Splenic Sequestration Crisis Research Articles

Splenic complications of sickle cell anemia and the role of splenectomy.

Sickle cell disease is one of the common hemoglobinopathies in the world. It can affect any part of the body and one of the most common and an early organ to be affected in SCA is the spleen. It is commonly enlarged during the first decade of life but then undergoes progressive atrophy leading to autosplenectomy. This however is not the case always and sometimes splenomegaly persist necessitating splenectomy for a variety of reasons including acute splenic sequestration crisis, hypersplenism, massive splenic infarction and splenic abscess. Splenic complications of SCA are known to be associated with an increased morbidity and in some it may lead to mortality. To obviate this, splenectomy becomes an essential part of their management. This review is based on our experience in the management of 173 children with various splenic complications of SCA necessitating splenectomy.

Clinical events after surgical splenectomy in children with sickle cell anemia

Despite advances in immune prophylaxis, sepsis remains the most feared complication following splenectomy for acute splenic sequestration crisis (ASSC) in children with sickle cell anemia (SCA). We seek to investigate the true prevalence of sepsis and other complications of splenectomy in this patient population. We reviewed the records of children with SCA (HbSS) who underwent splenectomy for ASSC between 1993 and 2008 at a single institution. Fifty-eight patients (33 males) at a median age of 2 years at splenectomy were included with an average post-splenectomy follow-up of 6.4 years (range 6 months-14 years). Thirty-seven patients (64%) underwent laparoscopic splenectomy, and acute chest syndrome (ACS) was the most common post-operative complication (6.9%). There was no difference in the incidence of sepsis pre- and post-splenectomy. The occurrence of vaso-occlusive pain crises (VOC) and ACS was significantly higher after splenectomy. In addition, 14 patients (24%) developed stroke (n = 5) or an abnormal transcranial Doppler (TCD) (n = 9) after splenectomy. Our data suggest that splenectomy can be safely performed in children with SCA given a low risk of sepsis. However, the increased incidence of VOC, ACS, and stroke or abnormal TCDs after splenectomy remains a concern.

Hyperhaemolysis in sickle cell disease — an unusual and potentially life‐threatening complication

As sickle cell disease is increasing in Australia, paediatricians and other health care providers need to be aware of the broad range of complications that can occur in this condition. Although the complications of splenic sequestration and chest crises are well recognised, the infrequent but equally dramatic complication of hyperhaemolysis is less well appreciated. Here, we report a case of hyperhaemolysis in a Victorian paediatric patient.

Partial splenectomy prevents splenic sequestration crises in sickle cell disease

Purpose Acute splenic sequestrations (SSs) are potentially fatal complications in sickle cell disease (SCD). Total splenectomies in young patients may predispose them to a higher risk of overwhelming infections, whereas partial splenectomy may maintain immunocompetence. We present our series of partial splenectomies in patients with multiple SS episodes. Methods We retrospectively reviewed the records of 6 patients who underwent open partial splenectomies for SS. Data on their clinical courses were collected and analyzed. Results None of the 6 patients had SS postprocedure, down from 2.1 ± 1.0 ( P = .003) sequestrations per year and 3.5 ± 1.4 ( P = .002) total sequestrations per patient. The transfusion requirements were significantly reduced postoperatively (10.2 ± 5.6 vs 2.0 ± 3.1 per year; P = .002). There was no increase in the infection-related hospital admissions during the period of follow-up (1.5 ± 1.8 vs 0.8 ± 0.8 per year after partial splenectomy; P = .363). The upper pole was preserved in all cases with blood supply off the main splenic artery. Conclusions Partial splenectomy decreases the risk of SS in SCD and reduces the need for blood transfusions. Infection rates did not increase after the procedure during the follow-up period. Partial splenectomy should be considered for patients who experience multiple acute SS crises or have long-term transfusion requirements.

