Acute Splenic Sequestration Crises Research Articles

Avascular Bone Necrosis in Pediatric Patients with Sickle Cell Disease in Basrah, Iraq

BACKGROUND: Avascular necrosis (AVN) is regarded as a manifestation of severe sickle cell disease (SCD), with the femoral head being the most affected. OBJECTIVES: The main objectives of this study were to look for the frequency of AVN in pediatric patients with SCD, evaluate the clinical pattern and severity of AVN, and study the possible risk factors associated with AVN. MATERIALS AND METHODS: A cross-sectional study has been conducted on SCD patients, aged 6–18 years, who visited the Basrah Center for Hereditary Blood Diseases from the first of February 2021 to August 2021. Patients were screened for AVN by hip plain radiography and magnetic resonance imaging. The modified Ficat-Arlet staging system was used to classify different stages of AVN. RESULTS: The total number of screened patients was 291; 193 (66.3%) had sickle cell anemia, 71 (24.4%) with S/β0 thalassemia, 21 (7.2%) with S/β+ thalassemia, and 6 (2.1%) had S/D disease. Fifty-eight (19.9%) patients were found to have different stages of AVN; 7 (12.1%) were asymptomatic and 51 (87.9%) were symptomatic. The logistic regression analysis has revealed that frequent vaso-occlusive crises requiring hospitalization (B = 1.576, P = 0.003), acute splenic sequestration crises (B = 1.256, P = 0.003), homozygous sickle hemoglobin genotype (B = −0.208, P = 0.001), and low reticulocyte count (B = 1.452, P = 0.027) are significant variables associated with AVN. CONCLUSION: AVN was reported in a significant percentage of pediatric patients with SCD and was associated with selected indicators of disease severity. Further studies that evaluate the natural history, progress of AVN, and variations in selected variables over time like reticulocytes and the co-inheritance of α-thalassemia are important.

197 Annual Hospitalization and Mortality Trends Among Patients With Sickle Cell Disease and Clostridioides difficile Infection

INTRODUCTION: Frequent hospitalizations, antibiotic exposure, dysbiosis and impaired immune function associated with Sickle Cell Disease (SCD) predisposes pts to Clostridioides difficile infection (CDI). As a result, we hypothesize that the incidence of CDI in those with SCD has been increasing in the United States and that those with SCD and CDI have worse outcomes. METHODS: Using ICD-9 codes within the National Inpatient Sample Database, we identified pts between 2005–2012 with SCD. For each pt, we obtained various factors (e.g., demographics, medical co-morbidities and outcomes), grouped them as SCD and CDI (SCD + CDI) or those with SCD without CDI (SCD − CDI) and statistically compared. Using propensity score matching, SCD + CDI pts were matched with SCD − CDI in 1:1 ratio. All categorical variables were compared with Pearson's χ2 test and continuous variables were analyzed with paired t-test (SAS 9.4, Cary, NC). RESULTS: Among 747,127 hospitalized pts with SCD, 804 (0.1%) had SCD + CDI. SCD + CDI were more likely to be African Americans (88.0% v 32.7%, P < 0.0001), smokers (10.5% v 7.1%, P < 0.0001), and had a higher association with HIV (2.2% v 0.9%, P = 0.0001), Hepatitis C (1.6% v 0.8%, P = 0.007), chronic liver disease (6.0% v 2.8%, P < 0.0001), cholelithiasis (4.0% v 2.2%, P < 0.001), acute pancreatitis (2.5% v 1.4%, P < 0.0001) and chronic pancreatitis (2.0% v 0.6%, P < 0.0001). SCD + CDI were less likely to be associated with atrial fibrillation (4.9% v 15.1%, P < 0.0001), CHF (13.9% v 18.5%, P = 0.001) and hypothyroidism (3.7% v 9.4%, P < 0.0001). SCD + CDI had significantly higher complication rates including acute chest syndrome (4.4% v 1.8%, P < 0.0001), splenic sequestration (1.6% v 0.3%, P < 0.0001), and vaso-occlusive crises (2.7% v 1.4%, P < 0.0001). Though prematch inpatient mortality was not different between the two groups (5.9% v 6.5%, P = 0.43), SCD + CDI pts had significantly higher inpatient mortality than SCD − CDI pts after match (5.6% v 3.3%, P = 0.04). Over time, we noted increases in hospitalization-requirement and inpatient mortality for SCD + CDI pts (Tables 1 and 2). CONCLUSION: Ours is the largest study exploring the association of CDI in SCD patients. Hospitalization rates and mortality rates for hospitalized SCD pts with CDI has been rising in the United States and it seems that hospitalized SCD pts with CDI are more likely to develop SCD related complications and have a higher mortality. In SCD+CDI pts, clinicians should consider more aggressive initial treatment to minimize these poor outcomes.

