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1.  Autosplenectomy of Sickle Cell Disease in Zaria, Nigeria: An Ultrasonographic Assessment 
Oman Medical Journal  2012;27(2):121-123.
During infancy and early childhood, the spleen commonly enlarges in patients with sickle cell anemia (SCA), and it thereafter undergoes progressive atrophy due to repeated episodes of vaso-occlusion and infarction, leading to autosplenectomy in adult life. However, this may not always be the case as some studies have reported splenomegaly persisting into adult life. This study aims to determine and review the prevalence of autosplenectomy by abdominal ultrasonography in sickle cell anemic patients in Zaria, Nigeria.
An ex-post-facto cross study of 74 subjects was carried out between May to July in 2010. Hematological parameters were determined by an analyzer while B mode Ultrasonography was used to determine the craniocaudal length of the spleen, if visualized.
The mean age of the sickle cell subjects was 23.2 ±5.3 years, while that of the controls was 22.7±12.4 years. Of the 74 sickle cell subjects, 55.4% were females; while of the 20 controls, 50% were females. Forty one subjects (55.4%) had autosplenectomy and a significant difference existed in the mean splenic size compared with the control (p<0.0001). Only 3 (4.05%) subjects had splenomegaly, while 23 (31%) had a shrunken spleen.
Anatomical autosplenectomy is not an uncommon finding in SCA patients. This may be related to inadequate clinical care due to the lack of good health education, ignorance, poverty, and poor standard of care, as well as the lack of newer therapeutic agents.
PMCID: PMC3321339  PMID: 22496936
Sickle cell anemia; Autosplenectomy; Ultrasonography
2.  Elevated IL-1α and CXCL10 serum levels occur in patients with Homozygous Sickle Cell Disease and a history of Acute Splenic Sequestration 
Disease Markers  2012;32(5):295-300.
Acute splenic sequestration (ASS) and chronic hypersplenism are common features of homozygous sickle cell (SS) disease in the first 5 years of life affecting one-third of subjects in the Jamaican Cohort Study. The risk factors are largely unknown and the current study explores a possible role of genetic factors. We have explored these in subjects who received splenectomy in the management of ASS (n=8) or chronic hypersplenism (n=9) along with age, gender, and genotype matched controls using Luminex Technology to assess 42 human cytokines/chemokines, including IL-1α and CXCL10 (IP-10). Levels of IL-1α (p=0.008) and CXCL10 (p=0.009) were significantly elevated in patients treated by splenectomy compared with the control group. Levels of IL-1α were significantly higher in those with a history of ASS compared with matched normal controls (p= 0.028) but not in those treated for hypersplenism (p=0.093). Furthermore, several significant differences were found in the median ratios of some cytokine biomarkers between the splenectomized group and the normal controls. These observations are consistent with acute splenic sequestration having a distinct phenotype which may be helpful in predicting those at risk of this complication and suggest that the mechanism of these differences merit further study.
PMCID: PMC3375690  PMID: 22674409
Sickle cell anemia; biomarkers; splenectomy; spleen; interleukins; cytokine; acute splenic sequestration; chronic hypersplenism
3.  Elevated IL-1α and CXCL10 Serum Levels Occur in Patients with Homozygous Sickle Cell Disease and a History of Acute Splenic Sequestration 
Disease markers  2012;32(5):295-300.
Acute splenic sequestration (ASS) and chronic hypersplenism are common features of homozygous sickle cell (SS) disease in the first 5 years of life affecting one-third of subjects in the Jamaican Cohort Study. The risk factors are largely unknown and the current study explores a possible role of genetic factors. We have explored these in subjects who received splenectomy in the management of ASS (n=8) or chronic hypersplenism (n=9) along with age, gender, and genotype matched controls using Luminex Technology to assess 42 human cytokines/chemokines, including IL-1α and CXCL10 (IP-10). Levels of IL-1α (p=0.008) and CXCL10 (p=0.009) were significantly elevated in patients treated by splenectomy compared with the control group. Levels of IL-1α were significantly higher in those with a history of ASS compared with matched normal controls (p=0.028) but not in those treated for hypersplenism (p=0.093). Furthermore, several significant differences were found in the median ratios of some cytokine biomarkers between the splenectomized group and the normal controls. These observations are consistent with acute splenic sequestration having a distinct phenotype which may be helpful in predicting those at risk of this complication and suggest that the mechanism of these differences merit further study.
PMCID: PMC3375690  PMID: 22674409
Sickle cell anemia; biomarkers; splenectomy; spleen; interleukins; cytokine; acute splenic sequestration; chronic hypersplenism
4.  Global Burden of Sickle Cell Anaemia in Children under Five, 2010–2050: Modelling Based on Demographics, Excess Mortality, and Interventions 
PLoS Medicine  2013;10(7):e1001484.
Frédéric Piel and colleagues combine national sickle cell anemia (SCA) frequencies with projected demographic data to estimate the number of SCA births in children under five globally from 2010 to 2050, and then estimate the number of lives that could be be saved following implementation of specific health interventions starting in 2015.
