

A

polycythemia vera

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27
Q

Men after age 60

A

polycythemia vera

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28
Q

Splenomegaly, HA, dizziness, tinnitus, blurred vision, fatigue/malaise, pruritus, epistaxis

A

polycythemia vera

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29
Q 
Lab:
Men Hct>54%
Women Hct>51%
Normal RBC morphology
High WBC and platelets
A

polycythemia vera

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30
Q

Survival 10-16 yrs

AML in 10-15%

A

polycythemia vera

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31
Q 
Trmt:
Rehydrate if relative
Phlebotomy to remove blood
Daily asa to prevent thrombosis
Myelosuppressive agents such as hydroxyurea and busulfan to reduce pain
A

polycythemia vera

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32
Q

MC severe bleeding disorder

2nd MC congenital bleeding disorder

A

hemophilia A

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33
Q

Signs and sx:
Hemarthrosis, IM bleeding, GI bleeding, bleeding from mild trauma such as dental exams
First events in early childhood

A

hemophilia A or B

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34
Q 
Labs:
High PTT 
Normal PT 
Normal platelets
Factor VIII or IX decreased on assay
A

hemophilia A or B

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35
Q

Trmt:
Factor VIII or IX concentrate
Prophylactic (preventative) prior to dental/surgical procedures
Avoid trauma/contact sports

A

hemophilia A or B

36
Q

Asymptomatic

A

hemophilia C and von willebrand disease

37
Q

Labs:
High PTT
Normal PT
Factor XI decreased or absent on assay

A

hemophilia C

38
Q

Trmt:
Fresh frozen plasma prior to surgery
Factor XI concentrate

A

hemophilia C

39
Q

Deficient or defective vWF (glycoprotein)

A

Von Willebrand disease

40
Q

MC inherited bleeding disorder

A

Von Willebrand disease

41
Q

Affects 1% of the population, but only 1% of those ppl are symptomatic

A

Von Willebrand disease

42
Q

vWF binds to surfaces and prolongs the half life of factor VIII

A

Von Willebrand disease

43
Q

Dx:
FH and personal Hx of bleeding
No spontaneous hemarthrosis
Petechiae, easy bruising, inc mucosal and menstrual bleeding

A

Von Willebrand disease

44
Q

3 types: type 1 is the most common and least severe

A

Von Willebrand disease

45
Q

Labs:
PTT and PT normal
Platelet count normal or low
vWF low?

A

Von Willebrand disease

46
Q

MC inherited thrombophilia- 5% caucasians and 1% AAM

A

Factor V Leiden mutation

47
Q

Consider in someone with recurrent thromboembolic events

A

Factor V Leiden mutation

48
Q

Management:
Prophylactic anticoagulation NOT recommended unless they have had a thromboembolic event
Anticoagulation if pregnant or post op
Avoid smoking and OCP

A

Factor V Leiden mutation

49
Q

2nd MC inherited thrombophilia after factor V leiden

A

Factor II mutation

50
Q

Increases VTE by 3-4 times

A

Factor II mutation

51
Q

Can be acquired- sepsis

A

Protein C and S deficiency

52
Q

Risk for thromboembolic event

A

factor 5 leiden mutation, factor 2 mutation, Protein C and S deficiency, antiphospholipid Ab syndrome

all of hypercoagucble states/thrombophilias

53
Q

MC acquired thrombophilia

A

Antiphospholipid antibody syndrome

54
Q

Arterial or venous clots

A

Antiphospholipid antibody syndrome

55
Q

May present as recurrent pregnancy loss

A

Antiphospholipid antibody syndrome

56
Q

Dx: 1 or more of the following anti-phospholipid (aPL) antibodies:
Lupus anticoagulant
Anticardiolipin antibody ELISA
Anti B2 glycoprotein I ELISA

A

Antiphospholipid antibody syndrome

57
Q 
Screen for this if:
SLE
Thromboembolic event in SLE pt
First thromboembolic event in young pt
Women 3+ spontaneous abortions
A

Antiphospholipid antibody syndrome

58
Q

Platelet redistribution/hypersplenism-

A

platelets stuck in spleen

59
Q

MC platelet disorder is from

A

myelosuppression from an acute illness

60
Q

Increased platelet destruction from platelet autoantibodies

A

Immune thrombocytopenia ITP

61
Q 
Exam:
Low platelets <30,000
Mucocutaneous bleeding
Kids- petechiae and purpura
Petechiae and purpura progress to major hemorrhage
Hemorrhagia (heavy menses)
Petechiae: 1-3 mm
Purpura: over 3mm
A

