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Coagulation Flashcards

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1
Q

Pattern; impaired plt to plt

A

Glanzmann’s

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2
Q

Pattern: impaired GP11b/IIIa glycoprotein

A

Glanzmann

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3
Q

Pattern: impaired GP1b, V, IX

A

Bernard Soulier Syndrome

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4
Q

Pattern: impaired plt-vwf

A

Bernard Soulier syndrome

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5
Q

Pattern: mild to severe bleeding of mucosa, GI, epistaxis, normal PT/PTT, delayed bleeding time, normal plts and morphology

A

Glanzmann

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6
Q

Pattern: Prolonged bleeding time, severe mucosal bleeding, giant platelets

A

Bernard Soulier Syndrome

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7
Q

Rx for Glanzman and Bernard-Soulier syndrome

A

platelet transfusion, aminocaproic acid, rFVIIA

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8
Q

What questions should you ask if thrombocytopenia seen in neonate?

A

Ask maternal history, dysmorphic features such as skeletal defects, hemangiomas, IUGR, blueberry muffin

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9
Q

What are some acquired platelet causes?

A

Uremia that strips glycoproteins, cardiopulmonary bypass, ECMA, aspirin (irreversible), NSAIDS (transient), ethanol, clopidogrel, ticlopidine

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10
Q

Name four quantitative congenital platelet abnormalities leading to small platelets.

A
  1. Bone marrow failure, Fanconi anemia, pancytopenia
  2. TAR
  3. Congenital amegakaryocytic thrombocytopenia
  4. Wiskott-Aldrich
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11
Q

What is the difference in presentation of neonatal-allo-immune ITP and Maternal ITP?

A

Absent or present of maternal thrombocytopenia (chronic ITP, SLE history)

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12
Q

Rx for neonatal-allo-immune ITM versus maternal ITP?

A

1) Maternal platelets, PLA- platelets, IVIg

2) steroids and/or IVIg given to mother, prenatal or to baby post-natal

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13
Q

Besides ITP what other causes of thrombocytopenia in newborn

A

congenital marrow failure disorders, DIC, sepsis, viral infection (CMV, EBV, rubella)

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14
Q

Pattern: isolated thrombocytopenia, peak age 2-4 yrs, post-infectious, self-limited, petechiae, purpura, epitaxis, gingival bleeding

A

acute ITP

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15
Q

When does acute ITP become chronic

A

6 months

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16
Q

Pattern: mild to moderate bleeding, mucocutaneous bleeding, ecchymosis, menorrhagia, post-surgical bleeding (s/p tonsillectomy, dental extraction)

A

vWD

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17
Q

Name three types of vWD, inheritance pattern, and what the problem is

A

1) AD, not enough vWF, 80%
2) AD, doesn’t work, 20%
3) AR, absent, rare

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18
Q

Name causes of quantitative acquired platelet disorders

A
  1. Destruction
  2. ITP
  3. auto-immune disease (SLE, immunodeficiency, AIDS)
  4. Drug-induced (HIT, quinidine)
  5. Neonatal
  6. Hypersplenism
  7. IV trapping, DIC, Kasabach Merritt, HUS
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19
Q

Pattern: mucocutaneous bleeding and family history, PT nml, PTT nml to prolonged, bleeding time prolonged, vWF antigen absent to decreased, ristocetin cofactor assay decreased, factor VIII nml to decreased

A

vWD

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20
Q

Rx for vWD: mild to moderate and severe

A

mild to moderate: DDAVP for type 2, aminocapric acid inhibits fibrinolysis
severe: replacement with factor VIII, vWF concentrate or cryo fibrinogen, FVIII and vWF

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21
Q

What is the major side effect of DDAVP

A

hyponatremia

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22
Q

What are three drugs that interfere with Vit K

A

warfarin, INH, phenobarbital

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23
Q

Pattern: catastrophic bleeding at birth

A

maternal ingestion of Vit K antag

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24
Q

Pattern: purpura, oozing from umbilical cord, circumcision bleeding, GI, hematuria within 1 wk