Please see later in the article for the Editors' Summary
The global burden of sickle cell anaemia (SCA) is set to rise as a consequence of improved survival in high-prevalence low- and middle-income countries and population migration to higher-income countries. The host of quantitative evidence documenting these changes has not been assembled at the global level. The purpose of this study is to estimate trends in the future number of newborns with SCA and the number of lives that could be saved in under-five children with SCA by the implementation of different levels of health interventions.
Methods and Findings
First, we calculated projected numbers of newborns with SCA for each 5-y interval between 2010 and 2050 by combining estimates of national SCA frequencies with projected demographic data. We then accounted for under-five mortality (U5m) projections and tested different levels of excess mortality for children with SCA, reflecting the benefits of implementing specific health interventions for under-five patients in 2015, to assess the number of lives that could be saved with appropriate health care services. The estimated number of newborns with SCA globally will increase from 305,800 (confidence interval [CI]: 238,400–398,800) in 2010 to 404,200 (CI: 242,500–657,600) in 2050. It is likely that Nigeria (2010: 91,000 newborns with SCA [CI: 77,900–106,100]; 2050: 140,800 [CI: 95,500–200,600]) and the Democratic Republic of the Congo (2010: 39,700 [CI: 32,600–48,800]; 2050: 44,700 [CI: 27,100–70,500]) will remain the countries most in need of policies for the prevention and management of SCA. We predict a decrease in the annual number of newborns with SCA in India (2010: 44,400 [CI: 33,700–59,100]; 2050: 33,900 [CI: 15,900–64,700]). The implementation of basic health interventions (e.g., prenatal diagnosis, penicillin prophylaxis, and vaccination) for SCA in 2015, leading to significant reductions in excess mortality among under-five children with SCA, could, by 2050, prolong the lives of 5,302,900 [CI: 3,174,800–6,699,100] newborns with SCA. Similarly, large-scale universal screening could save the lives of up to 9,806,000 (CI: 6,745,800–14,232,700) newborns with SCA globally, 85% (CI: 81%–88%) of whom will be born in sub-Saharan Africa. The study findings are limited by the uncertainty in the estimates and the assumptions around mortality reductions associated with interventions.
Our quantitative approach confirms that the global burden of SCA is increasing, and highlights the need to develop specific national policies for appropriate public health planning, particularly in low- and middle-income countries. Further empirical collaborative epidemiological studies are vital to assess current and future health care needs, especially in Nigeria, the Democratic Republic of the Congo, and India.
Please see later in the article for the Editors' Summary
Editors' Summary
More than seven million babies are born each year with a structural or functional abnormality. Although some birth defects are caused by environmental factors, many are caused by the inheritance of a defective gene. One common inherited birth defect is sickle cell anemia (SCA). SCA arises when a baby inherits the gene for sickle hemoglobin (HbS), a structural variant of normal adult hemoglobin (HbA, the protein in the disc-shaped red blood cells that carry oxygen round the body), from both its parents. Every cell in the human body contains two full sets of genes, and babies inherit one set of genes from each parent. The parents usually each have one HbS gene and one HbA gene, and are unaffected. However, the red blood cells of their offspring who inherit two copies of HbS develop a sickle (crescent) shape. Sickle cells can block blood vessels in the limbs and organs and have a shorter lifespan than normal red blood cells, which causes anemia. Together, these changes can cause acute pain and organ damage, and can increase the risk of severe infections. SCA can be prevented by prenatal diagnosis and managed by interventions such as the provision of antibiotics and vaccination to prevent infections.
Why Was This Study Done?
Without early diagnosis and treatment, children with SCA often die within the first few years of life. Having one copy of the HbS gene provides people with protection from malaria, therefore SCA occurs mainly in low- and middle-income countries in tropical regions, where early diagnosis and treatment is often unavailable. Recent improvements in overall infant and childhood survival in these countries and population migration to higher-income countries mean that the global burden of SCA is likely to increase over the coming decades. To date, no one has tried to quantify this increase, although this information is needed to guide decisions on public health spending. In this modeling study, the researchers assess the size of the expected global burden of SCA between 2010 and 2050 in children under five years old and estimate the number of newborn lives that might be saved by implementation of various health interventions.
What Did the Researchers Do and Find?
The researchers used estimates of national SCA frequencies and data on projected birth rates to calculate that the number of newborns with SCA will increase from about 305,800 in 2010 to about 404,200 in 2050. They estimated that Nigeria, the Democratic Republic of Congo (DRC), and India accounted for 57% of newborns with SCA in 2010, and that Nigeria and the DRC will probably still be the countries most in need of policies for the prevention and management of SCA in 2050. The researchers then assessed how many newborns might be saved by the implementation of various health measures in 2015 that affect excess mortality (the difference between the frequency of SCA in newborns and in five-year-olds divided by the frequency of SCA in newborns) among children born with SCA. Implementation of prenatal diagnosis and newborn screening programs, and provision of antibiotics and vaccinations (interventions assumed by the researchers to reduce excess mortality from 90% to 50% in low- and middle-income countries and from 10% to 5% in high-income countries) could prolong the life of more than five million newborns with SCA by 2050. Implementation of universal screening and provision of other specific measures predicted to reduce excess mortality to 5% and 0% in low-to-middle-income countries and high-income countries, respectively, could save nearly ten million lives by 2050.
What Do These Findings Mean?
