Abstract

Background

Priapism is a known complication of sickle cell disease (SCD). Though, blood transfusion is often used in the management of SCD-associated priapism (SAP), its safety and efficacy is not known. Transfusion has been associated with neurologic events including ASPEN syndrome (Association of Sickle cell disease, Priapism, Exchange transfusion, and Neurologic events) [Siegel et al. J Urol 1993]. A recent systematic review suggests that transfusion does not appear to be effective in SAP as determined by time to detumescense (Merritt et al. Can J Emerg Med 2006). Our objective is to examine the relationship between blood transfusions and neurologic complications (NCs) in the management of SAP.

Methods

We reviewed the data from 33 children’s hospitals within the Pediatric Health Information System (PHIS) database from 2000-2011. Patients ≤ 21 years of age with SCD were included for the analysis. SCD, priapism and all related conditions were identified by ICD-9 codes. We abstracted data on demographics, hospitalizations, transfusion, urologic procedures and neurologic changes (including stroke, seizure, headache, weakness, transient-ischemic attacks (TIAs), intracranial hemorrhage, and mental status changes). Fisher’s exact test was used to compare the prevalence of NCs. A p-value <.05 was considered statistically significant.

Results

From the year 2000 to 2011, a total of 7,929 unique male pediatric patients with SCD were identified. Among these 465 (5.9%) patients with 1,069 hospitalizations were identified as having SAP. The majority of these patients were HbSS (n=447; 96%). The frequency of SAP hospitalizations at various age groups were; 4.7% (0-2 years of age), 11.2% (3-5 years), 38% (6-12 years) and 46% (13-21 years). In the majority of SAP hospitalizations (63%, n=673) neither transfusions nor urologic procedures were performed, whereas blood transfusions, urologic procedures or both were performed in 25.2% (n=269), 6.6% (n=71) and 5.2% (n=56) of SAP hospitalizations respectively. There was no significant difference in the incidence of NCs between the patients who received transfusions and those who did not (4.1% vs. 4.7%, p = NS). Both bivariate (Table 1) and multivariate regression analysis (Table 2) revealed no statistically significant associations between NCs and blood transfusion, urologic procedure, age at hospitalization for priapism, or other SCD co-morbidities (VOC and ACS).

Table 1

Bivariate analysis of risk factors for neurological complications in SAP hospitalizations

Variable Neurological Complications p-value 
No (n=1021) Yes (n=48) 
Frequency Percent Frequency Percent 
Acute Chest Syndrome 0.202 
No 962 95.72 43 4.28  
Yes 59 92.19 7.81  
Vaso-Occlusive Crisis 0.869 
No 274 95.80 12 4.20  
Yes 747 95.40 36 4.60  
Transfusion/Procedure Categorization 0.856 
No Transfusion/Procedure 641 95.25 32 4.75  
Transfusion-only 258 95.91 11 4.09  
Procedure-only 69 97.18 2.82  
Both Transfusion/Procedure 53 94.64 5.36  
Variable Neurological Complications p-value 
No (n=1021) Yes (n=48) 
Frequency Percent Frequency Percent 
Acute Chest Syndrome 0.202 
No 962 95.72 43 4.28  
Yes 59 92.19 7.81  
Vaso-Occlusive Crisis 0.869 
No 274 95.80 12 4.20  
Yes 747 95.40 36 4.60  
Transfusion/Procedure Categorization 0.856 
No Transfusion/Procedure 641 95.25 32 4.75  
Transfusion-only 258 95.91 11 4.09  
Procedure-only 69 97.18 2.82  
Both Transfusion/Procedure 53 94.64 5.36  
Table 2

Predictors of neurological complications in SAP hospitalizations.

Variable Log Odds Std. Error p-value 
Acute chest syndrome 0.740 0.517 0.1527 
Vaso-occlusive crisis 0.053 0.354 0.8807 
No Transfusion or Procedure reference -- -- 
Transfusion Only -0.234 0.366 0.5243 
Procedure Only -0.546 0.748 0.4666 
Both Transfusion & Procedure 0.240 0.633 0.7054 
Age at first Priapism    
0-2 years reference -- -- 
3-5 years -0.335 1.193 0.7791 
6-12 yrs -0.045 1.065 0.9664 
13-15 yrs 0.431 1.059 0.6840 
16-21 yrs 0.196 1.078 0.8554 
Variable Log Odds Std. Error p-value 
Acute chest syndrome 0.740 0.517 0.1527 
Vaso-occlusive crisis 0.053 0.354 0.8807 
No Transfusion or Procedure reference -- -- 
Transfusion Only -0.234 0.366 0.5243 
Procedure Only -0.546 0.748 0.4666 
Both Transfusion & Procedure 0.240 0.633 0.7054 
Age at first Priapism    
0-2 years reference -- -- 
3-5 years -0.335 1.193 0.7791 
6-12 yrs -0.045 1.065 0.9664 
13-15 yrs 0.431 1.059 0.6840 
16-21 yrs 0.196 1.078 0.8554 
Conclusion

In our large pediatric sickle cell cohort, NCs associated with priapism were rare. Moreover, there was no significant association between the neurologic complications and blood transfusions or urologic procedures in SAP hospitalizations. Further studies are needed to understand the mechanism behind these neurologic complications and any associated risk factors.

Disclosures:

No relevant conflicts of interest to declare.

Author notes

*

Asterisk with author names denotes non-ASH members.