Double heterozygosity for hemoglobin S and D Punjab in Basra, Iraq: A Clinical and hematological study of 42 patients

BACKGROUND: Patients with compound heterozygosity for sickle hemoglobin (HbS) and hemoglobin DPunjab (Hb SDPunjab) may present with a variable clinical course and may be indistinguishable from those with homozygous HbS. OBJECTIVES: The objective was to identify Hb SDPunjab phenotypes and the association of fetal Hb with disease severity. PATIENTS AND METHODS: This descriptive, cross-sectional study included 42 (17 males and 25 females) patients with double heterozygosity for HbS and D. In addition to full clinical data, the complete blood count and Hb quantitation were assessed. Statistical analyses were performed using SPSS software version 20.0. RESULTS: The mean age at diagnosis was 4.6 ± 0.7 years. Pallor and acute painful episodes (>90%) were the most common presenting symptoms, followed by jaundice (76.2%). Six patients (14.3%) had acute splenic sequestration crises and five (11.9%) had cardiac complications. Of the 42 studied patients, 17 (40.5%) had nonsevere disease and 25 (59.5%) had severe disease. Patients with severe disease had statistically significantly more blood transfusions (47.4 ± 10.27) than those with nonsevere disease (17.14 ± 1.82, P Conclusion: Double heterozygosity for Hb S and DPunjab in Basra results in SCD with a severe phenotype, and the Hb F level did not modulate the disease severity.

Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease.

Acute splenic sequestration crises are a complication of sickle cell disease, with high mortality rates and frequent recurrence in survivors of first attacks. Splenectomy and blood transfusion, with their consequences, are the mainstay of long-term management used in different parts of the world. This is a 2017 update of a Cochrane Review first published in 2002, and previously updated, most recently in 2015. To assess whether splenectomy (total or partial), to prevent acute splenic sequestration crises in people with sickle cell disease, improved survival and decreased morbidity in people with sickle cell disease, as compared with regular blood transfusions. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises of references identified from comprehensive electronic database searches and handsearching relevant journals and abstract books of conference proceedings. We also searched clinical trial registries. Additional trials were sought from the reference lists of the trials and reviews identified by the search strategy.Date of the most recent search: 14 August 2017. All randomized or quasi-randomized controlled trials comparing splenectomy (total or partial) to prevent recurrence of acute splenic sequestration crises with no treatment or blood transfusions in people with sickle cell disease. No trials of splenectomy for acute splenic sequestration were found. No trials of splenectomy for acute splenic sequestration were found. Splenectomy, if full, will prevent further sequestration and if partial, may reduce the recurrence of acute splenic sequestration crises. However, there is a lack of evidence from trials showing that splenectomy improves survival and decreases morbidity in people with sickle cell disease. There is a need for a well-designed, adequately-powered, randomized controlled trial to assess the benefits and risks of splenectomy compared to transfusion programmes, as a means of improving survival and decreasing mortality from acute splenic sequestration in people with sickle cell disease.There are no trials included in the review and we have not identified any relevant trials up to August 2017. We will continue to run searches to identify any potentially relevant trials; however, we do not plan to update other sections of the review until new trials are published.

Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease.