In estimating the global burden of SCA in children under five years old between 2010 and 2050 and the number of newborn lives that could be saved by implementation of health interventions, the researchers made numerous assumptions reflected in the uncertainty associated with the projections. For example, they assumed that implementation of specific interventions would lead to an immediate reduction of excess mortality in newborns with SCA. The study's findings confirm, however, that the global burden of SCA is increasing and indicate that the implementation of specific interventions could extend the lives of millions of newborns with SCA. Although further studies are needed to assess the current and future health care needs of children with SCA, these findings highlight the need to develop and implement national public health planning and funding policies for SCA, particularly in low- and middle-income countries.
Additional Information
Please access these websites via the online version of this summary at
This study is further discussed in a PLOS Medicine Perspective by Edward Fottrell and David Osrin
The US National Heart, Lung, and Blood Institute provides detailed information (including personal stories) about sickle cell anemia (in English and Spanish)
The UK National Health Service Choices website also provides detailed information and a personal story about sickle cell anemia
The Sickle Cell Society, a UK-based not-for-profit organization, provides information for patients and carers and includes a children's website
The World Health Organization has a factsheet on sickle cell anemia and other inherited hemoglobin diseases (in several languages)
MedlinePlus provides links to further resources about sickle cell anemia (in English and Spanish)
The Malaria Atlas Project provides epidemiological information on the inherited blood disorders (including sickle cell anemia) that affect our response to malaria infection
The Global Sickle Cell Disease Network is a portal bringing together leading sickle cell disease researchers and clinicians from high-, middle-, and low-income countries to form a network
PMCID: PMC3712914  PMID: 23874164
5.  Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. 
Archives of Disease in Childhood  1981;56(10):765-769.
A cord blood screening programme initiated in June 1973 had screened 68 000 normal deliveries by February 1979 with the detection of 216 cases of homozygous sickle cell disease. Regular review of these children in the Medical Research Council paediatric clinic has identified acute splenic sequestration as a major cause of morbidity and mortality in the first 5 years of life. In addition to classical episodes characterised by peripheral circulatory failure, minor episodes of increasing anaemia associated with an enlarging spleen and an active marrow were also common. These minor episodes appeared to have predictive value in children who later developed severe life-threatening episodes of acute splenic sequestration. Sequestration. Sustained hypersplenism was also appreciably more common in children developing minor or major episodes of acute splenic sequestration compared with those without such a history. It is proposed that the classification of acute splenic sequestration be expanded to include these minor episodes, and that consideration be given to prevention of recurrences by splenectomy particularly in patients who also develop sustained hypersplenism.
PMCID: PMC1627327  PMID: 7305414
6.  Perioperative Management in Children with Sickle Cell Disease Undergoing Laparoscopic Surgery 
The aim of this study was to evaluate our experience with laparoscopic surgery in children with sickle cell disease.
A retrospective chart review was performed to analyze the indication for surgery, perioperative management, surgical technique, complications, duration of hospitalization, and outcome. One pediatric surgeon performed all procedures.
Thirteen children underwent laparoscopic surgery for the following indications: symptomatic cholelithiasis/cholecystitis in 9; recurrent splenic sequestration in 3; and hypersplenism/symptomatic cholelithiasis in 1. The 7 boys and 6 girls had a median age of 7.8 years. Patients undergoing splenectomy only were younger than those undergoing cholecystectomy (median age, 3.6 years versus 11.5 years, respectively). Four children underwent endoscopic retrograde cholangiopancreatography (ERCP) and sphincterotomy because of common bile duct dilatation and stones. Twelve patients received packed red blood cell transfusions prior to surgery. The median operative time was 150 minutes, and the median hospitalization was 3 days. Four patients suffered postoperative complications (2 with acute chest syndrome, 1 with recurrent abdominal pain, and 1 with priapism). The patient with abdominal pain was found to have a retained stone in the common bile duct, which was retrieved via endoscopic retrograde cholangiopancreatography and sphincterotomy. All complications resolved with medical management.
Laparoscopic surgery is safe in children with sickle cell disease. Meticulous attention to perioperative management, transfusion guidelines, and pulmonary care may decrease the incidence of acute chest syndrome.
PMCID: PMC3043404  PMID: 12002293
Sickle cell anemia; Laparoscopic surgery; Child; Adolescence
7.  Partial splenectomy in sickle cell syndromes. 
Archives of Disease in Childhood  1991;66(9):1070-1072.
Partial splenectomy was carried out in four children with homozygous sickle cell disease and eight children with sickle cell beta thalassaemia. It was performed in order to preserve splenic contribution to the host defence against infections while suppressing hypersplenism or the risk of recurrence of acute splenic sequestration. Indications for this surgical operation were acute splenic sequestration (n = 1), hypersplenism (n = 5), and acute splenic sequestration and hypersplenism (n = 6). Surgery was uneventful in 11 patients. A significant reduction of blood requirements and a significant decrease of the number of hospitalisations/patient/year were observed after splenectomy. No recurrence of hypersplenism or acute splenic sequestration occurred and no severe infection was noticed during the follow up period after surgery (mean (SD) 4.2 (2.8) years; range 6 months-7 years). Mean haemoglobin concentration and leucocyte and platelet counts increased after surgery. The benefit of partial splenectomy compared with total splenectomy to treat acute splenic sequestration or hypersplenism in sickle cell disease is discussed.