Outcome of splenectomy in children younger than 4 years with sickle cell disease

Purpose Removal of the spleen in patients younger than 4 years has been reported to carry an increased risk of postsplenectomy sepsis and has not been universally accepted. We reviewed our experience with splenectomy in children with acute splenic sequestration crisis (ASSC) younger than 4 years. Methods The study involved retrospective review of demographic and operative data, number of ASSC, operative complications, infections, and death. Results From 1993 to 2008, 53 patients (28 males, 25 females) younger than 4 years had open (43.8%) or laparoscopic (56.6%) splenectomy after one or more events of ASSC. Six (11.3%) were younger than 18 months, 28 (52.8%) were 18 to 24 months old, and 21 (39.6%) were 24 to 48 months old. Operative complications were diaphragm laceration (laparoscopy, n = 3; 5.7%) and reoperation for bleeding (open, n = 1; 1.8%). Length of stay was similar for laparoscopic (3.6 days) vs open (3.8 days) splenectomy. Mean postoperative follow-up was 5.6 years. In 353 postsplenectomy admissions, 3 (5.7%) patients had positive blood cultures requiring treatment. Three (5.7%) patients died within the 15-year study period; one (1.8%) had documented pneumococcal sepsis. Discussion The advantage of early splenectomy may outweigh the risks of long-term transfusion. Splenectomy in young children with sickle cell disease carries a low risk of postsplenectomy sepsis with appropriate vaccination and prophylactic antibiotics. We conclude that splenectomy in young children with ASSC is safe and effective, especially with penicillin prophylaxis and improved vaccination strategies.

Simultaneous acute splenic sequestration and transient aplastic crisis in children with sickle cell disease

Acute splenic sequestration crisis (ASSC) is a hematological emergency in young children with sickle cell disease (SCD), characterized by worsening anemia and splenomegaly, usually with reticulocytosis and thrombocytopenia. Transient aplastic crisis (TAC) due to parvovirus B19 infection occurs in older children with SCD, and typically manifests as worsening anemia with reticulocytopenia and no splenomegaly. Five older children with SCD (4 HbSC, 1 HbSS on hydroxyurea) developed ASSC concurrent with TAC and had a severe clinical course. Our cases suggest that older children with SCD and acute parvovirus infection should be monitored closely for splenomegaly and multi-system dysfunction.

Laparoscopic splenectomy and/or cholecystectomy for children with sickle cell disease

In 1991, Delaitre reported the first laparoscopic splenectomy (LS). Since then LS has become the procedure of choice to treat hematological diseases requiring splenectomy. The Eastern province of Saudi Arabia is known to have a high incidence of hemoglobinopathies including sickle cell disease (SCD), which is known to be associated with complications necessitating splenectomy and/or cholecystectomy. This report describes our experience with LS and/or laparoscopic cholecystectomy (LC) for children with SCD. The medical records of all children with SCD who had LS and/or LC were retrospectively reviewed for age, sex, indication for splenectomy, operative time, hospital stay, and post-operative complications. The results were compared to a similar group of children with SCD who had open splenectomy (OS) and/or open cholecystectomy (OC). Over a period of 3.5 years (January 2005 and June 2008), a total of 45 children had LS with or without LC, 30 (66.7%) of them had SCD. Their age ranged from 2 to 12 years (mean 7 years). There were 16 males and 14 females. In all, LS was done because of recurrent splenic sequestration crisis except one who had a large spleen with multiple infarcts that was causing abdominal pain. The operative time ranged from 1.5 to 9 h (mean 2.75 h). Their hospital stay ranged from 3 to 9 days (mean 4.5 days). There was no mortality. Two patients (6.7%) required conversion to OS due to a large-sized spleen and severe adhesions in one and uncontrolled intra-operative bleeding in the other. The results were compared to a group of 120 children with SCD who had OS only (88) and OS with OC (32). From 1994 to 2006, a total of 55 children had LC only, 47 (26 M:21 F) of them (85.5%) had SCD. Their age ranged from 4 to 15 years (mean 11.4 years). The indications for cholecystectomy were: biliary dyspepsia (20), biliary colic (35), acute cholecystitis (5), obstructive jaundice (5), asymptomatic (6), and biliary pancreatitis (1). There was no mortality, but one (2.1%) required conversion to OC because of severe adhesions and another underwent postoperative exploration because of bleeding from an accessory cystic artery. The results were compared to a similar group of 27 children with SCD who underwent OC. With good peri-operative management, LS is feasible and safe in children with SCD and can be done concomitantly with cholecystectomy. Currently, it requires more operative time than the open approach. This is specially so for children with SCD who are known to have a large spleen with severe adhesions. It is, however, superior to OS with regard to duration of hospital stay, cosmetic appearance, post-operative complications, and post-operative recovery. LC is also safe in children with SCD. When compared with OC, it is associated with less post-operative complications, a shorter hospital stay, better cosmetic appearance and a faster recovery.