Acute splenic sequestration crises are a complication of sickle cell disease, with high mortality rates and frequent recurrence in survivors of first attacks. Splenectomy and blood transfusion, with their consequences, are the mainstay of long-term management used in different parts of the world. This is a 2015 update of a Cochrane review first published in 2002, and previously updated in 2013. To assess whether splenectomy (total or partial), to prevent acute splenic sequestration crises in people with sickle cell disease, improved survival and decreased morbidity in people with sickle cell disease, as compared with regular blood transfusions. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises of references identified from comprehensive electronic database searches and handsearching relevant journals and abstract books of conference proceedings.Additional trials were sought from the reference lists of the trials and reviews identified by the search strategy.Date of the most recent search: 10 June 2015. All randomized or quasi-randomized controlled trials comparing splenectomy (total or partial) to prevent recurrence of acute splenic sequestration crises with no treatment or blood transfusions in people with sickle cell disease. No trials of splenectomy for acute splenic sequestration were found. No trials of splenectomy for acute splenic sequestration were found. Splenectomy, if full, will prevent further sequestration and if partial, may reduce the recurrence of acute splenic sequestration crises. However, there is a lack of evidence from trials showing that splenectomy improves survival and decreases morbidity in people with sickle cell disease. There is a need for a well-designed, adequately-powered, randomized controlled trial to assess the benefits and risks of splenectomy compared to transfusion programmes, as a means of improving survival and decreasing mortality from acute splenic sequestration in people with sickle cell disease.There are no trials included in the review and we have not identified any relevant trials up to June 2015. We will continue to run searches to identify any potentially relevant trals; however, we do not plan to update other sections of the review until new trials are published.

Incidence of sickle cell disease and other hemoglobin variants in 10,095 Lebanese neonates.

Hemoglobinopathies are highly prevalent diseases and impose a public health burden. Early diagnosis and treatment can ameliorate the course of these diseases and improve survival. Despite purported high incidence of hemoglobinopathies in Lebanon, there are no nationwide screening programs. In this study, newborn screening utilizing high pressure liquid chromatography was executed in all public hospitals across Lebanon between 2010 and 2013. All newborns with an abnormal hemoglobin (Hb) were offered genetic counseling and all those with disease were enrolled in comprehensive hemoglobinopathy clinics. Among newborns, 2.1% were found to have an abnormal Hb variant with sickle Hb being the most common while 0.1% were found to have sickle cell disease (SCD). The majority of those with SCD had non-Lebanese origins. The most common causes of hospitalizations in infants with SCD were acute splenic sequestration and pain crises. No bacteremia or other life threatening infections were noted. At a median follow up 14 months (follow up range 7 to 34 months), all children with disease are alive and compliant with treatment. Systematic screening for SCD and other Hb variants was shown to be feasible, cost effective, and of accurate predictive value. This program was also clinically effective because it led to the identification of babies with disease and to providing them with free early multidisciplinary care. Conclusively, a newborn screening program should be implemented across Lebanon to detect hemoglobinopathies and initiate early therapeutic and preventive strategies and genetic counseling.

