CT imaging of splenic sequestration in sickle cell disease

2000 ◽  
Vol 30 (12) ◽  
pp. 830-833 ◽  
Author(s):  
S. Sheth ◽  
C. Ruzal-Shapiro ◽  
S. Piomelli ◽  
W. E. Berdon
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Ct Imaging ◽  
Cell Disease ◽  
Splenic Sequestration

scholarly journals A case of severe multi-system disease in a sickle cell SC patient caused by parvovirus B19 infection

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Mohamed Almuqamam ◽  
◽  
Swetha Madhavarapu ◽  
Nataly Apollonsky ◽  
◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Acute Infection ◽  
Organ Injury ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Increased Risk ◽  
Parvovirus B19 Infection

Sickle Cell Disease (SCD) is an inherited hemoglobinopathy, which results in production of abnormal hemoglobin S. HbSC disease is a variant of SCD, which shares a similar clinical complication profile to HbSS disease, but often thought to be a milder condition. In patients with SCD, Hb S in deoxygenated state undergoes polymerization, leading to hemolysis, vaso-occlusive events, and eventually end-organ damage. Among other complications in patients with SCD is increased risk of complications caused by parvovirus B19. We present a case of a 14-year-old female with HbSC disease who presented to the emergency room with complaint of abdominal pain and found to have splenic sequestration. Splenic sequestration progressed rapidly, Hemoglobin (hb) dropped to 4.6 g/dl and acute chest syndrome (ACS) developed. She was treated following the ACS protocol, received 4 units of Packed Red Blood Cells (PRBC) and subsequently underwent a single volume PRBC exchange transfusion. Considering her unusual presentation, with severe ARDS from alveolar hemorrhage requiring mechanical ventilation and multi-organ injury, several autoimmune and infectious conditions with a cytokine storm component including COVID-19 disease, were considered. Results of viral testing revealed parvovirus B19 IgM antibodies signifying an acute infection. She fully recovered with supportive care and was discharged home. Multisystem involvement simulating connective tissue disorders or malignancies with acute parvovirus B19 infection has been reported and is considered extremely rare. To our knowledge, there were no reports of pediatric patients with SC disease presenting with splenic sequestration and ACS in the setting of parvovirus B19 multisystem disease. Keywords: sickle cell disease; acute respiratory distress syndrome; acute chest syndrome; parvovirus B19.

Prevalence and predictive factors of splenic sequestration crisis among 423 pediatric patients with sickle cell disease in Tunisia

2020 ◽  
Vol 80 ◽  
pp. 102374
Author(s):  
Monia Ben Khaled ◽  
Monia Ouederni ◽  
Yosra Mankai ◽  
Samia Rekaya ◽  
Ilhem Ben Fraj ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Predictive Factors ◽  
Pediatric Patients ◽  
Cell Disease ◽  
Splenic Sequestration

scholarly journals Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease [see comments]

Blood ◽  
1995 ◽  
Vol 86 (2) ◽  
pp. 776-783 ◽  
Author(s):  
FM Gill ◽  
LA Sleeper ◽  
SJ Weiner ◽  
AK Brown ◽  
R Bellevue ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Course ◽  
The United States ◽  
Beta Thalassemia ◽  
Acute Chest Syndrome ◽  
Cooperative Study ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Clinical Events

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.

scholarly journals SUSTAIN: A Multicenter, Randomized, Placebo-Controlled, Double-Blind, 12-Month Study to Assess Safety and Efficacy of SelG1 with or without Hydroxyurea Therapy in Sickle Cell Disease Patients with Sickle Cell-Related Pain Crises

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1-1 ◽  
Author(s):  
Kenneth I. Ataga ◽  
Abdullah Kutlar ◽  
Julie Kanter ◽  
Darla Liles ◽  
Rodolfo Cancado ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Chest Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Annual Rate ◽  
Cell Disease ◽  
Double Blind ◽  
Splenic Sequestration ◽  
Secondary Endpoints ◽  
Equity Ownership

