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Royal College Studying > Hematology > Flashcards

Hematology Flashcards

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1
Q

G6PD medications to avoid

A

Medications: sulfonamides, cipro, moxifloxacin, norfloxacin, ofloxacin, dapsone, septra, nalidixic acid, chloramphenicol, nitrofurantoin, primaquine, pamaquine, chloroquine, quinacrine, B-naphthol, niridazole, acetanilide, vitamin K analogs, methylene blue, toluide blue, probenecid, dimercaprol, ASA, rasburicase, phenazopyridine, benzene, naphthalene, diabetic acidosis, hepatitis, sepsis

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2
Q

Inheritance G6PD

A

x-linked

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3
Q

Pathophys G6PD

A

G6PD catalyzes conversion of glucose 6 phosphate to 6-phosphogluconic acid which produces NADPH which maintains GSH which provides protection against oxidant threat from certain drug and infections that would otherwise cause precipitation of hemoglobin (Heinz bodies) or damage RBC membrane

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4
Q

ethnic groups for G6PD

A
  • 13% of males of African descent have G6PD
    Italians, Greeks, Mediterranean, middle eastern, African and Asian ethnic groups also have a high incidence ranging from 5-40%
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5
Q

heniz bodies

A

G6PD

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6
Q

Ethnicity fo hereditary aphserocytosis

A

Northern European

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7
Q

Proteins involved in hereditary spherocytosis

A

Abnormalities of ankyrin or spectrin are the most common molecular defects

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8
Q

Inheritance spherocytosis

A

AD

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9
Q

Labs in HS

A

MCV is normal or een slightly increased, increased MCHC, low RDW

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10
Q

Mgmt Hereditary spherocytosis

A
  • Should have vaccinations, spleen size monitored, screening for gallbladder disease at age 4 and repeated every 3-5 years
    • Folic acid supp if moderate or severe HS
      Splenectomy is curative
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11
Q

Pyruvate kinase deficiency inhertiance

A

AR

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12
Q

Hemophilia inhetiance

A

x-linked

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13
Q

Is PT abnormal inhemophilia

A

No

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14
Q

Earliest joint involved in hemophilia

A

ankle

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15
Q

What lab value is abnormla in hemophilia

A

PTT, usually 2-3 times uLN

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16
Q

Severe hemophilia how much factor

A

<1%

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17
Q

Fanconi anemia ethnicity

A

Higher in Ashkenazi jews and Afrikaners

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18
Q

Rare, multisystem hereditary disorder results in the development of bone marrow failure in those affected and predisposition to malignancy including myelodysplasia and AML, epithelial cancers

A

Fanconi anemia

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19
Q

Triad for fanconi anemia

A

TRIAD of bone marrow failure, congenital anomalies and elevated chromosome fragility

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20
Q

Congen abnormalities in fanconia enmia

A

Common congenital anomalies are absent radii, hypoplastic thumbs, supernumerary, bifid or absent, anomalies of the feet, congenital hip dislocation and leg abnormalities can also be seen

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21
Q

Cancers in Fanconi

A
  • squamous cell carcinomas of head and neck, esophagus, vulva, anus or cervix
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22
Q

Screening fanconi

A
  • Should get ultrasound of abdomen and echo to rule out other abnormalities when diagnosed
    • CBC every 3mo and bone marrow every year
    • Annual TSH for hypothyroidism, glucose checks
      Solid tumors annually with physical exam and inspection of skin, oral cavity and other organs
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23
Q

Bloom Syndrome ethnicity

A

ashkenzi jews

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24
Q

bloom inheritance

A

AR

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25
Q

bloom clinical fts

A
  • Sensitivity to UV radiation leading to chromosomal breaks
    • May have café au lait spots
    • IGUR, short stature
    • Intellect is normal to average
    • Immunodeficiency
    • GI malabsorption, GERD, hypogonadism is common
    • Increased risk for solid tumors (esp of skin) and lymph malignancies
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26
Q

Schwachmann diamond inhertiance

A

AR

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27
Q

Schwachmann diamond CBC findings

A

Most common to have neutropenia, then anemia, only 21% have pancytopenia, 98% have exocrine pancreatic insufficiency

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28
Q

Schwachmann diamond clinicla ft

A

Exocrine pancreatic insufficiency and metaphyseal dysplasia
- Shor stature is common, usually normal growth velocity though
- Metaphyseal dysplasia, osteopenia, delayed appearance of secondary ossification centers, short or flared ribs and thoracic dystrophy
Some patients have hepatomegaly and elevations in liver enzymes
- Most patients have dental abnormalities
Many have neurocognitive problems and poor social skills

