SILENT DAMAGE IN SICKLE
CELL DISEASE (SCD) CAN
PROGRESS WITH SEVERE
CLINICAL CONSEQUENCES1-5

Chronic organ damage is a major cause of morbidity and mortality6,7

CHRONIC COMPLICATIONS MAY GO UNDERREPORTED6,7

75%

Had evidence of
chronic organ damage at autopsy

but only

25%

had chronic organ damage
previously noted in their clinical histories

(n=306; autopsy study)

Chronic organ damage driven by the downstream effects of HbS polymerization—hemolysis, anemia, and vaso-occlusion—is responsible for the morbidity and mortality of most patients with SCD. However, it can often go unrecognized.6,9

There is an ongoing need for improved management of patients with SCD and related end-organ damage. Inhibiting polymerization may provide a key therapeutic strategy to impact red blood cell sickling, which drives the broad cascade of SCD pathologies.10

The pathologic processes of chronic hemolysis,
chronic anemia, and
vaso-occlusion may lead to long-term
tissue and end-organ damage8,9

Complications of SCD8,9

Animated man standing
  • Ischemic/hemorrhagic stroke
  • Silent cerebral infarction (SCI)
  • Cognitive impairment
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  • Retinopathy
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  • Restrictive lung disease
  • Pulmonary hypertension
  • Restrictive cardiomyopathy
  • Acute chest syndrome
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  • Jaundice
  • Gallstones
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  • Infarction
  • Hypersplenism
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  • Hyposthenuria
  • Hyperfiltration
  • Glomerulosclerosis
  • Albuminuria
  • End-stage renal disease
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  • Avascular necrosis
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  • Leg ulcers
  • Priapism
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Common causes of mortality in patients with SCD11

Iron overload
7%
Bleeding
8%
Pneumonia or acute chest syndrome
10%
Cirrhosis (liver disease)
11%
Thromboembolism (blood clots)
15%
Sepsis
18%
Renal failure
23%
Sudden death
23%
Cardiopulmonary (heart/lung disease)
38%
  • 0
  • 5
  • 10
  • 15
  • 20
  • 25
  • 30
  • 35
  • 40

Percentage (%)

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Read about the link between low hemoglobin levels and SCD damage

See the meta-analysis

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References: 1. Gladwin MT. Cardiovascular complications and risk of death in sickle-cell disease. Lancet. 2016;387(10037):2565-2574. 2. Bush AM, Borzage MT, Choi S, et al. Determinants of resting cerebral blood flow in sickle cell disease. Am J Hematol. 2016;91(9):912-917. 3. DeBaun MR, Armstrong FD, McKinstry RC, Ware RE, Vichinsky E, Kirkham FJ. Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood. 2012;119(20):4587-4596. 4. Guasch A, Navarrete J, Nass K, Zayas CF. Glomerular involvement in adults with sickle cell hemoglobinopathies: prevalence and clinical correlates of progressive renal failure. J Am Soc Nephrol. 2006;17(8):2228-2235. 5. Babitt JL, Lin HY. Mechanisms of anemia in CKD. J Am Soc Nephrol. 2012;23(10):1631-1634. 6. Vichinsky E. Chronic organ failure in adult sickle cell disease. Hematology Am Soc Hematol Educ Program. 2017;2017(1):435-439. doi: 10.1182/asheducation-2017.1.435. 7. Manci AE, Culberson DE, Yang YM, et al. Causes of death in sickle cell disease: an autopsy study. Br J Haematol. 2003;123(2):359-365. 8. Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-323. 9. Rees DC, Williams TN, Gladwin MT. Sickle cell disease. Lancet. 2010;376(9757):2018‐2031. 10. Telen MJ, Malik P, Vercellotti GM. Therapeutic strategies for sickle cell disease: towards a multi‐agent approach. Nat Rev Drug Discov. 2019;18(2):139-158. 11. Darbari DS, Kple-Faget P, Kwagyan J, Rana S, Gordeuk VR, Castro O. Circumstances of death in adult sickle cell disease patients. Am J Hematol. 2006;81(11):858-863.

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