Abstract Introduction: Acute painful episodes, frequently called sickle cell-related pain crises (SCPC), are a substantial cause of morbidity in sickle cell disease (SCD). Although hydroxyurea (HU) is known to decrease the frequency of SCPC in sickle cell anemia, many patients continue to experience acute painful episodes despite such therapy. P-selectin is an adhesion molecule expressed on activated vascular endothelial cells and platelets. It is a key molecule in the initiation of leukocyte rolling on the vessel wall that leads to firm attachment and extravasation to underlying tissues during inflammation. Upregulation of P-selectin on endothelial cells and platelets also contributes to the cell-cell interactions involved in the pathogenesis of SCPC. The SUSTAIN study evaluated the safety of SelG1, a first-in-class humanized anti-P-selectin antibody, and its effect on the frequency of SCPC in SCD patients. Methods: We conducted a randomized, double-blind, placebo-controlled, multinational study. Patients were randomized to receive placebo, 2.5 mg/kg or 5.0 mg/kg SelG1; patients received their initial dose, a dose 14 days later, and then every 4 weeks through week 50 for a total of 14 doses. The primary efficacy endpoint was the annual rate of SCPC in the 5.0 mg/kg SelG1 group vs. placebo. A hierarchical testing procedure was employed (α = 0.05 for high dose vs. placebo, and if significant, low dose vs. placebo). An SCPC was defined as acute sickle cell-related pain that resulted in a visit to a medical facility and required a parenteral or oral narcotic or parenteral NSAID. Acute chest syndrome (ACS), priapism, hepatic and splenic sequestration were also included in this definition. A blinded, independent committee adjudicated all SCPC events. Key inclusion criteria included patients 16 to 65 years of age; diagnosis of SCD (HbSS, HbSC, HbSβ0 thalassemia or HbSβ+ thalassemia); and history of 2 to 10 SCPC in the previous 12 months. Patients receiving HU or erythropoietin were included if prescribed for the preceding 6 months and dose was stable for at least 3 months. The randomization was stratified by historical SCPC in the prior year (2-4 or 5-10) and concomitant HU use (yes or no). Secondary endpoints included annual rate of days hospitalized, times to first and second SCPC and annual rate of uncomplicated SCPC (defined as typical SCPC other than ACS, priapism and hepatic or splenic sequestration) and ACS. Results: 198 SCD patients were randomized for the 1-year study. The Intent-To-Treat (ITT) population included all randomized patients; 67, 66 and 65 patients in the 5.0 mg/kg, 2.5 mg/kg and placebo groups, respectively. Demographic parameters were evenly distributed in the treatment groups. The primary endpoint, the annual rate of SCPC in the ITT population at 5.0 mg/kg vs. placebo, was reduced 47% (medians of 1.6 vs. 3.0, p = 0.010, Table 1). The SelG1 drug effect was dose-dependent as the annual rate of SCPC at 2.5 mg/kg vs. placebo was reduced 33% (medians of 2.0 vs. 3.0, p = 0.180). Time to first SCPC at 5.0 mg/kg vs. placebo was increased 2.9-fold (medians of 4.1 vs. 1.4 months, p = 0.001, Fig. 1) and time to second SCPC was increased 2.0-fold (medians of 10.3 vs. 5.1 months, p = 0.022, Fig. 2). The annual rate of uncomplicated SCPC at 5.0 mg/kg vs. placebo was reduced by 62% (medians of 1.1 vs. 2.9, p = 0.015). ACS events were rare in this study. The annual rate of days hospitalized at 5.0 mg/kg vs. placebo showed a non-significant, 42% reduction (medians of 4.0 vs. 6.9, p = 0.450). Adverse events that occurred in 5% or more of patients in an active dose group and were elevated over placebo by at least 2-fold were arthralgia, pruritus, vomiting, chest pain, diarrhea, road traffic accident, fatigue, myalgia, musculoskeletal chest pain, abdominal pain, influenza and oropharyngeal pain. There were no apparent increases in infections with SelG1 treatment. Five deaths occurred during the study, 2 at 5.0 mg/kg, 1 at 2.5 mg/kg and 2 in placebo; no deaths were deemed related to study drug. Conclusions: The P-selectin inhibitor SelG1 significantly reduced SCPC and appeared to be safe and well tolerated. Significant improvements were also achieved for several secondary endpoints including increases in times to first and second SCPC. Chronic inhibition of P-selectin with once a month IV dosing of SelG1 represents a novel and potentially new disease-modifying, prophylactic treatment option for patients with SCD. clinicaltrials.gov: NCT01895361 Disclosures Kutlar: Novartis Pharmaceuticals: Research Funding. Kanter:Novartis: Consultancy. Rollins:Selexys Pharmaceuticals: Equity Ownership, Other: Previous Employment. Stocker:Selexys Pharmaceuticals: Equity Ownership, Other: Previous Employment. Rother:Selexys Pharmaceuticals: Equity Ownership, Other: Previous Employment.