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.

Alloimmunization Does Not Modify the Clinical Profile of Sickle Cell Disease Patients from Salvador-Brazil

Introduction: The hemoglobinopathies are the most common monogenic disorders known. A mutation in the gene for β globin gave origin to hemoglobin S, an abnormal hemoglobin originated in Africa. Sickle cell disease (SCD) is characterized by the presence of hemoglobin S, which results in vasoocclusion episodes and hemolytic anemia throughout patients life. Vascular occlusion leads to acute events and progressive disabling organ damage. Sickle cell anemia is the homozygous state SS, while hemoglobinopathy SC is a doubly heterozygous state, where hemoglobin S occurs in combination with hemoglobin C. Brazil has a prominent African ancestry and SCD is highly prevalent in some regions of the country. In Bahia State, for example, neonatal screening data have shown that, from every 650 children born alive, one has SCD, mostly homozygous SS. Among other therapeutic measures, packed red blood cells (RBC) play a prominent role in SCD management. In situations such as acute chest syndrome (ACS), primary and secondary prevention of stroke, splenic or hepatic sequestration crisis, severe anemia, complicated pregnancy, isquemic organ damages and others, the transfusions may save lives. Although RBC may contribute to reduce morbidity and improve quality of life in SCD patients, there still are risks. Among other risk categories, alloimmunization may result from transfusions and occurs in 5 % to 50 % of SCD patients. It is still not known whether allosensibilization significantly affects the clinical outcomes in SCD.Objecive: The purpose of this study was to compare the clinical profile of multitransfused adult SCD patients who developed alloantibodies (ALO) to patients with the same disease, coming from the same population who did not become alloimmunized (non-ALO).Methods: This is a cross sectional study where medical records of SCD patients, referred to a reference center of Salvador, the capital of Bahia State, Brazil, were reviewed. Only SCD patients 18 years of age or older were included. They had received at least 3 RBC transfusions from 2004 to 2007, or had any alloantibody identified during this period. Patient characteristics, clinical findings, number of transfusions, frequency and specificity of alloantibodies, laboratory data, and the main clinical outcomes were reviewed.Results: a hundred and eight patients were included: 105 SS and 3 SC. The pre-transfusional RBC matching was done to ABH, D,C,c,E,e and Kell antigens. 56 patients developed alloantibodies (53 SS and 3 SC). Anti-E, anti-K, and anti-C were the most prevalent alloantibodies identified (39,3 %, 21,4 % and 16,1 %, respectively). Among the variables addressed in this study, age (higher in non-ALO, .041) and antiglobulin test positivity, more prevalente in ALO (.0001), depicted statistically significant difference. A few patients developed immune hemolysis, controlled successfully with corticosteroids. Alloimmunization was more prevalent among women, although no statistically significant difference was reached between ALO and non-ALO Other variables such as number of transfusions, hematological profile, biochemical data and complications such as stroke, leg ulcers, osteonecrosis, renal disease, abnormal cardiac features, and pulmonary hypertension did not show significant difference between both groups.Conclusion: his study shows that, although alloimmunization is a potential dangerous consequence of RBC transfusions, it did not modify the clinical profile of SCD alloimmunized patients. The concomitance of allosensibilization and autoantibodies in SCD leads to additional difficulties in the RBC matching for transfusion and may exacerbate hemolysis. In order to address autoimmunity in SCD, prospective studies with larger samples are needed.

