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Heme/Onc > SCD > Flashcards

SCD Flashcards

1
Q

Which chain of hemoglobin is defective in SCD?

A

Beta chain

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2
Q

What are the possible defects in the beta chain in SCD?

A

HbSS
HbSC
SB+-thalassemia (some beta-globulin produced)
SB(null)-thalassemia (no beta-globulin produced)

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3
Q

What are the clinical features of HbSS?

A 
Pain crises
Microvascular disruption of organs
Gallstone
Priapism
Leg ulcers
Anemia
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4
Q

What are the clinical features of HbSC?

A

Painless hematuria
Pain crises less common and occur later in life
Mild anemia

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5
Q

What are SB+-thalassemia clinical features?

A

Rare crises

Less severe than SCD (production of HbA)

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6
Q

What are SB(null)-thalassemia clinical features?

A

Similar severity to HbSS

Anemia

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7
Q

What are the clinical features for sickle cell trait?

A

Generally asx

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8
Q

What element is a sickled RBC lacking?

A

Oxygen

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9
Q

What are the outcomes of sickled cells?

A

Increased viscosity
Adhesion
Occlusion

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10
Q

When do s/sx appear after birth?

A

4-6 months

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11
Q

What are lab findings for SCD?

A

Decreased hgb
Increased retic count
Sickled cells on peripheral blood smear

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12
Q

What is the clinical presentation for SCD?

A 
Acute pain
Dactylitis
Pallor
Weakness
Splenomegaly
Hepatomegaly
Abdominal pain
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13
Q

What infections are we worried about in SCD?

A 
SHiNS
Step pneumoniae
H influenzae type b
Neisseria meningitides
Salmonella
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14
Q

What vaccinations do patients with SCD receive?

A

Pneumococcal
Hib
Meningococcal
Flu

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15
Q

If a patient has HbSS, SB(null) thalassemia, or HbSC, and is 2 months to 3 years old, what penicillin dose do we prophylax with?

A

Oral penicillin VK 125mg PO BID

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16
Q

If a patient has HbSS, SB(null) thalassemia, or HbSC, and 3 years old to 5 years old, what penicillin dose do we prophylax with?

A

Penicillin VK 250 mg PO BID

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17
Q

Which vitamins (and their doses) do we regularly replace?

A

Folic acid 1 mg PO/day

B12 up to 1000mcg PO/day

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18
Q

What are the types of chronic blood transfusion therapy?

A

Simple

Exchange

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19
Q

What are indications for blood transfusions?

A

May reduce stroke risk in children at high risk
Symptomatic severe acute chest syndrome
Acute splenic sequestration and severe anemia

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20
Q

What are risks of chronic blood transfusions?

A

Alloimmunization
Infection transmission
Iron overload

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21
Q

How can we reduce risks in chronic blood transfusions?

A

Blood cross-matching
Immunizations and screening
Iron chelation therapy

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22
Q

When is iron chelation therapy used after chronic transfusions?

A

Consider after a year of transfusions
OR
When ferritin > 1500 mcg/L

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23
Q

What are the types of iron chelation therapies?

A

Deferoxamine
Deferasirox
Deferiprone

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24
Q

Which iron chelation therapies are not oral?

A

Deferoxamine (IV/SQ)

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25
Q

What is the common dose of deferoxamine?

A

20-50 mg/kg/d over 8-24 hours

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26
Q

What is the common dose for deferasirox?

A

Exjade: 20-40 mg/kg/d
Jadenu: 14-28 mg/kg/d

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27
Q

What is the common dose for deferiprone?

A

75-99 mg/kg/d

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28
Q

What is the frequency of deferoxamine dosing?

A

5-7 days/week

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29
Q

What is the frequency of deferasirox dosing?

A

Daily

30
Q

What is the frequency of deferiprone dosing?

A

TID

31
Q

What are the AEs of deferoxamine?

A 
Injection site reactions
Arthralgias
Ocular and ototoxicities
Growth retardation
Renal disturbances
32
Q

What are the AEs of deferasirox?

A

Renal and hepatic failure
Agranulocytosis
Gastrointestinal hemorrhage

33
Q

What are the AEs of deferiprone?

A

QT prolongation
GI upset
Elevated LFT
Agranulocytosis

34
Q

What is the rationale for use of hydroxyurea of SCD?

A

Increased HbF levels
Increased HbF-containing reticulocytes and intracellular HbF
Ribonucleotide reductase inhibitor
Antioxidant properties

35
Q

What are the baseline labs?

A

CBC, reticulocyte count, WBC differential
HbF measurement
Renal and liver function tests
Pregnancy test

36
Q

What is the dosing for hydroxyurea?

A

15 mg/kg PO QD

Max = 35 mg/kg

37
Q

How can hydroxyurea be increased/

A

5 mg/kg/day every 12 weeks

38
Q

What is the dosing of the liquid hydroxyurea suspensions?

A

100mg/ml

39
Q

How do we monitor for hydroxyurea?