Acute splenic sequestration crisis in an adult with sickle cell anaemia

British Journal of Hospital MedicineVol. 73, No. 3 Case ReportAcute splenic sequestration crisis in an adult with sickle cell anaemiaAniket N Tavare, David Wrench, Nichola CooperAniket N TavareSearch for more papers by this author, David WrenchSearch for more papers by this author, Nichola CooperSearch for more papers by this authorAniket N Tavare; David Wrench; Nichola CooperPublished Online:16 Aug 2013https://doi.org/10.12968/hmed.2012.73.3.168AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References Koduri PR, Kovarik P (2006) Acute splenic sequestration crisis in an adult with sickle beta-thalassemia. Ann Hematol 85(9): 633–5 Crossref, Medline, Google ScholarKoduri PR, Nathan S (2006) Acute splenic sequestration crisis in adults with hemoglobin S-C disease: a report of nine cases. Ann Hematol 85(4): 239–43 Crossref, Medline, Google ScholarMichel JB, Hernandez JA, Buchanan GR (1992) A fatal case of acute splenic sequestration in a 53-year-old woman with sickle-hemoglobin C disease. Am J Med 92(1): 97–100 Crossref, Medline, Google ScholarMoll S, Orringer EP (1996) Case report: splenomegaly and splenic sequestration in an adult with sickle cell anemia. Am J Med Sci 312(6): 299–302 Crossref, Medline, Google ScholarOrringer EP, Fowler VG Jr., Owens CM et al. (1991) Case report: splenic infarction and acute splenic sequestration in adults with hemoglobin SC disease. Am J Med Sci 302(6): 374–9 Crossref, Medline, Google ScholarRivera-Ruiz M, Varon J, Sternbach GL (2008) Acute splenic sequestration in an adult with hemoglobin S-C disease. Am J Emerg Med 26 (9): 1064 e5–8 Crossref, Medline, Google ScholarSarma PS (1989) Acute splenic sequestration crisis in a young woman with homozygous sickle cell anaemia. Postgrad Med J 65(760): 105–7 Crossref, Medline, Google ScholarSolanki DL, Kletter GG, Castro O (1986) Acute splenic sequestration crises in adults with sickle cell disease. Am J Med 80(5): 985–90 Crossref, Medline, Google ScholarTopley JM, Rogers DW, Stevens MC, Serjeant GR (1981) Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. Arch Dis Child 56(10): 765–9 Crossref, Medline, Google ScholarWang-Gillam A, Lee RS, Hsi ED, Brotman DJ (2004) Acute splenic sequestration crisis resembling sepsis in an adult with hemoglobin SC disease. South Med J 97(4): 413–15 Crossref, Medline, Google Scholar FiguresReferencesRelatedDetails 1 March 2012Volume 73Issue 3ISSN (print): 1750-8460ISSN (online): 1759-7390 Metrics History Published online 16 August 2013 Published in print 1 March 2012 Information© MA Healthcare LimitedPDF download

Hydroxyurea Treatment of Young Children with Sickle Cell Anemia: Safety and Efficacy of Continued Treatment – the BABY HUG Follow-up Study

Abstract 7▪BABY HUG [Clinical Trials #NCT00006400], an NIH-NICHD sponsored randomized placebo-controlled trial showed that hydroxyurea (HU) administered to 9–18 month old children with sickle cell anemia (SCA) provides substantial clinical benefit. Benefits include a decrease in pain crises, acute chest syndrome events, need for transfusion and hospital admission; hematologic improvement include higher total and fetal hemoglobin concentration, larger red cell size, and lower WBC counts with toxicity limited to transient reduction in absolute neutrophil count (ANC) [Lancet 2011; 377:1663–72]. The parent or guardian of all 176 children who completed at least 18 months of randomized treatment were offered participation in an initial observational BABY HUG Follow-Up Study and 163 (93%) consented to participate. Clinical and laboratory data were collected every 6 months by structured abstraction of the medical record regarding use of clinically prescribed HU (dose escalation recommended), blood counts, clinical imaging, and sickle cell-related events. At the time of enrollment the family did not know their child’s randomized study treatment assignment; 133 (82%) initially chose clinical prescription of open-label HU. Acceptance of HU has remained high through 36 months of follow-up; during each 6 month data collection period 68–75% of participants reported having taken HU.Only 2 patients have left the study (due to relocation) and more than 93% of expected data have been collected. Preliminary analyses as of May 2011, including 417 patient years (pt-yrs) of follow up, demonstrate that in comparison to participants not taking HU, children who continue to take HU have statistically lower rates of pain crises requiring emergency department (ED) visits, episodic transfusions, and hospital admissions for any reason, including acute chest syndrome or febrile illness (see table). The substantial decrease in acute chest syndrome episodes is similar to the effect demonstrated with HU use in the randomized BABY HUG trial in younger infants and consistent with published trials detailing the benefit of HU therapy in older children and adults. The decrease in the rate of admission for febrile events in HU-treated patients is also comparable to that in the randomized trial, but the reason for this benefit is uncertain. There was no difference in hospitalization rates for painful events including dactylitis. Two patients in the non-HU group had a stroke. There were no differences between groups in the frequency of a palpable spleen or rate of acute splenic sequestration crises. Through 36 months of follow up children taking HU had persistently higher hemoglobin and MCV, and lower WBC and ANC than those not taking HU.Event Rate per 100 pt-yrsHUNo HUp valueED visit for Pain Crisis28.853.60.004Episodic Transfusion18.334.00.010Hospital Admission (any cause)74.9133.20.001Acute Chest Syndrome (admission)9.522.30.0001Febrile Illness (admission)30.764.3<.001Pain Crisis (admission)18.630.40.102Results of these analyses including growth and development assessments will enhance our understanding of the impact of HU use in children with SCA starting at a very young age. The accruing data from the BABY HUG Follow-Up Study demonstrate a continuation of the substantial benefits of early HU therapy with no discernable additional toxicities. Ongoing follow up of this cohort is essential to fully define these benefits as children grow, and to observe for late toxicity. Disclosures:Off Label Use: Hydroxyurea is not indicated for treatment of children with sickle cell disease. Use of this medication was for clinical indications and not mandated by this observational study.