PMCID: PMC1793058  PMID: 1929516
8.  A Unique Cause of Intestinal and Splenic Infarction in a Sickle Cell Trait Patient 
Case Reports in Surgery  2013;2013:580453.
Sickle-cell trait is a common genetic abnormality in the African American population. A sickle-cell crisis in a patient with sickle-cell trait is uncommon at best. Abdominal painful crises are typical of patients with sickle cell anemia. The treatment for an abdominal painful crisis is usually medical and rarely surgical. We present the case of a cocaine-induced sickle-cell crisis in a sickle-cell trait patient that resulted in splenic, intestinal, and cerebral infarctions and multisystem organ failure necessitating a splenectomy, subtotal colectomy, and small bowel resection. This case highlights the diagnostic dilemma that abdominal pain can present in the sickle-cell population and illustrates the importance of recognizing the potential for traditionally medically managed illnesses to become surgical emergencies.
PMCID: PMC3662113  PMID: 23738181
9.  Splenic lymphoma with massive splenomegaly: Case report with review of literature 
As per strict criteria of Das Gupta et al, primary splenic lymphoma is very rare. Herein, we are reporting an unusual case of primary large cell splenic lymphoma of B lineage in a middle aged female presenting with massive splenomegaly (3.8 kg) and hypersplenism. After performing therapeutic splenectomy for hypersplenism, a precise diagnosis of diffuse large B cell lymphoma was made on histopathology and confirmed by immunohistochemistry. The patient responded well to standard (Cyclophosphamide, Hydroxydaunorubicin, Oncovin (vincristine), Prednisone or prednisolone) regimen last year and is now in full remission. The splenectomy thereby has prevented the potential grave complications related to hypersplenism and splenic rupture. Our aim behind highlighting the topic is to specify that emergency splenectomy followed by anticoagulation therapy is an effective plan of management to prevent untoward complications related to disease and treatment.
PMCID: PMC4163774  PMID: 25232555
Huge splenic lymphoma; Pancytopenia; Splenectomy; Anticoagulation
10.  Laparoscopic Splenectomy in Colorectal Cancer Patients with Chemotherapy-Associated Thrombocytopenia due to Hypersplenism 
Case Reports in Oncology  2012;5(3):601-607.
Hypersplenism due to chemotherapy-related liver injury has been associated with severe thrombocytopenia that may preclude continuation of systemic therapy for cancer patients. Patients treated for metastatic colorectal cancer (mCRC) are among the most common patients affected by hypersplenism. Cessation of systemic therapy invariably leads to progression of disease. While partial splenic embolization has been employed successfully to reverse the effects of hypersplenism, the role of laparoscopic splenectomy for this problem has not been completely defined.
A retrospective review was conducted of mCRC patients treated with laparoscopic splenectomy at our institution to reverse severe thrombocytopenia due to chemotherapy-related hypersplenism. An endpoint assessed was the ability to resume therapy after splenectomy.
Six patients with mCRC and hypersplenism requiring cessation of systemic therapy underwent laparoscopic splenectomy. All (6) patients had a postsurgical platelet count >150 × 103/μl and resumed chemotherapy after surgery. Median platelet count prior to surgery was 66 × 103/μl, and just prior to resuming systemic therapy it was 399.5 × 103/μl. Median spleen size was 14.0 cm. There were no surgical complications. Mean hospital stay was 2.8 days and the median time from surgery to resumption of therapy was 23.5 days.
Laparoscopic splenectomy appears to offer selected patients with mCRC the opportunity to resume systemic therapy that otherwise would be discontinued due to thrombocytopenia from hypersplenism.
PMCID: PMC3531924  PMID: 23275773
Laparoscopic surgery; Splenectomy; Hypersplenism; Splenomegaly; Thrombocytopenia; Colorectal cancer; Neoplasm metastasis
11.  Effects of Chronic Transfusions on Abdominal Sonographic Abnormalities in Children with Sickle Cell Anemia 
The Journal of Pediatrics  2011;160(2):281-285.e1.
To assess the effects of chronic erythrocyte transfusions on prevalence of sonographic incidence of organ damage in children with sickle cell anemia (SCA).
Study design
Children (n=148; mean age, 13.0 years) with SCA, receiving chronic transfusions (average, 7 years) underwent abdominal sonography at 25 institutions. After central imaging review, spleen, liver and kidney measurements were compared with published normal values. Potential relations between ultrasound, clinical and laboratory data were explored via Analysis of Variance, Student t-test and Cochran Mantel Haenzel tests of non-zero correlation.
Average spleen length was similar to normal children, but over one-third had spleen volumes > 300mL, 15 had previous splenectomy for splenomegaly and 24 had abnormal splenic echotexture. Two-thirds had hepatobiliary disease; 37 had prior cholecystectomy, 46 had gallstones, 16 had gallbladder sludge. Gallbladder disease correlated with older age (p = 0.002), longer liver length (p < 0.001), longer duration of transfusions (p = 0.034) and higher total bilirubin (p < 0.001). Liver (p < 0.001) and renal lengths (p ≤ 0.005) were larger than published norms.