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29
Q

Risk in schwacmann diamond

A

25% develop leukemia by age 18

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30
Q

is neurodevelopment impairment reversible with iron supp

A

No

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31
Q

Most frequent event preceding ACS

A

VOC needing opiods for pain

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32
Q

Platelet lifespan

A

7-10 days

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33
Q

Neutrophil lifespan

A

< 24h

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34
Q

Causes of normocytic anemia

A

TEC, anemia of choronic disease, medications, infections, renal failure, bleeding, hemolysis, de

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35
Q

Macrocytic anemia causes

A

Vitamin B12, folate, liver disease, myelodysplastic syndrome, bone marrwo dailure syndromes, down syndrome

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36
Q

Signs/ Symptoms of IDA

A

pica, koilpnychia, breath holding spells

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37
Q

Red cell indices in IDA

A

RDW elevated, RBC is low or noaml, MCV very low

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38
Q

Peripheral smear in IDA

A

hypochromic microcytic pencil cells

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39
Q

Iron studies in IDA

A

elevated TIBC, decreased ferritin, decreased transferrin saturation

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40
Q

When to repeat bw after starting treatment for IDA

A

check CBC, retic, iron at 1month and 3 months

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41
Q

how much and how long to supplement LBW infants 2-2.5kg iron

A

1-2mg/kg/day for 0-6 months

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42
Q

How much and how long to supplement LBW infants < 2 kg

A

2-3mg/kg/day for 0-12mo

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43
Q

Is iron supp required for LBW infants fed preterm formulas

A

no, these formulas are higher in iron

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44
Q

Pathophys of anemia of chronic disease

A

involves hepcidin elevated levels that decrease GI absorption of iron and limit the expor of iron from amcrophages into red blood cells

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45
Q

TIBC in anemia of chronic disease

A

low/normal

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46
Q

HbA is made of

A

2 alpha, 2 beta chains

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47
Q

emoblogin A2 is made of

A

2 alpha, 2 delta chains

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48
Q

emoglobin F is made of

A

2 alpha, 2 gamma chains

49
Q

Beta thal trait labs

A

Hb is typically normal or low normal, RBC elevated, RDW is low

50
Q

Hb electropheresis of Heta thal trait

A

Hb A2 will be eelbated > 3.5%

51
Q

Mentzer index formula

A

MCV/RBC

52
Q

Mentzer > 13

A

IDA

53
Q

Mentzer 13>

A

thallassemia

54
Q

When does beta thal present

A

after 6 months (no longer making HbF)

55
Q

alpha thal trait

A

microcytosis, asymptomatic, normal hemoglobin, can be cis/trans for genetics implications

56
Q

HbH disease

A

decreased hemoglobin, typically not transfusion dependent, dont typically require chelation, present at birth, three alpha gene deletion

57
Q

Hb Bart

A

Four alpha gene deletion, cannot make hemoglobin A, A2 or F, severe fetal anemia

58
Q

How to diagnose alpha thal trait

A

diagnosed by genetic testing

59
Q

Should you have sickle cells on smear in sickle cell trait

A

no

60
Q

Complications of SCD

A

clinical stroke, silenet stroke, neurocog changes, proliferative retinopathy, asthma, OSA, acute chest, pulm hypertension, cncentrating defects, papillary necrosis, enuresis, liver of splenic sequestration, gallstones, priapism, VOC, aplasticc crisis, functionally asplenic

61
Q

ACS diagnosis

A

fever or resp symptoms and new infiltrated on CXR

62
Q

What does ACS put patients at riskf ro

A

stroke

63
Q

What is clinical stroke in SCD an indication for

A

acute exchange tranfusion

64
Q

Sickle cell surveillance

A

follow-up q6mo, monthyl CBC on hyroxyurea, pen prophylaxis age 2mo until 5 years, folic acid, all extra vaccines, BP at each visit, TCS- annually startni at age 2, at age 10 retinopathy screening, annual u/a and creatinine

65
Q

How often to do PFT and echo in SCD

A

Newest guidelines only if symptomatic

66
Q

Reasons for splenectomy in children

A

sickle cell disease after one episode of sevre splenic sequestration, certain inhertied hemolytic anemias with severe symptoms > 5 years old, transufion dependent thallassemia, chronic ITP refractory to all treatment

67
Q

Complications post splenectomy

A

infection with encapsulated orgnaisms and certain parasites, thrombosis

68
Q

Diamond Blackfan inheritance

A

AD

69
Q

When does DBA present

A

First year of life < 6 mo usually

70
Q

What type of anemia in DBA

A

macrocytic

71
Q

Kids with DBA are at increased risk of

A

MDS/AML

72
Q

ITP treatment with doses

A

prednisone 4mg/kg/day for four days or pred 2mg/kg/day for 1-2 weeks with tapering dose
IVIG 1g/kg