Acute splenic sequestration in an adult with hemoglobin S-C disease

Acute splenic sequestration crisis is a common, potentially life-threatening complication of sickle cell anemia in children that is uncommon in adults.We present the case of a 44-year-old gentleman with undiagnosed hemoglobin S-C disease who developed intense back pain, marked abdominal distension, systemic inflammatory response syndrome, and multisystem organ failure that first presented as acute splenic sequestration crisis. The hemoglobinopathy SC is a disease caused by heterozygous-globin chain mutations with over-lapping clinical features of sickle cell disease with changes in the frequency of these manifestations reflected by the combination of characteristics of hemoglobin C and hemoglobin S. In hemoglobin S-C disease, autosplenectomy is rare and splenomegaly usually persists until adulthood;vasoocclusive complications are seen less habitually and become evident at a later time compared with sickle cell disease. The diagnosis of hemoglobin S-C disease is essentially done by exclusion. Transfusion of red blood cells is the treatment of choice, but splenectomy is indicated if transfusion therapy fails. A review of the literature and keypoints for the emergency practitioner are included.

Ten-Year Review of Hospital Admissions among Children with Sickle Cell Disease in Kuwait

Objective: This study was designed to document the common causes and patterns of hospitalization among sickle cell disease (SCD) patients in Kuwait. Subjects and Methods: The case files of all SCD patients admitted to the hospital between 1995 and 2004 were studied. Their personal data, hemoglobin genotype, diagnosis on admission, complete blood count, treatment received, length of stay and outcome were documented. Results: Fifty SCD patients aged from 10 months to 14.8 years (mean: 8.7 ± 2.8 years) had 351 admissions over the 10-year period accounting for 0.6% of all admissions to the pediatric wards. The 50 patients were made up of 18 Hb SS, 28 Sβ⁰thal and 4 Hb SD patients. Common causes of admission were vaso-occlusive crisis: 222 (63.2%), acute splenic sequestration crisis: 32 (9.1%), hemolytic crisis: 31 (8.8%) and acute chest syndrome: 23 (6.6%). Stroke was seen in only 1 patient, and bacteriologically proven (Salmonella) acute osteomyelitis in 1 patient. There were no deaths during the study period. Conclusions: Vaso-occlusive crisis was the commonest cause of hospitalization among our SCD patients. Efforts should be intensified to give advice on preventive measures. The use of hydroxyurea should be encouraged in patients with frequent severe pain crises.

Acute splenic sequestration crisis (ASSC) in an adult patient with β-thalassemia sickle cell disease: a life-threatening complication