In children with SCA, long-term transfusion therapy may not prevent development or progression of abdominal organ dysfunction.
PMCID: PMC3237893  PMID: 21907352
sickle cell anemia; iron overload; splenomegaly; gallstones; hepatomegaly; nephromegaly
12.  Life-threatening hypersplenism due to idiopathic portal hypertension in early childhood: case report and review of the literature 
BMC Gastroenterology  2010;10:122.
Idiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia. This study evaluates the pathogenic concept of the disease by a systematic review of the literature and illustrates novel pathologic and laboratory findings.
Case Presentation
We report the first case of uncontrolled splenic hyperperfusion and enlargement with subsequent hypersplenism leading to life-threatening complications of IPH in infancy and emergent splenectomy.
Our results suggest that splenic NO and VCAM-1, rather than ET-1, have a significant impact on the development of IPH, even at a very early stage of disease. The success of surgical interventions targeting the splenic hyperperfusion suggests that the primary defect in the regulation of splenic blood flow seems to be crucial for the development of IPH. Thus, beside other treatment options splenectomy needs to be considered as a prime therapeutic option for IPH.
PMCID: PMC2988068  PMID: 20961440
13.  Laparoscopic Splenectomy in Pediatric Patients with Hematologic Diseases 
The aim of this study was to evaluate our experience with laparoscopic splenectomy in pediatric patients with hematologic diseases.
A retrospective chart review was performed to analyze the following: indication for splenectomy, pre- and peri-operative management, surgical technique, complications, duration of hospitalization, and outcome.
Eleven patients underwent laparoscopic splenectomy for the following indications: recurrent thrombocytopenia (<10,000) in seven with chronic immune thrombocytopenic purpura; anemia in two with hereditary spherocytosis; and hypersplenism in one and recurrent splenic sequestration in another with homozygous hemoglobin S. The six girls and five boys had a median age of 7 years. The median operative time was 180 minutes, and the median hospitalization was 72 hours. Among the patients with immune thrombocytopenic purpura (median platelet count, 153,000), those patients (n=3) with platelet counts of <100,000 did not suffer any bleeding complications. The patient with hypersplenism and homozygous hemoglobin S required a small incision in the left lower quadrant to facilitate removal of a 558-gram spleen. This patient also underwent cholecystectomy for cholelithiasis. The operative time was 295 minutes, and he was hospitalized for 5 days because of atelectasis.
Laparoscopic splenectomy is a safe and effective procedure in children with hematological disorders.
PMCID: PMC3015371  PMID: 10917117
Hematologic diseases; Splenectomy; Laparoscopic surgery; Child; Adolescence
14.  Preeminence of Lesser Splanchnic Blood Flow in Selected Patients With Generalized Portal Hypertension 
HPB Surgery  1990;2(4):233-251.
Although restricted transhepatic portal flow is necessary for development of generalized portal hypertension (GPH), increased splanchnic arterial inflow also contributes to GPH and its clinical sequelae. In this context, we describe 7 male and 6 female patients (mean age 48 years) in whom the lesser splanchnic (gastrosplenic) system played a key role in the signs and symptoms of GPH. These 13 patients (9 with hepatic cirrhosis, 3 with primary myeloproliferative disorder, and 1 with extrahepatic portal block) shared common features of massive splenomegaly, huge splenofundic gastric varices, often with a prominent natural shunt to the left renal vein. Total or near total splenectomy alone or combined where appropriate with coronary vein ligation was effective in controlling varix hemorrhage (10 patients), ascites (3), or complications of an enlarged spleen-anorexia and abdominal pain (3), hemolytic anemia (1) and profound thrombocytopenia with severe epistaxis (1). Intraoperative jejunal portal venography was crucial in operative management in order to establish definitively the presence or absence of coronary venous collaterals, and when present, to verify their operative ligation.
These distinctive patients illustrate: 1) GPH is a heterogeneous syndrome of divergent splanchnic circulatory patterns, a feature which should be taken into account in selecting operative treatment; 2) one well-defined subgroup displays prominent hyperdynamic lesser splanchnic and specifically, splenic blood flow as a major contributor to clinical complications; and 3) within this subgroup, splenectomy combined with documented absence or surgical interruption of coronary venous collaterals as corroborated by intraoperative portography is effective alternative treatment.
PMCID: PMC2423585  PMID: 2278922
15.  Fetal haemoglobin and early manifestations of homozygous sickle cell disease. 
Archives of Disease in Childhood  1992;67(4):517-520.
The relevance of fetal haemoglobin (HbF) concentration to the development of early clinical manifestations of homozygous sickle (SS) disease has been investigated by examining the time to first occurrence and the proportional hazard of these complications in three groups of the HbF distribution at age 5 years. HbF was significantly related to dactylitis, painful crises, acute chest syndrome, and acute splenic sequestration. The relationship suggested that a critically low HbF concentration increased the risk, little difference in risk occurring between the medium and high HbF groups. The abdominal painful crisis and hypersplenism were not related to HbF concentration suggesting that the degree of sickling may not be important in their genesis. Parental education on acute splenic sequestration should be focused on children with HbF concentrations in the lowest part of the HbF distribution for age.