Immune thrombocytopenia ITP

62
Q 
Dx of exclusion 
No other abnormal cell lines
R/O HIV and hep B/C
ONLY thrombocytopenia/low platelets
Normal WBC, RBC, and reticulocytes
Normal PT and PTT
May need BM biopsy in some cases
A

Immune thrombocytopenia ITP

63
Q

Kids: 60% follows viral illness and resolves within months

A

Immune thrombocytopenia ITP

64
Q

Adults: chronic, F:M 3:2

A

Immune thrombocytopenia ITP

65
Q

Trmt: only if significant bleeding or platelet count is less than 30,000

A

Immune thrombocytopenia ITP

66
Q

Thrombotic: Platelets aggregate and form microthrombi. These occlude arterioles and capillaries
Thrombocytopenic: no platelets bc they are all used from the clots

A

Thrombotic Thrombocytopenic purpura (TTP)

67
Q

Exam:
Flu like sx, fever, fatigue
Pallor, jaundice, purpura
Neuro involvement: change in mental status, focal deficits, seizures
Kidney failure less severe compared to HUS
Acute multisystem organ failure- bc of clots occurring everywher

A

Thrombotic Thrombocytopenic purpura (TTP)

68
Q 
Lab:
Thrombocytopenia (low platelets)
Anemia
Schistocytes and helmet cells
High reticulocytes
High indirect bilirubin
High LDH
Low haptoglobin
High BUN/Cr
PT/PTT normal- diff from DIC
Inhibitory antibodies ADMTS-13
A

Thrombotic Thrombocytopenic purpura (TTP)

69
Q 
Trmt:
Plasma exchanges
Corticosteroids
Splenectomy
95% fatal if untreated
A

Thrombotic Thrombocytopenic purpura (TTP)

70
Q

Atypical and endemic form

A

Hemolytic uremic syndrome (HUS)

71
Q

MC in children- the endemic type follows diarrhea from e coli and shiga toxin)

A

Hemolytic uremic syndrome (HUS)

72
Q

Atypical- deficiency in regulatory proteins

A

Hemolytic uremic syndrome (HUS)

73
Q

Renal failure is MC and more severe than TTP

Neurological defects are less common than TTP

A

Hemolytic uremic syndrome (HUS)

74
Q

Lab:
Hemolytic anemia
Thrombocytopenia/low platelets- not as low as TTP
If endemic type: + stool culture for E coli or shigella toxin

A

Hemolytic uremic syndrome (HUS)

75
Q 
Over 50% platelet reduction 7-10 days after heparin exposure
IgG antibodies are formed
Thrombosis
NO Bleeding 
Limb ischemia
A

Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome

76
Q

Dx:
Thrombocytopenia 5-10 days after heparin exposure (usually hospitalized pts) or sooner if exposed to heparin in past 30 days then re exposed

A

Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome

77
Q

Labs:
Thrombocytopenia/ low platelets
+ platelet factor 4 (PF4) antibody
Serotonin release assay (SRA) after 1 wk confirms results

A

Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome

78
Q

Trmt:
Begin trmt when suspected
Stop heparin
anticoagulant/thrombin inhibitor until platelets recover
Warfarin * 30 days after platelets recovered
Check for DVT/thromboembolism

A

Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome

79
Q

Labs:
Thrombocytopenia/ low platelets
+ platelet factor 4 (PF4) antibody
Serotonin release assay (SRA) after 1 wk confirms results

A

Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome

80
Q

Uncontrolled activation of coagulation so depletion in coagulation factors, fibrinogen, and platelets occurs
Bleeding and clotting occur

A

Disseminated intravascular coagulation (DIC)

81
Q

Causes:

Sepsis, cancer, trauma, burns, pregnancy, snake bites, aortic aneurysm, incompatible blood transfusion

A

Disseminated intravascular coagulation (DIC)

82
Q

Dx:
No one lab finding
Widespread bleeding at many sites
Trousseau syndrome- malignancy related DIC primarily thrombotic

A

Disseminated intravascular coagulation (DIC)

83
Q 
Lab:
High aPTT and PT
Low fibrinogen
High D dimer
Schistocytes/helmet cells
thrombocytopenia/ dec platelets
A

Disseminated intravascular coagulation (DIC)

84
Q

Trmt:
Treat underlying disorder
ATB
FFP (fresh frozen plasma- contains all coag factors)- only if high aPTT, PT AND lots of bleeding

A

Disseminated intravascular coagulation (DIC)

85
Q

Form of DIC in pregnancy
Hemolysis/hemolytic anemia
Elevated liver enzymes
Low platelets

A

HELLP syndrome

clin med (35 decks)

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