blood Flashcards
1
Q
thalassemia
A
AR
2
Q
G6PD
A
XR
3
Q
SSA
A
AR
4
Q
hemophilia A and B
A
XR
5
Q
hemophilia C
A
AR
6
Q
von willebrand disease
A
AD
7
Q
factor 5 leiden
A
AD
8
Q
factor 2 mutation
A
AD
9
Q
protein C and S deficiency
A
AD
10
Q
American black men
A
G6PD
11
Q
High reticulocyte count
High LDH
Bite cells, blister cells, heinz bodies
A
G6PD
12
Q
Healthy without chronic hemolytic anemia or splenomegaly
A
G6PD
13
Q
Avoid oxidant drugs: nitrofurantoin, bactrim, quinolones, chloroquine, quinine
A
G6PD
14
Q
Episodic hemolytic anemia under oxidative stress
A
G6PD
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
16
Q
Onset during 1st year of life
A
SSD
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
18
Q
Acute chest syndrome- leading cause of death; pulmonary infiltrates on chest x ray, distinguish from pneumonia
A
SSD
19
Q
Multisystem organ failure
A
SSD
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
21
Q
Hydroxyurea- reduces pain crisis
Folic acid- 1 mg/day
L glutamine- (voxelotor and crizanlizumab) meds to reduce pain crisi
A
SSD
22
Q
BM failure
A
aplastic anemia
23
Q
Causes: autoimmune suppression MC is idiopathic Stem cell injury: radiation, chemo, toxins, drugs SLE Post viral
A
aplastic anemia
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
25
Dx:
| must get BM biopsy and see hypocellular bone marrow
aplastic anemia
26
Increase in RBC, WBC, and platelets
| Risk of thrombosis
polycythemia vera
27
Men after age 60
polycythemia vera
28
Splenomegaly, HA, dizziness, tinnitus, blurred vision, fatigue/malaise, pruritus, epistaxis
polycythemia vera
29
```
Lab:
Men Hct>54%
Women Hct>51%
Normal RBC morphology
High WBC and platelets
```
polycythemia vera
30
Survival 10-16 yrs
| AML in 10-15%
polycythemia vera
31
```
Trmt:
Rehydrate if relative
Phlebotomy to remove blood
Daily asa to prevent thrombosis
Myelosuppressive agents such as hydroxyurea and busulfan to reduce pain
```
polycythemia vera
32
MC severe bleeding disorder
| 2nd MC congenital bleeding disorder
hemophilia A
33
Signs and sx:
Hemarthrosis, IM bleeding, GI bleeding, bleeding from mild trauma such as dental exams
First events in early childhood
hemophilia A or B
34
```
Labs:
High PTT
Normal PT
Normal platelets
Factor VIII or IX decreased on assay
```
hemophilia A or B
35
Trmt:
Factor VIII or IX concentrate
Prophylactic (preventative) prior to dental/surgical procedures
Avoid trauma/contact sports
hemophilia A or B
36
Asymptomatic
hemophilia C and von willebrand disease
37
Labs:
High PTT
Normal PT
Factor XI decreased or absent on assay
hemophilia C
38
Trmt:
Fresh frozen plasma prior to surgery
Factor XI concentrate
hemophilia C
39
Deficient or defective vWF (glycoprotein)
Von Willebrand disease
40
MC inherited bleeding disorder
Von Willebrand disease
41
Affects 1% of the population, but only 1% of those ppl are symptomatic
Von Willebrand disease
42
vWF binds to surfaces and prolongs the half life of factor VIII
Von Willebrand disease
43
Dx:
FH and personal Hx of bleeding
No spontaneous hemarthrosis
Petechiae, easy bruising, inc mucosal and menstrual bleeding
Von Willebrand disease
44
3 types: type 1 is the most common and least severe
Von Willebrand disease
45
Labs:
PTT and PT normal
Platelet count normal or low
vWF low?