Dear Editor, We present a case of an adult patient with sickle cell βthalassemia and a severe episode of recurrent acute splenic sequestration crisis (ASSC). A 21-year-old white man with sickle cell β-thalassemia was admitted to our hospital with headache and abdominal pain. At the age of six, he had an acute thrombotic cerebrovascular event, and since then, he was placed on repeated sessions of exchange transfusion. At the age of seven, he had suffered an episode of ASSC that was treated conservatively. Since then, the patient presented frequent pain crises that did not respond to hydroxyurea treatment. At admission, his blood pressure was 140/70 mmHg, body temperature was 38°C, and heart rate was 105 beats/min. He had hepatomegaly and splenomegaly (4 cm under the left costal margin). Hemoglobin (Hb) was 9.4 g/dl, hematocrit 29.8%, MCV 75.8 fl, white blood cell count 4,500/μl, platelet count 206,000/μl, LDH 372 U/l, and total bilirubin 2.21 mg/dl/ indirect 0.78mg/dl. HbSwas 71%. An abdominal ultrasound at admission showed an enlarged spleen (19 cm). Intravenous fluids and pain relief medications were started and the patient initially improved. On the third hospital day, the patient was suddenly found tachycardic (125 beats/min), confused, short of breath with significant hypoxemia (SatO2=80%). His Hb and platelet count dropped to 3.5 g/dl and 52,000/μl, respectively, while his LDH (1,368 U/l) and total bilirubin (3.70 mg/dl) were increased. A urinalysis was negative for Hb. A repeated abdominal ultrasound revealed an increase in the size of spleen (21 cm) with new peripheral hypoechoic areas. Intravenous fluids, oxygen, and emergency transfusion with four packed RBC units and fresh frozen plasma (FFP) were promptly administered. The patient rapidly improved and a progressive rise in Hb and SatO2 was documented. An abdominal computed tomography scan confirmed the U/S findings (see Fig. 1). The patient was transfused with two more RBC units and was discharged on the tenth hospital day with a Hb of 9.2 g/dl. An elective splenectomy was scheduled. ASSC represents a rare complication of sickle cell disease and its variants with increased mortality during the first decade of life [1–6, 14, 15]. This adult patient with sickle cell β-thalassemia presented with an acute, life-threatening, and recurrent episode of ASSC. The diagnosis was established by the acute splenic enlargement, the severe and rapid drop of Hb, and the accompanying new onset thrombocytopenia. Because his Hb decreased to 3 g/dl, this episode is considered as major by definition [8]. A minor episode is defined as a drop in Hb levels but with an Hb level >6 g/dl. Ten cases of ASSC in adults with sickle cell βthalassemia have been reported in the English literature [7–10, 14]. Of these, nine were men (90%) with a mean age of 36 years. Three of these episodes could be classified as major. The lowest Hb level recorded was 3.5 g/dl, and it was reported in our case. The mortality rate in these patients was high (50%). Four of the five (80%) surviving patients experienced recurrent ASSC [7–10, 14]. Ann Hematol (2008) 87:499–500 DOI 10.1007/s00277-007-0422-3

Indications and complications of splenectomy for children with sickle cell disease

Background Sickle cell anemia (SCA), which is characterized by high hemoglobin (Hb) F level and persistent splenomegaly into the older age group (up to 18 years of age) or even adults, is one of the commonest hemoglobinopathies in the Eastern Province of Saudi Arabia. This makes them liable to develop splenic complications requiring splenectomy. This is a review of our experience in the management of 134 children with SCA who had splenectomy as part of their management at our hospital, with emphasis given to the indications and complications of splenectomy. Patients and methods The medical records of all children who had splenectomy at our hospital were retrospectively reviewed for the following: age at splenectomy, sex, Hb electrophoresis, indication for splenectomy, preoperative investigations, type of surgery, spleen weight, histology, perioperative management, and postoperative complications. Results From 1990 to 2004, 170 children with various hematologic disorders had splenectomy at our hospital. Of these, 134 had SCA (118 had sickle cell disease and 16 had sickle- β-thalassemia). Recurrent acute splenic sequestration crisis (ASSC) was the commonest indication for splenectomy in 103 (76.9%) patients, followed by hypersplenism in 18 (13.4%). Seven (5.2%) of our patients had splenectomy for splenic abscess (SA) and 2 had splenectomy for massive splenic infarction; 103 (61 boys, 42 girls) patients with a mean age of 7.6 years (range, 1.8-13 years) had splenectomy for ASSC. Their mean Hb F level was 20.5% (range, 9.2%-39.6%). Thirty-two of them had major attacks. Their Hb levels at the time of admission ranged from 1.4 to 4.1 g/dL (mean, 2.5 g/dL). The remaining 71 had minor recurrent attacks. Eighteen had splenectomy for hypersplenism and all had a significant increase in their blood parameters after splenectomy. Seven had splenectomy for SA. In 5 patients, Salmonella was the causative organism; in 1, it was Enterobacter sakazaki, whereas in 1, no organisms were identified. Two of our patients had splenectomy for massive splenic infarction because of persistent left upper quadrant abdominal pain, and 1 had splenectomy for splenomegaly with a nonfunctioning spleen. Twenty-eight (21%) of our patients had splenectomy and cholecystectomy. In 4 patients, this was because of symptomatic gallstones, whereas in the other 24, it was because of asymptomatic gallstones discovered on ultrasound. There was no mortality, but 8 (6%) developed postoperative complications. Conclusions With good perioperative management, splenectomy in children with SCA is not only safe, but also beneficial in treating SA, reducing the patients' transfusion requirements, eliminating the risks of ASSC, and eliminating the discomfort and mechanical pressure of the enlarged spleen. Abdominal ultrasound should be done routinely preoperatively for all children with SCA undergoing splenectomy, and if gallstones are discovered, they should undergo concomitant cholecystectomy. This is even so for asymptomatic gallstones. The addition of cholecystectomy to splenectomy does not increase the morbidity, but eliminates the subsequent complications of gallstones and simplifies their future management in case of abdominal crisis as the possibility of cholecystitis is eliminated.