Sickle Cell Disease

The specimen was received from a 13-year-old girl with Salmonella heidelberg bacteremia. A magnetic resonance imaging (MRI) revealed septic arthritis of the left shoulder and possible osteomyelitis of the left femur. After a month of therapy with antibiotics, she developed a large soft tissue abscesses over her left hip. An area identified by MRI as a small focus of osteomyelitis was curetted. The curettage specimen showed fragments of bone and bone marrow with slightly decreased but maturing hematopoiesis. There were areas with plasma cells and neutrophils associated with focal destruction of the bony spicules; these findings indicate acute and chronic osteomyelitis. In addition, there are numerous drepanocytes featuring elongated, tapered ends, characteristic of sickle cell disease (Figure 1). These findings were then correlated with a clinical history of hemoglobin SS and a sickle cell crisis in this patient. However, and especially in the absence of a similar clinical history, it is important to communicate to the clinician the presence of drepanocytes in tissue sections in order to intervene and stave off numerous potentially severe manifestations of the disease, including vaso-occlusion, acute splenic sequestration, and aplastic or hemolytic crises. Bone and joint disease, in particular septic arthritis of the hip, is a frequent complication in sickle cell anemia. The major differential diagnosis in evaluating a sickle cell patient with bone pain includes osteomyelitis and bone infarction. Because clinical presentations are similar, and imaging is of limited value, the histological diagnosis is paramount.

Therapeutic challenges in childhood sickle cell disease. Part 1: current and future treatment options.

Therapeutic challenges in childhood sickle cell disease. Part 1: current and future treatment options.

Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease.

Acute splenic sequestration crises are a complication of sickle cell disease, with high mortality rates and frequent recurrence in survivors of first attacks. Splenectomy and blood transfusion, with their consequences, are the mainstay of long term management used in different parts of the world. To assess whether splenectomy (total or partial) to prevent acute splenic sequestration crises in people with sickle cell disease improved survival and decreased morbidity in patients with sickle cell disease, as compared with regular blood transfusion. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references identified from comprehensive electronic database searches, hand searching relevant journals and hand searching abstract books of conference proceedings Additional randomized controlled trials were sought from the reference lists of the studies found and reviews identified by the search strategy. Date of the most recent search: December 2001 All randomized or quasi-randomized controlled trials comparing splenectomy (total or partial) to prevent recurrence of acute splenic sequestration crises to no treatment or blood transfusion in people with sickle cell disease. No trials of splenectomy for acute splenic sequestration were found. No trials of splenectomy for acute splenic sequestration were found. Splenectomy, if full, will prevent further sequestration and if partial, may reduce the recurrence of acute splenic sequestration crises but there is lack of evidence from trials that it improves survival and decreases morbidity in sickle cell disease. There is a need for a well-designed, adequately powered randomised controlled trial to assess the benefits and risks of splenectomy compared to transfusion programmes as a means of improving survival and decreasing mortality from ASS in people with sickle cell disease.