Von Willebrand disease
46
MC inherited thrombophilia- 5% caucasians and 1% AAM
Factor V Leiden mutation
47
Consider in someone with recurrent thromboembolic events
Factor V Leiden mutation
48
Management:
Prophylactic anticoagulation NOT recommended unless they have had a thromboembolic event
Anticoagulation if pregnant or post op
Avoid smoking and OCP
Factor V Leiden mutation
49
2nd MC inherited thrombophilia after factor V leiden
Factor II mutation
50
Increases VTE by 3-4 times
Factor II mutation
51
Can be acquired- sepsis
Protein C and S deficiency
52
Risk for thromboembolic event
factor 5 leiden mutation, factor 2 mutation, Protein C and S deficiency, antiphospholipid Ab syndrome
all of hypercoagucble states/thrombophilias
53
MC acquired thrombophilia
Antiphospholipid antibody syndrome
54
Arterial or venous clots
Antiphospholipid antibody syndrome
55
May present as recurrent pregnancy loss
Antiphospholipid antibody syndrome
56
Dx: 1 or more of the following anti-phospholipid (aPL) antibodies:
Lupus anticoagulant
Anticardiolipin antibody ELISA
Anti B2 glycoprotein I ELISA
Antiphospholipid antibody syndrome
57
```
Screen for this if:
SLE
Thromboembolic event in SLE pt
First thromboembolic event in young pt
Women 3+ spontaneous abortions
```
Antiphospholipid antibody syndrome
58
Platelet redistribution/hypersplenism-
platelets stuck in spleen
59
MC platelet disorder is from
myelosuppression from an acute illness
60
Increased platelet destruction from platelet autoantibodies
Immune thrombocytopenia ITP
61
```
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
```
Immune thrombocytopenia ITP
62
```
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
```
Immune thrombocytopenia ITP
63
Kids: 60% follows viral illness and resolves within months
Immune thrombocytopenia ITP
64
Adults: chronic, F:M 3:2
Immune thrombocytopenia ITP
65
Trmt: only if significant bleeding or platelet count is less than 30,000
Immune thrombocytopenia ITP
66
Thrombotic: Platelets aggregate and form microthrombi. These occlude arterioles and capillaries
Thrombocytopenic: no platelets bc they are all used from the clots
Thrombotic Thrombocytopenic purpura (TTP)
67
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
Thrombotic Thrombocytopenic purpura (TTP)
68
```
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
```
Thrombotic Thrombocytopenic purpura (TTP)
69
```
Trmt:
Plasma exchanges
Corticosteroids
Splenectomy
95% fatal if untreated
```
Thrombotic Thrombocytopenic purpura (TTP)
70
Atypical and endemic form
Hemolytic uremic syndrome (HUS)
71
MC in children- the endemic type follows diarrhea from e coli and shiga toxin)
Hemolytic uremic syndrome (HUS)
72
Atypical- deficiency in regulatory proteins
Hemolytic uremic syndrome (HUS)
73
Renal failure is MC and more severe than TTP
| Neurological defects are less common than TTP
Hemolytic uremic syndrome (HUS)
74
Lab:
Hemolytic anemia
Thrombocytopenia/low platelets- not as low as TTP
If endemic type: + stool culture for E coli or shigella toxin
Hemolytic uremic syndrome (HUS)
75
```
Over 50% platelet reduction 7-10 days after heparin exposure
IgG antibodies are formed
Thrombosis
NO Bleeding
Limb ischemia
```
Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome
76
Dx:
Thrombocytopenia 5-10 days after heparin exposure (usually hospitalized pts) or sooner if exposed to heparin in past 30 days then re exposed
Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome
77
Labs:
Thrombocytopenia/ low platelets
+ platelet factor 4 (PF4) antibody
Serotonin release assay (SRA) after 1 wk confirms results
Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome
78
Trmt:
Begin trmt when suspected
Stop heparin
anticoagulant/thrombin inhibitor until platelets recover
Warfarin * 30 days after platelets recovered
Check for DVT/thromboembolism
Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome
79
Labs:
Thrombocytopenia/ low platelets
+ platelet factor 4 (PF4) antibody
Serotonin release assay (SRA) after 1 wk confirms results
Heparin induced thrombocytopenia and thrombosis HIT(T) syndrome
80
Uncontrolled activation of coagulation so depletion in coagulation factors, fibrinogen, and platelets occurs
Bleeding and clotting occur
Disseminated intravascular coagulation (DIC)
81
Causes:
| Sepsis, cancer, trauma, burns, pregnancy, snake bites, aortic aneurysm, incompatible blood transfusion
Disseminated intravascular coagulation (DIC)
82
Dx:
No one lab finding
Widespread bleeding at many sites
Trousseau syndrome- malignancy related DIC primarily thrombotic
Disseminated intravascular coagulation (DIC)
83
```
Lab:
High aPTT and PT
Low fibrinogen
High D dimer
Schistocytes/helmet cells
thrombocytopenia/ dec platelets
```
Disseminated intravascular coagulation (DIC)
84
Trmt:
Treat underlying disorder
ATB
FFP (fresh frozen plasma- contains all coag factors)- only if high aPTT, PT AND lots of bleeding
Disseminated intravascular coagulation (DIC)
85
Form of DIC in pregnancy
Hemolysis/hemolytic anemia
Elevated liver enzymes
Low platelets
HELLP syndrome