Children with Sickle Cell Disease on Chronic Red Cell Transfusion Experience Fewer Hospitalizations for Acute Vaso-Occlusive Episodes Irrespective of the Indication for Transfusion

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4282-4282
Author(s):  
Carmen C Wallace ◽  
Hilda Mata ◽  
Nancy J Wandersee ◽  
J. Paul Scott ◽  
Amanda M Brandow ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Admission Rate ◽  
T Test ◽  
Acute Chest Syndrome ◽  
Red Cell ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Red Cell Transfusion ◽  
The Impact

Abstract While there is strong evidence that chronic red cell transfusion is effective in preventing primary stroke and reducing the risk of recurrent stroke in sickle cell disease (SCD), it is less clear whether chronic transfusions will prevent admissions for other acute vaso-occlusive complications, including pain, priapism and/or acute chest syndrome. To our knowledge, no study to date has investigated the effect of chronic transfusion on the frequency of admissions for acute vaso-occlusive complications in children with all diagnoses of SCD and treated with chronic transfusion for a variety of indications. In addition, this study included a special focus on the effect of chronic transfusion on children who were transfused specifically for recurrent vaso-occlusive episodes. We performed a single-site retrospective chart review. We selected subjects from all children aged 0 to 19 years who were treated (lived in the Milwaukee area) and followed by the Wisconsin Sickle Cell Center at Children’s Hospital of Wisconsin from 1984 to May 2014 (n=695 subjects). Data was extracted from any individual who was enrolled in a chronic transfusion program for a minimum of six months. Data on admissions for painful vaso-occlusive crises, acute chest syndrome (ACS), other SCD complications as well as sickle diagnosis, age at time of transfusion, CBC, reticulocyte count, and percent sickle hemoglobin (HbS%) were collected for 24 months prior to onset of transfusion and for all months during transfusion until the age of 19 yrs. Unless otherwise indicated, all statistical analyses on extracted data were done by paired Student’s t-Test. We extracted data from 103 unique subjects for 108 chronic transfusion programs (as defined above); 5 subjects were chronically transfused twice, separated by at least 4 years without chronic transfusion. 55% were female; average age was 8.6 ± 5.6 (mean ± SD) years and the sickle diagnosis included 94% SS, 3% SC, 2% Sβ°-Thalassemia and 1% SD. The indication for transfusion included pain (n=31), priapism (n=6), ACS (n=5), central nervous system complications (n=37, including stroke, TIA, and abnormal TCD), splenic sequestration (n=25), pulmonary hypertension (n=2), retinopathy (n=1) and osteomyelitis (n=1). The hemoglobin level increased from a baseline of 7.6 ± 2.2 gm/dL to 9.6 ± 0.8 gm/dL during transfusion (p<0.0001, paired t-Test). HbS% was also reduced from a baseline of 84.2 ± 10.8% to 35.8 ± 0.3% during transfusion (p<0.0001). We found that rate of admissions for acute painful episodes, including priapism, dropped from 2.2 ± 2.9 admits/yr during the 24 months pre-transfusion to 1.0 ± 1.9 admits/yr during transfusion (p<0.0001). Similarly, the rate of admission for ACS decreased from 0.3 ± 0.5 admits/yr for 24 months pre-transfusion to 0.1 ± 0.3 admits/yr during transfusion (p=0.0001). Subanalyses were performed on specific indications for transfusion. For children transfused due to frequent acute vaso-occlusive complications (pain, priapism and ACS were arbitrarily included in this group), the average age at initiation of transfusion was 11.9 ± 4.4 yr, and admissions for acute painful episodes dropped from 4.0 ± 3.2 admits/yr during the 24 months pre-transfusion to 2.1 ± 2.6 admits/yr during transfusion (p=0.003). When the indication for transfusion was splenic sequestration (age 2.3±2.7 yr), the admission rate for acute painful episodes did not change (0.8±1.7 vs 0.3±0.5 admits/yr, p= 0.14). For children transfused for CNS complications (age 8.5±4.6 yr), the admission rate for pain improved from 0.9±1.3 to 0.2±0.5 admissions/yr (p=0.007). In agreement with previous studies, our data also showed an increase in the rate of admissions for pain (nontransfused) as subjects aged (r2=0.19, p<0.0001). Thus, the significant improvement in admission rate for pain during transfusion, while the child continues to age, further accentuates the impact of transfusion on the natural history of pain in SCD. In summary, our data suggest that chronic transfusion reduces hospital admissions for pain and acute chest syndrome in children with SCD. Our data also support the notion that chronic transfusion is an effective treatment to prevent not only stroke, but also other painful, life-threatening and life-limiting complications of sickle cell disease. Disclosures No relevant conflicts of interest to declare.