73
Q

How many with ITP that repond to treatment will relapse

A

1/3

74
Q

When is ITP considered chronic

A

> 12 mo

75
Q

%neonates with NAIT with ICH

A

10-25%

76
Q

When to do cranial ultrasound in NAIT

A

plt , 50000

77
Q

Mgmt NAIT

A

HPA1a-ve platelets ASPA, rnadom donor platelets if infant is bleeding and no HPA1a-ve in region

78
Q

iwskott Aldrich syndrome triad

A

small platelets, thrombocytopneia, eczema, immunodeficiency

79
Q

Fanconi anemia buzzwords

A

anmeia,pancytopenia, absent or abnormal thumbs, short and small features, cafe au lait macules, VACTERL anomalies

80
Q

Dyskeratosis congenita buzz words

A

dystrophic nails, lacy reticular rash, white tongue coating (leukoplakia), pancytopenia

81
Q

Schwachman diamond syndrome buzz words

A

neutropenia, expocrine pacratic insuffiicnecy, bony abnomrlaities

82
Q

CAMT

A

thrmbocytopenia, bo birth defects

83
Q

Kassabach Meritt labs

A

thrombocytopenia, low fibronogen, elevated didimer, PTT may be minimally prolonged, usually not a MAHA

84
Q

isoalted INR prolongation

A

factor 7

85
Q

isolated pTT prolongation

A

factor 8, 9, 11, 12, lupus anticoagulant

86
Q

PTT and INR normal, bleeding

A

factor 13 deficiency or not the clotting casacade (vWF, plt nnumber or function problem)

87
Q

test for lupus anticoagulant

A

dilute russel viper venom time

88
Q

vitamin K dependent factors

A
89
Q

Vitamin K deficiency will cause?

A

elevated INR and PTT

90
Q

What becomes abnomrla first in vit k deficiency

A

INR because of short half life of F7

91
Q

vwf is a carrier for

A

f8

92
Q

Normal vwf antigen/activity level

A

> .5

93
Q

Type 1 vWF disease

A

AD, most commonl, botn antigen/activity will be proprtionally low, ratio >.7

94
Q

Type 2 vWF disease

A

AD, ratio < .7

95
Q

type 3 vwf disease

A

levels undetectable, AR, factor 8 severely low range < 1%

96
Q

Treatment vwf type 3

A

vwf/VIII concentrate

97
Q

labs in hemophilia

A

INR normal, PTT prolonged often > 60, fibrinogen normaml, vWF normal, factor 8 levels low < 40%

98
Q

mild/mod/severe hemophilia factor levels

A

5-40, 1-5, < 1%

99
Q

How does Factor 13 defieicny present

A

umbilical stump bleeding or ICH

100
Q

Genetic disorders that increase risk for thrombosis

A

factor V leiden, prothrombin, antithrombin deficiency, protein C and S deficiency
Homocystinura, proteus

101
Q

How does heparin work

A

binding to antithrombin 3 to inhibit factor 10

102
Q

infections from transfusions

A

Hep B» Hep C» HIV

103
Q

Management for VOE in SCD

A

analgesi within 30-60 minutes
avoid cold packs
regular tylenol and advil
acoid overhydration
incentive spirometry

104
Q

ACS management

A

maintain O2 sat gretaer than 95
acoid over hydration
incentive spirometry
broad spectrum anitbiotics
cross match for possible transfusion

105
Q

When are SCD patients highest risk for splenic sequenstration

A

before age 5

106
Q

what are indications for simple RBC transfusion in SCD

A

splenic sequestration
aplasitc crisis

107
Q

indication for exchange transfusion in SCD

A

stroke

108
Q

age for TCD screening SCD patients

A

2-16 to identify high risk pateints

109
Q

Most common human genetic disease

A

SCD

110
Q

what do SCD kids need more vaccines against

A

pneumococcal, meningococcal and addition dose of Hib, Hep A nand B and annual flu vaccine

111
Q

when to give prophylactic antibitooics SCD patients

A

age 2mo-5y, can be extended if splenectomy or hsitory of invasive bacterial infections

112
Q

alternative of SCD prophylaxis if pen allergy

A

cotrimoxazole or erythromycin

113
Q

when should hydroxyurea be offerred

A

all SCD kids over 9mo

114
Q

oxygen sat target SCD patients

A

95 greater

115
Q

ACS definition

A

new pulmonary infiltrate in the presence of fever and respiratory signs or symptoms

116
Q

who is more likely to have splenic sequestration

A

HbSC and HbSB

117
Q

most common cause pediatric stroke

A

SCD

118
Q

when should use screen TCD in SCD patients

A

2-16, no evidence after age 16

119
Q

Low risk criteria for SCD patient with fever

A

These patients can be managed as outpaitnet
fever < 40
> 6 months
WBC 5-20, plt > 100, HB not mroe than 20 below baseline
no resp distress or CSR abnormalitiy
no clinical findings of meningitis
no higostry of sepsis or meningitits
no signfiicant pain or dehydration
initial visit
ability to follow up closely

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