A

failure to administer K at birth

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25
Pattern: purpura, oozing from umbilical cord, circumcision bleeding, GI, hematuria within 1 to 3 months
Vit K deficiency from breastfeeding
26
Hemophilia A from deficiency of which factor
VIII
27
Hemophila B from deficiency of which factor
IX
28
Pattern: PT normal, PTT prolonged, deep soft tissue/joint bleeds
hemophilia A or B
29
Pattern: myocardial infarction, stroke, DVT, miscarriages
hypercoagulable state
30
What labs to get for hypercoaguable workup
PT, PTT, INR, protein C/S, factor V leiden/activated protein C, MTHFR mutation, prothrombin mutation, DRVVT, ANTIII
31
Protein C is similar to which molecules
All serine protease, similar to Vit K dependent factors
32
Protein S function
cofactor for protein C
33
Pattern: neonatal pupura fulmins
homozygous protein c/s deficiency
34
Pattern: risk of VTE
heterozygous protein c/s deficiency
35
Pattern: warfarin skin necrosis
acquired protein c/s deficiency
36
Pattern: sudden, massive microthrombosis, skin, necrosis, DIC
neonatal purpura fulminans, think of homozygous protein c/s deficiency, consider sepsis
37
Rx for neonatal purpura fulminans
FFP, heparin, then long term warfarin
38
Long term complication of VitK deficiency
chornic hemarthrosis leading to joint destruction, infections comlications (HIV, hepatitis A, V, C, parvovirus), muscle hematomas causing contractures and atrophy
39
Pattern: heparin fails to anticoagulate
low ATIII
40
Pattern: recurrent severe VTE, fmhx thrombosis (PTT does change), DIC, liver dysfunction, nephrotic syndrome, IBD, L-asparaginase
ATIII deficiency
41
Pattern: late onset thrombosis
factor V Leiden
42
function of mutation in Factor V Leiden
prevents protein C from cleaving it
43
Pattern: VTE, hx of DVT, increased prothrombin concentration
prothrombin gene mutation
44
Pattern: increased risk of arterial and VTE, increased homocysteine which causes vascular damage
hyperhomocysteinemia
45
Pattern: increased PTT, doesn't cause bleeding instead recurrent VTE, stroke, recurrent pregnancy loss
antiphospholipid syndrome
46
What are some acquired thrombotic states?
nephrotic, IBD, malignancy, HIT
47
Pattern: relative or absolute thrombocytopenia, heparin exposure >5 days, serotonin release assay positive, heparin associated antibody positive
HIT
48
Pattern: similar to ITP. increasing pallor, normochromic normocytic anemia with low retic count, normal WBC and platelets, in todders with antecedent infection
transient erythroblastopenia of childhood
49
Pattern: infant, macrocytic anemia, short stature, upper limb abnormalities, non-megaloblastic anemia
Diamond Blackfan Anemia
50
Rx for Diamond Blackfan Anemia
steroids
51
Pattern: Child, has non-meg anemia, short stature, upper limb abnormalities, leukemia in 15%, progression to severe aplastic anemia
Fanconi's
52
Pattern: recurrent skin infection, photosensitivity, muscle weakness, ataxia, sensory loss, nystagmus
Chediak Higashi Syndrome
53
Giant neutrophil granules
Chediak Higashi Syndrome
54
Pattern: oculocutaneous albinism, defects in monocytes, lymphocytes, NK cells, CN and peripheral neuropathies
Chediak Higashi Sydrome
55
Pattern: early diagnosis, severe infection with Staph, E. coli, pseudomonas, diagnostic bone marrow shows developmental arrest, no mature neutrophils
Kostmann's
56
Pattern: 8 to 11 month old, increased minor but not serious infections, just neutropenia
nothing abnormal except neutropenia
57
Pattern: c. perfringens, recurrent episodes of fever, mouth ulcers and furunculosis, oscillation of ANC every 9 to 21 days
Cyclic neutropenia
58
How must you work up cyclic neutropenias?
CBCs 2x/week over at least 3 weeks
59
Folate or B12? | Hypersegmented polys
Both
60
Folate or B12? | Poor diet, preb, chronic hemolysis
Folate
61
Folate or B12? | Ileal resection
B12
62
Folate or B12? | Diphyllobothrium latum infection
B12
63
Folate or B12? | Breast fed/vegetarian
B12
64
Alpha or beta thal? | Blacks, SE Asian
alpha
65
Alpha or beta thal? | Mediterranean
beta
66
Alpha or beta thal? | onset newborn
alpha
67
Alpha or beta thal? | after 6 months
beta
68
Alpha or beta thal? | jaundice
alpha
69
Alpha or beta thal? | hepatosplenomegaly
beta
70
Alpha or beta thal? | HbH, HbBarts
alpha
71
Alpha or beta thal? | HbA2 or HbF
beta
72
Alpha or beta thal? | Hydrops fetalis
alpha
73
Alpha or beta thal? | Hair on ends on Xray
beta
74
Alpha or beta thal? | Milder form characteried more by ineffective erthyropoiesis than hemolytic anemia
beta
75
Alpha or beta thal? | Severity is proportional to dose of defective gene
beta
76
Alpha or beta thal? | HbH is present
alpha
77
Alpha or beta thal? | Elevations in HbA2 and HbF
beta
78
Alpha or beta thal? | Errors in globin
beta
79
Absorption site Fe
duodenum
80
Absorption site B12
terminal ileum
81
Absorption site folate
proximal jejunum
82
Iron deficiency or lead toxicity | Hypochromic/microcytic anemia
both
83
Iron deficiency or lead toxicity | Acute encephalopathy
lead
84
Iron deficiency or lead toxicity | elevated Free Erythrocyte Protoporphyrin (FEP)
both
85
Iron deficiency or lead toxicity | common in children of low income families
both
86
Extravascular or intravascular hemolysis? | IgG mediated
extra
87
Extravascular or intravascular hemolysis? | IgM mediated
intra
88
Extravascular or intravascular hemolysis? | macrophages digest
extra
89
Extravascular or intravascular hemolysis? | complement lysis
intra
90
Extravascular or intravascular hemolysis? | increased indirect bili
extra
91
Extravascular or intravascular hemolysis? | spherocytosis
extra
92
Extravascular or intravascular hemolysis? | hemoglobinuria and emia
intra
93
Extravascular or intravascular hemolysis? | schistocytes
intra
94
Extravascular or intravascular hemolysis? | Hereditary spherocytosis
extra
95
Extravascular or intravascular hemolysis? | G6PD, HUS, Kassabach-Merritt
intra
96
Diamond blackfan anemia or Fanconi anemia? | infants
DBA
97
Diamond blackfan anemia or Fanconi anemia? | Children
Fanconi
98
Diamond blackfan anemia or Fanconi anemia? | short stature, tri-phalangeal thumbs, renal cardiac
DBA
99
Diamond blackfan anemia or Fanconi anemia? | short stature, upper limb abnormality, pigmentation changes
FA
100
Diamond blackfan anemia or Fanconi anemia? | Increased HbF
both
101
Diamond blackfan anemia or Fanconi anemia? | rarely see leukemia
DBA
102
Diamond blackfan anemia or Fanconi anemia? | SAA, leukemia
FA
103
Diamond blackfan anemia or Fanconi anemia? | Steroid and BMT
both

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