Complication Rates in Patients with Sickle Cell Disease Living Chronically at Moderate Altitude

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4095-4095 ◽  
Author(s):  
Claire L Stokes ◽  
Christopher M McKinney ◽  
Christopher C Silliman
Keyword(s):  
Sickle Cell Disease ◽  
Sea Level ◽  
Oxygen Tension ◽  
Sickle Cell ◽  
Rate Ratio ◽  
Incidence Rates ◽  
Complication Rates ◽  
Moderate Altitude ◽  
Cell Disease ◽  
Splenic Sequestration

Abstract Background: The increased risk of acute vaso-occlusive pain crisis and splenic infarction in children with sickle cell disease (SCD) acutely exposed to altitude has been well documented. However, little is known about complication rates in children chronically living at moderate altitude. We hypothesize that children with SCD experience more complications than children with SCD living at sea level. Methods: A retrospective chart review of all patients with sickle cell disease followed at the Children's Hospital Colorado between January 2001 and December 2010 was completed. Patients observed for less than one year were excluded from analysis. Incidence rates for vaso-occlusive crisis (VOC), acute chest syndrome (ACS), splenic sequestration, and stroke were calculated. Rate ratios and 95% confidence intervals were determined comparing to children with SCD from institutions located near sea level. Secondary measures looked at baseline hematologic indices collected at annual comprehensive care visits and percentage of patients with abnormal tricuspid regurgitation jet velocity (TRV). Two-tailed Student's t-tests were used to compare means of continuous variables. Results: 179 children were observed for a total of 1032.37 patient-years with demographics (Table 1). . At moderate altitude, patients with Hgb SS experienced about a 20% higher rate of VOC compared to historic controls with a rate ratio of 1.19 (1.03-1.39) (Table 2). Patients with Hgb SC had almost 3 times the number of admissions for splenic sequestration than those at sea level with a rate ratio of 2.93 (1.05-8.02). Incidence rates for ACS and stroke appeared to have been higher at moderate altitude than sea level, but did not reach statistical significance. There was also no difference in the percentage of patients with abnormal TRV. Baseline lab values were less than the 95%ile except for the hemoglobin of 11.7 for SC patients (Brown et al 1994). Discussion: The oxygen tension at an elevation of 5,280 feet (1,609 m) is 20% lower than at sea level due to the reduction in barometric pressure. Reduced oxygen tension may lead to increased hemoglobin S polymerization and red cell sickling. Hemoglobin SC patients have higher baseline hemoglobins, and their increase in splenic sequestration may be due to increased blood viscosity. Interestingly, the rate of ACS and pulmonary hypertension did not seem to be significantly elevated in our patients living at moderate altitude. This may be due to a lack of statistical power given the small size of this single institution study. Another limitation of this study is the comparison to data from multiple institutions near sea level which does not necessarily control for other possible contributing factors, e.g. climate. Also, VOC events were defined as hospitalizations requiring parenteral opioid administration, which is a stricter definition than used in the sea-level data. Thus, the risk ratio may be underestimated. Nevertheless, the data supports the anecdotal experience that patients living chronically at moderate altitude have increased sickle cell-related complication rates. Table 1 Table 1. Demographics Table 2 Table 2. Complication Incidence Rates 1 -compared to Gill et. al, Blood, 1995 2 - compared to Vichinsky et. al, Blood, 2012 3- compared to Brousse et. al, BJH, 1997 4- compared to Pashankar et. al, Pediatrics, 2007 5- compared to Quinn et. al, Blood, 2008 Disclosures No relevant conflicts of interest to declare.

O402 Acute splenic sequestration in pregnant woman with homozygous sickle cell disease

2009 ◽  
Vol 107 ◽  
pp. S206-S207
Author(s):  
A. Igai ◽  
R. Nomura ◽  
C. Maia ◽  
G. Fonseca ◽  
S. Gualandro ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Homozygous Sickle Cell Disease
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