PMCID: PMC1793326  PMID: 1374603
16.  Splenic injuries at Bugando Medical Centre in northwestern Tanzania: a tertiary hospital experience 
BMC Research Notes  2012;5:59.
Splenic injuries constitute a continuing diagnostic and therapeutic challenge to the trauma or general surgeons practicing in developing countries where sophisticated imaging facilities are either not available or exorbitantly expensive. The purpose of this review was to describe our own experience in the management of the splenic injuries outlining the aetiological spectrum, injury characteristics and treatment outcome of splenic injuries in our local environment and to identify predictors of outcome among these patients.
A prospective descriptive study of splenic injury patients was carried out at Bugando Medical Centre in Northwestern Tanzania between March 2009 and February 2011. Statistical data analysis was done using SPSS software version 17.0.
A total of 118 patients were studied. The male to female ratio was 6.4:1. Their ages ranged from 8 to 74 years with a median age of 22 years. The modal age group was 21-30 years. The majority of patients (89.8%) had blunt trauma and road traffic accidents (63.6%) were the most frequent cause of injuries. Most patients sustained grade III (39.0%) and IV (38.1%) splenic injuries. Majority of patients (86.4%) were treated operatively with splenectomy (97.1%) being the most frequently performed procedure. Postoperative complications were recorded in 30.5% of cases. The overall length of hospital stay (LOS) ranged from 1 day to 120 days with a median of 18 days. Mortality rate was 19.5%. Patients who had severe trauma (Kampala Trauma Score II ≤ 6) and those with associated injuries stayed longer in the hospital (P < 0.001), whereas age of the patient, associated injuries, trauma scores (KTS II), grade of splenic injuries, admission systolic blood pressure ≤ 90 mmHg, estimated blood loss > 2000 mls, HIV infection with CD4 ≤ 200 cells/μl and presence of postoperative complications were significantly associated with mortality (P < 0.001).
Trauma resulting from road traffic accidents (RTAs) remains the most common cause of splenic injuries in our setting. Most of the splenic injuries were Grade III & IV and splenectomy was performed in majority of the cases. Non-operative management can be adopted in patients with blunt isolated and low grade splenic injuries but operative management is still indispensable in this part of Tanzania. Urgent preventive measures targeting at reducing the occurrence of RTAs is necessary to reduce the incidence of splenic injuries in our centre.
PMCID: PMC3274421  PMID: 22269803
Splenic injuries; Aetiological spectrum; Injury characteristics; Treatment outcome; Predictors of outcome; Tanzania
17.  Selective splenic artery embolization for the treatment of thrombocytopenia and hypersplenism in paroxysmal nocturnal hemoglobinuria 
PNH is associated with abdominal vein thrombosis, which can cause splenomegaly and hypersplenism. The combination of thrombosis, splenomegaly, and thrombocytopenia (TST) is challenging because anticoagulants are indicated but thrombocytopenia may increase the bleeding risk. Splenectomy could alleviate thrombocytopenia and reduce portal pressure, but it can cause post-operative thromboses and opportunistic infections. We therefore sought to determine whether selective splenic artery embolization (SSAE) is a safe and effective alternative to splenectomy for TST in patients with PNH.
Four patients with PNH and TST received successive rounds of SSAE. By targeting distal vessels for occlusion, we aimed to infarct approximately 1/3 of the spleen with each procedure.
Three of 4 patients had an improvement in their platelet count, and 3 of 3 had major improvement in abdominal pain/discomfort. The one patient whose platelet count did not respond had developed marrow failure, and she did well with an allo-SCT. Post-procedure pain and fever were common and manageable; only one patient developed a loculated pleural effusion requiring drainage. One patient, who had had only a partial response to eculizumab, responded to SSAE not only with an improved platelet count, but also with an increase in hemoglobin level and decreased transfusion requirement.
These data indicate that SSAE can decrease spleen size and reverse hypersplenism, without exposing the patient to the complications of splenectomy. In addition, SSAE probably reduces the uptake of opsonised red cells in patients who have had a limited response to eculizumab, resulting in an improved quality of life for selected patients.
PMCID: PMC3984395  PMID: 24673826
PNH; Thrombosis; Selective Splenic Artery embolization; Hypersplenism
18.  Enlarged Spleen Syndrome 
Enlarged spleen without a clear-cut etiology, and believed to be related to malarial infestations, has been referred to as “tropical splenomegaly” and “cryptogenetic splenomegaly.” Splenectomy performed in such cases after a failure of antimalarial therapy shows histopathologically, while some of these meet the criteria for tropical splenomegaly, that most were cases of splenic abscess in various stages of formation, and some were lymphosarcoma. In sickle cell disease, one sequence of events involving the spleen is intermittent painful splenic infarction leading to splenic abscess or shrunken, fibrotic, and probably functionless spleen. It is proposed that an elective splenectomy may be indicated in cases of sickle cell disease to forestall this outcome. It is also proposed that an enlarged spleen that fails to respond to antimalarial medication after a certain period should be removed to establish the correct diagnosis, to prevent rupture, and to determine definitive management.