A

Efficacy: less pain episodes and hospitalizations, improved well being
HbF: Every 3 months x 2, then every 6 months
CBC w/diff
Periodic renal and liver function tests

40
Q

What are the toxicity cut offs for hydroxyurea?

A 
ANV < 2,000
Plt < 80,000
Hgb < 5 or 20% reduction
SCr > 50% above baseline
ALT 100% increase
41
Q

If there is a toxicity with hydroxyurea, what is the course of action?

A

Stop for 1-2 weeks and resume (if normal counts) at 5 mg/kg/d less than previous dose
If toxicity does not recur after 12 weeks on lower dose, the dose may be increased

42
Q

What is the counseling for hydroxyurea?

A

Bleeding risk
Infections
Status regarding pregnancy
Wash hands before and after handling

43
Q

What oral powder was approved to reduce severe complications associated with SCD in > age 5?

A

L-glutamine

44
Q

What is the MOA of Crizanlizumab?

A

Monoclonal antibody binds to P-selectin and blocks interaction with PSGL-1 (may prevent vasoocclusion by inhibiting adhesion of erythrocytes to vessel walls

45
Q

What SCD affect does crizanlizumab work on?

A

Significantly lower rate of pain crises

46
Q

What are the strategies to modify disease outcomes?

A

Hydroxyurea
L-glutamine
Crizanlizumab
HSCT

47
Q

What causes vasoocclusive crisis or acute pain crisis?

A

Bone or muscle infarction d/t vasoconstriction

48
Q

What is the most common reason for hospitalization?

A

Vasoocclusive crisis or acute pain crisis

49
Q

What is the treatment for vasoocclusive crisis or acute pain crisis?

A

Hydration and pain management

50
Q

What are acute complications of SCD?

A

Vasoocclusive crises/acute pain crisis
Fever and acute infection
Acute chest syndrome

51
Q

Compared to normal, how must we increase hydration in an acute pain crisis or vanoocclusive crisis?

A

1.5 times maintenance

52
Q

What is the regular hydration maintenance requirement for adults?

A

30-35 ml/kg

53
Q

What is the regular hydration maintenance requirement for children?

A

For the first 10kg: 100ml/kg per day
For the 2nd 10kg: 1000ml + 50Ml/kg per day
For more than 20kg: 1500mL + 20 ml/kg per day for each kg over 20 kg

54
Q

Why do we monitor fluid status during hydration?

A

Volume overload can lead to acute chest syndrome

55
Q

What are chronic complications of SCD?

A 
Retinopathy
Anemia, leukocytosis
OSA
Pulmonary hypertension
Indirect hyperbilirubinemia
Cardiomegaly
Chronic renal failure
Functional asplenia
Avascular necrosis
Delayed puberty
Skin ulcers
56
Q

What are acute complications of SCD?

A 
Stroke, meningitis
Post-hyperma glaucoma, retinal infarction
Acute chest syndrome
Sickle hepatopathy
Splenic sequestration, splenic infarction
Cholelithiasis
Priapism
Bone marrow infarction, osteomyelitis
57
Q

Does Fetal Hgb (HgF) sickle?

A

No

58
Q

What are non pharm approaches for pain management in SCD?

A

Hydration
Heating pads
Ect

59
Q

What are pharm options for pain management of SCD?

A

Opioids 1st line for mod-sev pain
Morphine sulfate or hydromorphone most common
PCA with basal and on demand options
NSAIDs in combo with opioids - esp for bone pain
Bowel regimen
Antihistamines for itching
Antiemetics for n/v

60
Q

Which medications are used for bowel regimens?

A

Senna-colace

Polyethylene glycol

61
Q

Why are fever and acute infection increased in SCD?

A

Loss of splenic function

62
Q

What are the manifestations of fever and acute infection in SCD?

A

Pneumonia
Osteomyelitis
Meningitis
Bacteremia

63
Q

What are the likely organisms that occur in SCD?

A

SHiNS
Mycoplasma pneumonia
Viral influenza

64
Q

How do we diagnose infections and fever in SCD?

A 
Fever
WBC
Cultures
Imaging
Lumbar punctures
65
Q

What leads to acute chest syndromes?

A

Vasoocclusive crisis of pulmonary vasculature

66
Q

What are the etiologies of acute chest syndrome?

A

Infection
Pulmonary vascular occlusion
Pulmonary edema

67
Q

What is the presentation of acute chest syndrome?

A 
Acute fever
Respiratory symptoms
New infiltrates on chest x-ray
Hypoxia
Pain
Wheezing
68
Q

What other condition is radiologically indistinguishable from acute chest syndrome?

A

Pneumonia

69
Q

What are the antimicrobials used in acute chest syndromes?

A 
Ceftriaxone 
Or
Cefotaxime + Azith
Or
Moxifloxacin
70
Q

What is the management for acute chest syndrome?

A 
Bronchodilators
Hydration
Pain management
O2
etc

Heme/Onc (20 decks)

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