The Role of Splenectomy in Patients with Sickle Cell Disease

The Role of Splenectomy in Patients with Sickle Cell Disease

Splenectomy and acute splenic sequestration crises in sickle cell disease

Acute splenic sequestration crises (ASSC) is one of the complications of sickle cell disease (SCD) that can be life-threatening due to loss of blood volume. Over a 5-year period, we have treated 19 patients ranging in age from 4 to 32 years with ASSC. There were 14 males and 5 females; 17 had homozygous SCD and the other 2 had sickle thalassemia. Two patients presented with severe anemia and acute circulatory collapse; 1 of them developed residual weakness of his limbs and decreased visual acuity. Nine patients underwent splenectomy after major episodes of sequestration while the remaining 10 had recurrent minor episodes of sequestration. The clinical features and the role of splenectomy are discussed.

Case report: splenomegaly and splenic sequestration in an adult with sickle cell anemia.

Splenomegaly in adult patients with homozygous sickle cell anemia (HbSS) is uncommon and splenic sequestration crises are rare. This paper describes a patient with HbSS who, at the age of 24, began to experience acute splenic sequestration crises. These episodes occurred with sufficient frequency and severity to warrant splenectomy. This case is presented to emphasize that, although rare, splenomegaly can persist in adults with homozygous HbSS and can be associated with severe and even life-threatening splenic sequestration. The incidence of splenomegaly in adults with HbSS and the factors linked to it will be discussed and the published reports of splenic sequestration crises in this patient population reviewed. It appears that high hemoglobin F (HbF) levels and alpha-thalassemia may be important etiologic factors in causing persistence of splenomegaly and predisposing patients to splenic sequestration crises.

Acute splenic sequestration crises in Saudi children with sickle cell disease

Sickle cell disease (SCD) is a common disease in the Eastern Province of Saudi Arabia. Twenty per cent of the population have sickle cell trait and 1.75% have SCD. In the first 2 years of life, infection, hand-foot syndrome and acute splenic sequestration crisis (ASSC) are the most common complications of this disease. The classical characteristics of an ASSC, the subject of this paper, are sudden and rapid enlargement of the spleen, secondary to the massive pooling of red blood cells in the splenic sinusoids in a functioning spleen. Less common minor attacks of ASSC have been described recently. An ASSC is one of the most common causes of death in infants with SCD. The underlying cause and the precipitants of attacks of ASSC remain unknown. Seventeen children with ASSC were seen. Clinically, they had minor ASSC; three developed hypersplenism and four underwent splenectomy for recurrent attacks of ASSC. None of these 17 children had the classical ASSC described in black children where peripheral circulatory shock is encountered.

Acute splenic sequestration crises in adults with sickle cell disease

Reports of acute splenic sequestration crises in adults with sickle cell hemoglobin C disease or sickle cell thalassemia are rare, although an enlarged and distensible spleen persists in half of these patients. Seven episodes of acute splenic sequestration crises in four adults, two with sickle C disease and two with sickle thalassemia, are described. The crises were life-threatening and recurrent in all, but there were no fatalities. One patient had mild steady-state thrombocytopenia suggesting hypersplenism. Technetium 99m/sulfur colloid scanning of the spleen during the acute splenic sequestration crises in three patients showed almost total lack of splenic uptake or decreased uptake with intrasplenic filling defects thought to be splenic infarcts or hematomas on follow-up computed tomographic scanning. The scanning abnormalities resolved following recovery from the crises. Acute splenic sequestration crises probably are common in adults with sickle C disease and sickle thalassemia but may be underdiagnosed or misdiagnosed as splenic infarctions. The hematologic and splenic findings during acute splenic sequestration crises resemble those following splenic vein ligation in animals.

Acute splenic sequestration crises (ASSC) in young children with sickle cell anemia. Clinical observations in 20 episodes in 14 children.

Acute splenic sequestration crises (ASSC) is a potentially life-threatening com plication in young children with sickle cell anemia. The clinical picture includes huge splenomegaly accompanied by signs and symptoms of acute circulatory insufficiency caused by profound anemia. The authors describe 20 such episodes in 14 children aged six to 55 months. Rapid transfusion of packed erythrocytes produced rapid and dramatic improvement. The spleens became nonpalpable in seven to ten days following transfusion. Four children died, four had one or more recurrences, four have splenic involution, and two were splenectomized. The recurrences were within four months of the previous ASSC. Our current recommendation is to consider splenectomy only after at least two ASSCs.