PMCID: PMC2552414  PMID: 7365820
19.  Spectrum of musculo-skeletal disorders in sickle cell disease in Lagos, Nigeria 
Sickle cell anemia (SCA) is a common genetic disease in Nigeria. Past studies from West Africa focused on isolated aspects of its medical and surgical presentations. To the best of our knowledge, the musculo-skeletal presentations amongst Nigerians with SCA have not been documented in a single all encompassing study. This work aims to prospectively document the musculo-skeletal disease burden among SCA patients.
In a prospective study of 318 consecutive patients with genotype-confirmed SCA at the Lagos University Teaching Hospital (LUTH), the musculo-skeletal pathologies, anatomic sites, grade of disease, age at presentation and management outcome were recorded over a one-year period. Data obtained were analyzed using Epi-Info software version 6.0. Data are presented as frequencies (%) and mean values (SD) as appropriate.
The HbSS genotype occurred in 296 (93.0%), while 22 (7.0%) were HbSC. 100 (31.4%) patients with average presenting haemoglobin concentration of 8.2 g/100 ml in the study group, presented with 131 musculo-skeletal pathologies in 118 anatomic sites. Osteomyelitis 31 (31%) and septic arthritis 19 (19%) were most commonly observed in children less than 10 years. Skin ulcers and avascular necrosis (AVN) occurred predominantly in the older age groups, with frequencies of 13 (13.0%) and 26 (26.0%) respectively. 20 (71.5%) of diagnosed cases of AVN presented with radiological grade 4 disease. The lower limbs were involved in 84 (71.1%) of sites affected. Lesions involving the spine were rare 11 (0.9%). Multiple presentations occurred in 89 (28.0%) of patients; 62 (69.7%) of which were children below 10 years.
Musculo-skeletal complications are common features of sickle cell anaemia seen in 31.4%. Infectious aetiologies predominate with long bones and joints of lower limbs more commonly affected by osteomyelitis and septic arthritis. Healthcare providers managing SCA should be aware of the potential morbidity and mortality of these conditions to ensure early diagnosis and adequate management.
PMCID: PMC2821293  PMID: 20157420
20.  Hearing thresholds in sickle cell anemia patients: emerging new trends? 
PURPOSE OF STUDY: Advances in medicine resulting in better understanding of sickle cell disease and general improvement of the well-being of the sufferers even in the developing countries have positively affected the dreadful outlook of this disease with resultant increase in the population of sickle cell disease patients reaching adulthood, and less severe complications. We therefore set out to evaluate the presence and severity of sensorineural hearing loss in sickle cell anemia (SCA) patients in the light of the overall improvement in the morbidity and mortality. METHODS: A prospective case control study of SCA patients attending our adult SCA clinic and control subjects from homozygous hemoglobin AA patients attending the staff clinic of the hospital for routine medical tests. Tympanometry and diagnostic audiometry were performed on each patient. MAIN FINDINGS: Forty-six SCA patients (21 males, 45.7%) aged 16-48 years with a mean age of 22.9 years +/- 6.45 and 42 controls (24 males, 57.1%) aged 15-39 years with a mean age of 23.7 years +/- 5.69 were included in this study. The average hearing thresholds of SCA patients were consistently higher than controls in all frequencies tested in both right and left ears. Of the 92 ears of SCA patients tested, 95.7% exhibited hearing thresholds within normal limits, and 4.3% had mild hearing loss. The controls had thresholds within normal limits. CONCLUSION: The incidence of significant sensorineural hearing loss in SCA seems to have reduced in line with the general improvement and survival of SCA patients. The hearing loss is worse in the right ear and has a female preponderance. We hope that more aggressive primary and secondary prevention and adequate treatment of sickle cell crisis would reduce if not eliminate the hearing loss found in SCA.
PMCID: PMC2575988  PMID: 16173329
21.  Splenic syndrome in patients at high altitude with unrecognized sickle cell trait: splenectomy is often unnecessary 
Canadian Journal of Surgery  2005;48(5):377-381.
The health risks associated with sickle cell trait are minimal in this sizable sector of the world's population, and many of these patients have no information about their sickle cell status. Splenic syndrome at high altitude is well known to be associated with sickle cell trait, and unless this complication is kept in mind these patients may be subjected to unnecessary surgery when they present with altitude-induced acute abdomen.
Four patients were admitted to the surgical ward with a similar complaint of acute severe left upper abdominal pain after arrival to the mountainous resort city of Abha, Saudi Arabia. All were subjected to splenectomy because of lack of suspicion regarding sickle cell status.
Histologic examination of the spleen showed all patients had sickle cells in the red pulp. On further assessment all were found to have sickle cell trait with splenic infarction. In a similar study of 6 patients with known sickle cell disease who had comparable problems when they travelled to the Colorado mountains, all made an uncomplicated recovery with conservative management.
In ethnically vulnerable patients with splenic syndrome, sickle cell trait should be ruled out before considering splenectomy. These patients could respond well to supportive management, and splenectomy would be avoided.
PMCID: PMC3211898  PMID: 16248136
22.  No further incidence of sepsis after splenectomy for severe trauma: a multi-institutional experience of the trauma registry of the DGU with 1,630 patients 
Non-operative management of blunt splenic injury in adults has been applied increasingly at the end of the last century. Therefore, the lifelong risk of overwhelming post-splenectomy infection has been the major impetus for preservation of the spleen. However, the prevalence of posttraumatic infection after splenectomy in contrast to a conservative management is still unknown. Objective was to determine if splenectomy is an independent risk factor for the development of posttraumatic sepsis and multi-organ failure.
