Partial splenectomy prevents splenic sequestration crises in sickle cell disease

Vick, Laura R.; Gosche, John R.; Islam, Saleem

doi:10.1016/j.jpedsurg.2009.06.007

Cited by 13 publications

(4 citation statements)

References 11 publications

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“…A medical history characterized by prescription pain medication, blood transfusions and a number of these diagnoses (i.e., retinopathy, avascular necrosis, leg ulcers, kidney disease, stroke, and pulmonary hypertension) in a person presenting with SCD could indicate severe disease [65–69]. In the absence of a consensus method for determining severity, we reasoned that a method which mimicked the clinical interview in content would identify patients who differed in the amount of SCD-related damage caused by their sickle cell, and could, thus, serve as a surrogate marker of disease severity.…”

Section: Methodsmentioning

confidence: 99%

Sensitivity of alternative measures of functioning and wellbeing for adults with sickle cell disease: comparison of PROMIS® to ASCQ-Me℠

Keller

Yang

Treadwell³

et al. 2017

Health Qual Life Outcomes

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BackgroundSickle Cell Disease (SCD) causes profound suffering and decrements in daily functioning. Demand is growing for valid and reliable measures to systematically document these effects, particularly in adults. The Adult Sickle Cell Quality of Life Measurement System, ASCQ-Me℠, was developed for this purpose. ASCQ-Me℠ is one of four measurement systems housed within the Person–Centered Assessment Resource (PCAR), funded by the National Institutes of Health, to support clinical research. To help users select the best of these measures for adults with SCD, we evaluated and compared two PCAR systems: one designed to be “universally applicable” (the Patient-Reported Outcome Measurement Information System, PROMIS®) and one designed specifically for SCD (ASCQ-Me℠).MethodsRespondents to PROMIS and ASCQ-Me questions were 490 adults with SCD from seven geographically-disbursed clinics within the US. Data were collected for six ASCQ-Me measures (Emotional Impact, Sleep Impact, Social Impact, Stiffness Impact, Pain Impact, SCD Pain Episode Frequency and Severity) and ten PROMIS measures (Pain Impact, Pain Behavior, Physical Functioning, Anxiety, Depression, Fatigue, Satisfaction with Discretionary Social Activities, Satisfaction with Social Roles, Sleep Disturbance, and Sleep-Related Impairment). Statistical analyses, including analysis of variance and multiple linear regression, were conducted to determine the sensitivity of measures to SCD severity. SCD severity was assessed via a checklist of associated treatments and conditions.ResultsFor those with the most severe SCD, PROMIS scores showed worse health compared to the general population for nine of ten health domains: the magnitude of the difference ranged 0.5 to 1.1 standard deviation units. The PROMIS domains most severely affected were Physical Functioning and Pain (Impact and Behavior). Significant differences by tertile of the SCD-MHC were shown for most PROMIS short forms and all ASCQ-Me short and fixed forms. In most models, ASCQ-Me measures explained statistically significant unique variance in SCD-MHC scores complementary to that explained by corresponding PROMIS measures.ConclusionsStudy results supported the validity of both PROMIS and ASCQ-Me measures for use in adults with SCD. Compared to comparable PROMIS scores, most ASCQ-Me scores were better predictors of SCD disease severity, as measured by a medical history checklist. The clinical implications of these results require further investigation.

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Section: Methodsmentioning

confidence: 99%

Sensitivity of alternative measures of functioning and wellbeing for adults with sickle cell disease: comparison of PROMIS® to ASCQ-Me℠

Keller

Yang

Treadwell³

et al. 2017

Health Qual Life Outcomes

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“…Due to concerns of the risks of TS such as OPSI, vascular thrombosis, and vascular derangements, there has been increasing interest in recent years in the use of PS for these children. Small case series have shown that PS is safe in children with SCD, although the clinical efficacy of PS compared to TS are unclear 7, 9, 18–24. In this retrospective study, we wanted to test whether a REDCap based disease registry could be used to record and analyze clinical outcomes of children with SCD undergoing partial or TS.…”

Section: Discussionmentioning

confidence: 99%

“…Clinically evident thrombosis occurs in up to 10% of patients following splenectomy, which may be increased further with use of laparoscopy 5–7. Following splenectomy, there is an increase in intravascular hemolysis, which may worsen pulmonary hypertension 8–10. This is a major concern for children with SCD who have a significant long‐term risk of developing pulmonary hypertension.…”

