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Heme boards > Sickle cell > Flashcards

Sickle cell Flashcards

1
Q

Etiology of sickle cell

A

Valine substituted for glutamic acid at 6th position of beta globin chain, leading to decreased solubility, overwhelming RBC skeleton, leading to sickling

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2
Q

hgb electrophoresis in sickle cell anemia

A

90% hgb SS
5% hgb A2
3-4% hgb F

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3
Q

hgb electrophoresis in sickle cell trait

A

60% hgb A
40% hgb S
A2 normal
F normal

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4
Q

Hgb SC electrophoresis

A

50% hgb S
0% hgb A
50% hgb C

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5
Q

hgb SC specific clinical features

A
  • ocular complications, *sickle cell retinopathy. They have a higher incidence of sickle cell retinopathy than HbSS
  • osteonecrosis
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6
Q

sickle b0 phenotype

A

very similar to hgb ss

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7
Q

sickle b0 hgb electrophoresis

A

hgb s at 80%
A2 at 3%
*Just remember very little to no hgb A
hgb F at 17%

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8
Q

sickle b+ hgb electrophoresis

A

hgb s at 80%
A 20%
A2 3%
hgb F at 17%

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9
Q

complications sickle cell trait patients are at risk of

A
  • hematuria
  • renal papillary necrosis
  • pyelo
  • VTE during pregnancy
  • hypothesthenuria (inability of the kidney to concentrate the urine normally)
  • increased UTI’s in pregnancy
  • renal medullary carcinoma
  • risk of severe splenic infarction at high altitude
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10
Q

Malignancy sickle cell patients are at higher risk of

A

Renal medullary carcinoma

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11
Q

Sickle cell additional clinical complications

A
  • developmental delay
  • retinal vessel occlusion
  • osteomyelitis
  • leg ulcers
  • biliary stones
  • hepatic sequestration crises
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12
Q

Encapsulated organisms sickle cell pts are at risk of

A

Please SHind ME Skis
Pseudomonas
Strep
H flu
Meningitis
E coli
Salmonella
Klebsiella
Group b strep

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13
Q

Vaccinations sickle cell patients need

A
  • Strep pneumonia
  • H flu
  • neisseria meningitis
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14
Q

GU complications of sickle cell

A
  • priapism
  • renal medullary necrosis
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15
Q

indications for exchange in sickle cell

A

*acute retinal artery occlusion
- CVA
- priapism
- severe ACS

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16
Q

How to reduce risk of alloimmunization in sickle cell patients

A

Extended red cell phenotyping (looking at kell antigens etc)

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17
Q

presentation of splenic sequestration crises

A
  • sudden drop in hgb (RBCs pool in spleen) + left upper quadrant pain + massive splenomegaly
    *can be in hypovolemic shock
    *Typically occurs in children <2. HOWEVER, adults who have milder forms (HbSC or HbS/b+ can happen because spleen is not auto-infarcted
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18
Q

hyperhemolytic crisis presentation

A
  • hematuria
  • severe hemolysis in the setting of transfusion
19
Q

management of hyperhemolysis in sickle cell

A

*Suppress the antibody
- steroids
- IVIG
+/- rituxan
**Transfuse compatible blood, not exchange transfusion

20
Q

L glutamine evidence in SCD

A
  • decreased frequency of pain crises
21
Q

crizanlizumab mechanism

A

antibody against P selectin, which prevents sickled RBC’s from adhering to vascular endothelium

22
Q

crizanlizumab clinical benefit

A

decreased frequency of pain crises

23
Q

voxelotor mechanism

A

oxygen-affinity modulator.[7] Voxelotor has been shown to have disease-modifying potential by increasing hemoglobin levels and decreasing hemolysis indicators
*So it’s a polymerization inhibitor

24
Q

voxelotor clinical benefit

A

Improved hgb, thus reducing transfusion requirements

25
Q

Howell-Jolly Body clinical significance

A

asplenia or significantly reduced splenic function

26
Q

How to differentiate hgb SS vs. Hgb Sbeta0

A

Hgb Sbeta0 will have low MCV (<70)

27
Q

Clinical features of hemoglobin S with persistence of fetal hemoglobin

A
  • asymptomatic
28
Q

Management of SCD pt with recurrent ACS

A

Chronic transfusion

29
Q

1) How to screen for risk of CVA in kids with SCD 2) next steps

A

transcranial doppler
IF abnormal, need regular transfusions to keep hgb S <30%
*After 12 months can switch to maximally tolerated hydrea

30
Q

Goal hgb S with exchange in setting of CVA

A

<20%

31
Q

Regular screening for SCD

A
  • MRI for silent CVA
  • TTE for pHTN ONLY if signs/symptoms suggestive of pHTN
  • annual albumin/creatinine ratio monitoring
    *IF albuminuria, ACEi
  • annual retinal exam for sickle cell retinopathy (need to see retinal specialist)
  • ferritin every 3 months for transfusional iron overload
32
Q

Management of splenic sequestration

A
  • transfusion (unplugs the spleen)
    *1 unit at a time. By unplugging spleen, blood can gush out and lead to volume overload and hyperviscosity.
33
Q

Sickle cell intrahepatic cholestasis clinical features

A
  • sickle cell occlusion in hepatic sinusoids
  • RUQ pain + very high bili + ALT can be in 1000s
34
Q

Sickle cell intrahepatic cholestasis management

A

Red cell exchange

35
Q

Renal papillary necrosis management

A
  • hydration, urinary alkalization, relieve obstruction
36
Q

Presentation of orbital infarction in SCD

A
  • protrusion of eye + eye pain + lid/orbital edema
37
Q

Management of orbital infarction in SCD

A

IV fluids, pain control, steroids

38
Q

acute aplastic crisis presentation

A

Rapid drop in hgb with reticulocytopenia

39
Q

Goal ferritin for SCD pts with transfusional iron overload

A

Less than 1000

40
Q

hydroxyurea counciling for patients

A

IF child-bearing age,
pregnancy test before starting
**contraception to avoid pregnancy
Men → discuss low sperm count and mobility
IF planning on conceiving, stop for 1-2 months
IF pregnant, STOP

41
Q

L-glutamine mechanism of action in SCD

A

anti-oxidant which may increase availability of reduced glutathione, reducing susceptibility to oxidative stress

42
Q

Management of elective surgery in SCD

A

High risk surgery (prolonged surgery or prolonged impaired blood supply, hypothermia, pulmonary or cardiac disease):
exchange transfusion
Intermediate risk surgery:
simple transfusion to target hgb 10
Low risk surgery
no transfusions

43
Q

approved gene therapy products for SCD

A
  • Lovotibeglogene autotemcel (lovo-cel) (gene addition using viral vector)
  • Exagamglogene autotemcel (exa-cel) (CRISPR gene edited)
44
Q

SE’s/sequelae of gene therapy for SCD

A

Primarily related to busulfan conditioning
- secondary malignancies from busulfan conditioning
- loss of fertility from busulfan conditioning

Heme boards (58 decks)

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