13,433 patients from 113 hospitals were prospective collected from 1993 to 2005. Patients with an injury severity score > 16, no isolated head injury, primary admission to a trauma center and splenic injury were included. Data were allocated according to the operative management into 2 groups (splenectomy (I) and conservative managed patients (II)).
From 1,630 patients with splenic injury 758 patients undergoing splenectomy compared with 872 non-splenectomized patients. 96 (18.3%) of the patients with splenectomy and 102 (18.5%) without splenectomy had apparent infection after operation. Additionally, there was no difference in mortality (24.8% versus 22.2%) in both groups. After massive transfusion of red blood cells (> 10) non-splenectomy patients showed a significant increase of multi-organ failure (46% vs. 40%) and sepsis (38% vs. 25%).
Non-operative management leads to lower systemic infection rates and mortality in adult patients with moderate blunt splenic injury (grade 1-3) and should therefore be advocated. Patients with grade 4 and 5 injury, patients with massive transfusion of red blood cells and unstable patients should be managed operatively.
PMCID: PMC3351995  PMID: 20696635
blunt abdominal trauma; splenic injury; splenectomy; transfusion; sepsis; mortality
23.  Unusual case of pancreatic inflammatory myofibroblastic tumor associated with spontaneous splenic rupture 
Spontaneous splenic rupture considered a relatively rare but life threatening. The three commonest causes of spontaneous splenic rupture are malignant hematological diseases, viral infections and local inflammatory and neoplastic disorders. We describe a unique and unusual case of inflammatory myofibroblastic tumor of the tail of pancreas presented with massively enlarged spleen and spontaneous splenic rupture.
Case presentation
A 19 years old male patient with no significant past medical history presented to emergency room with abdominal pain and fatigue. Massively enlarged spleen was detected. Hypotension and rapid reduction of hemoglobin level necessitated urgent laparatomy. About 1.75 liters of blood were found in abdominal cavity. A large tumor arising from the tail of pancreas and local rupture of an enlarged spleen adjacent to the tumor were detected. Distal pancreatectomy and splenectomy were performed. To our knowledge, we report the first case of massively enlarged spleen that was complicated with spontaneous splenic rupture as a result of splenic congestion due to mechanical obstruction caused by an inflammatory myofibroblastic tumor of the tail of pancreas. A review of the literature is also presented.
Inflammatory myofibroblastic tumor of the tail of pancreas should be included in the differential diagnosis of the etiological causes of massively enlarged spleen and spontaneous splenic rupture.
PMCID: PMC2995784  PMID: 21092210
24.  Evaluation of Partial and Total Splenectomy in Children with Sickle Cell Disease Using an Internet-Based Registry 
Pediatric blood & cancer  2012;59(1):100-104.
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.
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.
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.
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.
PMCID: PMC3330148  PMID: 22238140
Sickle cell disease; partial splenectomy; total splenectomy; electronic data capture; medical informatics
25.  Management of Postoperative Complications Following Splenectomy 
International Surgery  2013;98(1):55-60.
Complications of post-splenectomy, especially intra-abdominal hemorrhage can be fatal, with delayed or inadequate treatment having a high mortality rate. The objective of this study was to investigate the cause, prompt diagnosis, and outcome of the fatal complications after splenectomy with a focus on early diagnosis and management of hemorrhage after splenectomy. The medical files of patients who underwent splenectomy between January 1990 and March 2011 were reviewed retrospectively. The cause, characteristics, management, and outcome in patients with post-splenectomy hemorrhage were analyzed. Fourteen of 604 patients (1.19%) undergoing splenectomy had intraperitoneal hemorrhage: reoperation was performed in 13 patients, and 3 patients died after reoperation, giving the hospital a mortality rate of 21.43%; whereas, 590 of 604 patients (98%) had no hemorrhage following splenectomy, and the mortality rate (0.34%) in this group was significantly lower (P < 0.001). The complications following splenectomy, including pneumonia pancreatitis, gastric fistula, gastric flatulence, and thrombocytosis, in patients with postoperative hemorrhage were significantly higher than those without hemorrhage (P < 0.001). According to the reasons for splenectomy, 14 patients with post-splenectomy hemorrhage were grouped into two groups: splenic trauma (n = 9, group I) and portal hypertension (n = 5, group II). The median interval between splenectomy and diagnosis of hemorrhage was 15.5 hours (range, 7.25–19.5 hours). No differences were found between groups I and II in terms of incidence of postoperative hemorrhage, time of hemorrhage after splenectomy, volume of hemorrhage, and mortality of hemorrhage, except transfusion. Intra-abdominal hemorrhage after splenectomy is associated with higher hospital mortality rate and complications. Early massive intraperitoneal hemorrhage is often preceded by earlier sentinel bleeding; careful clinical inquiry and ultrasonography are the mainstays of early diagnosis.
PMCID: PMC3723162  PMID: 23438277
Splenectomy; Intra-abdominal hemorrhage; Postoperative complications; Splenic trauma; Portal hypertension

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