Section: Introductionmentioning

confidence: 99%

“…The goal of a PS is to achieve an acceptable hematologic outcome while minimizing the risks of TS. For children with SCD, PS has been less frequently employed; although several small studies have reported favorable clinical outcomes with a marked reduction in rate of splenic sequestration and transfusion requirements 7–10. However, pediatric centers have a wide variety of opinions for the use of TS and PS, citing a lack of quality comparative outcome data.…”

Section: Introductionmentioning

confidence: 99%

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Evaluation of partial and total splenectomy in children with sickle cell disease using an internet‐based registry

Mouttalib

Rice

Snyder

et al. 2012

Pediatric Blood & Cancer

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Background Clinical outcomes of children with sickle cell disease (SCD) who undergo total or partial splenectomy are poorly defined. The purpose of this retrospective study was to initiate an internet-based registry to facilitate analysis of clinical outcomes for these children. We hypothesized that both surgical procedures would be well tolerated and would eliminate risk of splenic sequestration. Methods We developed a web-based registry using the Research Electronic Data Capture (REDCap) platform. Children were included if they had SCD and underwent total splenectomy (TS) or partial splenectomy (PS) between 2003 to 2010. Clinical outcomes were compared between cohorts, with follow-up to one year. Results Twenty-four children were included, total splenectomy (n=15) and partial splenectomy (n=9). There were no differences in surgical time or intraoperative blood loss. The length of stay was longer after PS (4.1±1.7 days) compared to TS, (2.4±1.2 days, p=0.02). Within 30 days of surgery, 2 (20%) patients had acute chest syndrome following TS and 2 (15%) patients had acute chest syndrome after PS. During one year follow-up, no patient in either cohort had recurrent splenic sequestration, venous thrombosis or overwhelming post-splenectomy sepsis. All children who were transfused preoperatively to prevent recurrent splenic sequestration successfully discontinued transfusions. Conclusions Both TS and PS result in favorable hematologic outcomes and low risk of adverse events for children with SCD. A REDCap based registry may facilitate data entry and analysis of clinical outcomes to allow for comparison between different types of splenectomy.

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Clearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease

Sissoko,

Cissé,

Duverdier

et al. 2024

American J Hematol

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In children with sickle cell disease (SCD), splenectomy is immediately beneficial for acute sequestration crises and hypersplenism (ASSC/HyS) but portends a long‐term risk of asplenia‐related complications. We retrieved peripheral and splenic red blood cells (RBCs) from 17 SCD children/teenagers undergoing partial splenectomy for ASSC/HyS, 12 adult subjects without RBC‐related disease undergoing splenectomy (controls), five human spleens perfused ex vivo with HbSS‐ and HbAA‐RBC, and quantified abnormal RBC by microscopy, spleen‐mimetic RBC filtration, and adhesion assays. Spleens were analyzed by immunohistochemistry and transmission electron microscopy (TEM). In circulating blood of SCD and control subjects, dysmorphic (elongated/spherocytic) RBCs were <2%, while proportions of pocked‐RBC were 4.3‐fold higher in SCD children than in controls. Compared to controls, splenic RBCs were more frequently dysmorphic (29.3% vs. 0.4%), stiffer (42.2% vs. 12.4%), and adherent (206 vs. 22 adherent RBC/area) in SCD subjects. By TEM, both polymer‐containing and homogenous RBC contributed to spleen congestion, resulting in 3.8‐fold higher RBC population density in SCD spleens than in control spleens, predominantly in the cords. Perfused spleens with normal function displayed similar congestion and retention of dysmorphic RBC as SCD spleens. The population density of active macrophages was similar in SCD and control spleens, with a relative deficit in phagocytosis of polymer‐containing RBC. Despite the existence of hyposplenism, splenectomy in SCD children removes an organ that still efficiently filters out potentially pathogenic altered RBC. Innovative treatments allowing fine‐tuned reduction of RBC retention would alleviate spleen congestion, the major pathogenic process in ASSC/HyS, while preserving spleen protective functions for the future.

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Partial splenectomy prevents splenic sequestration crises in sickle cell disease

Cited by 13 publications

References 11 publications

Sensitivity of alternative measures of functioning and wellbeing for adults with sickle cell disease: comparison of PROMIS® to ASCQ-Me℠

Sensitivity of alternative measures of functioning and wellbeing for adults with sickle cell disease: comparison of PROMIS® to ASCQ-Me℠

Evaluation of partial and total splenectomy in children with sickle cell disease using an internet‐based registry

Clearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease

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