Acute splenic sequestration crisis in adults with sickle cell anemia

American Journal of HematologyVolume 82, Issue 2 p. 174-175 Letters and CorrespondenceFree Access Acute splenic sequestration crisis in adults with sickle cell anemia Prasad Rao Koduri, Prasad Rao Koduri Division of Hematology/Oncology, Cook County Hospital, Chicago, IllinoisSearch for more papers by this author Prasad Rao Koduri, Prasad Rao Koduri Division of Hematology/Oncology, Cook County Hospital, Chicago, IllinoisSearch for more papers by this author First published: 21 August 2006 https://doi.org/10.1002/ajh.20727Citations: 9AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL REFERENCES 1 Platt OS. Sickle syndromes. In: RI Handin, SE Lux, TP Stossel, editors. Blood, Principles and Practice of Hematology. Philadelphia: Lippincott, 2003. pp 1675– 1677. 2 Moll S, Orringer EP. Case report: Splenomegaly and splenic sequestration in an adult with sickle cell anemia. Am J Med Sci 1996; 312: 299– 302. 3 De Ceulaer K, Serjeant GR. Acute splenic sequestration in Jamaican adults with homo zygous sickle cell disease: A role of alpha thalassaemia. Br J Haematol 1991; 77: 563– 564. 4 Bowcock SJ, Nwabueze ED, Cook AE, Aboud HH, Hughes RG. Fatal splenic sequestration in adult sickle cell disease. Clin Lab Haematol 1988; 10: 95– 99. 5 Sarma PSA. Acute splenic sequestration crisis in a young woman with homozygous sickle cell anaemia. Postgrad Med J 1989; 65: 105– 107. Citing Literature Volume82, Issue2February 2007Pages 174-175 ReferencesRelatedInformation

Acute splenic sequestration crisis in an adult with sickle β-thalassemia

Acute splenic sequestration crisis (ASSC) is a major cause of morbidity and mortality in children with sickle cell disease. Reports of ASSC in adults with sickle beta-thalassemia (S-beta(thal)) are rare and consist of isolated case reports comprising a total of seven patients, three of whom died during the crisis. We report a 22-year-old man with S-beta(thal) who developed ASSC 1 day after suffering multiple blunt trauma. Systemic inflammatory response to severe blunt trauma may have precipitated ASSC in our patient. ASSC in adults with S-beta(thal) is a potentially life-threatening complication with a high risk of recurrence. Splenectomy is recommended after the first attack of ASSC in adults with S-beta(thal).

Acute splenic sequestration crisis in adults with hemoglobin S-C disease: a report of nine cases

Acute splenic sequestration crisis (ASSC) is a potentially life-threatening complication and one of the leading causes of death in children with sickle cell disease. It is rarely reported in adults with hemoglobin S-C disease and its natural history in these patients has not been well studied. We describe here the clinicopathological features of ASSC in nine adults with hemoglobin S-C disease treated between 1972 and 2000 and followed for a mean period of 9 years (range 0-21 years). ASSC was characterized by acute left upper quadrant abdominal pain, splenomegaly, fever, and a rapid decrease in hematocrit with active erythropoiesis. The hemoglobin decreased by a mean of 4.8 g/dl from the steady state value (range 3.0 to 6.7 g/dl) during ASSC. Two patients failed to respond to transfusion of packed erythrocytes and required urgent splenectomy. There was one fatality-a 76-year-old woman, who died 36 h after admission. There was no recurrence of ASSC in five patients followed for 2, 3, 16, 18, and 21 years, respectively. ASSC in adults is a serious and occasionally, fatal complication of hemoglobin S-C disease. Patients with hemoglobin S-C disease may remain at risk of ASSC into their eighth decade.

Types of anaemic crises in paediatric patients with sickle cell anaemia seen in Enugu, Nigeria

Background: Anaemic crises in paediatric patients with sickle cell anaemia are major causes of morbidity and mortality. Some children admitted to hospitals’ emergency rooms or paediatric wards of the hospitals...

Neonatal screening for hemoglobinopathies in Rio de Janeiro, Brazil

To describe the main results obtained in the first 15 months of neonatal screening for sickle cell disease in the state of Rio de Janeiro, Brazil, from August 2000 to November 2001. Starting in August 2000, blood samples began to be collected for sickle cell disease screening from all newborns receiving care in primary health care clinics in the state of Rio de Janeiro. The samples were submitted to high-resolution liquid chromatography. If the resulting chromatogram was compatible with sickle cell disease, the child and the parents were referred for diagnostic confirmation and treatment. Between August 2000 and November 2001, 99 260 newborns were screened. There was one case of homozygous Hb C. On average, one of every 27 newborns who were screened presented sickle cell trait (Hb AS). Sickle cell disease was observed in 83 cases, or one new case in each 1 196 births. The 83 consisted of: 62 Hb S, 18 Hb SC, and 3 Hb SD. One child did not appear for diagnostic confirmation. The 82 children who were followed up by the program presented 15 intercurrent illnesses (upper respiratory infections, fever, splenic sequestration crises, hand-foot syndrome, and vascular occlusion), resulting in seven hospital admissions. Blood transfusions were necessary with 15 children, but none developed alloimmunization. All the other babies were doing well with the use of prophylactic penicillin. Our data show the importance of early diagnosis for sickle cell disease, so as to prevent the frequent infectious complications faced by these patients.

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.

Should cholecystectomy be performed concomitantly with splenectomy in children with sickle-cell disease?

Splenectomy and cholecystectomy are among the common surgical procedures required to treat complications of sickle-cell disease (SCD), and when performed separately have been shown to be safe and effective. To determine whether cholecystectomy be performed concomitantly with splenectomy (CSC) in these children, we studied a total of 130 children who underwent splenectomy for various hematologic diseases at our hospital. The most common indication was SCD. Ninety-nine patients (86 SCD and 13 sickle-B-thalassemia) underwent splenectomy and 19 (19.2%) (12 males and 7 females, mean age 13.4 years [7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18]) underwent CSC due to concomitant gallstones, which were asymptomatic in 13 cases. Those with an admission hemoglobin (Hb) of less than 10 g/dl were transfused with packed erythrocytes to increase their Hb to 10-12 g/dl and their hematocrit to 30%-40%. All patients received intravenous hydration the night before surgery at a rate of 1(1/2) times their maintenance rate, which was continued postoperatively until they were able to tolerate adequate oral fluid intake. The indications for splenectomy in those who had CSC were: acute splenic sequestration crisis in 12, splenic abscess in 3, hypersplenism in 2, and massive splenic infarction in 2. Eight patients had massive splenomegaly (spleen weight >1 kg). In 7 cases CSC was done through a left-upper-quadrant (LUQ) transverse incision, in the remaining 12 through an upper midline incision. There was no mortality and only 2 patients developed postoperative complications; a wound infection in 1 and a hematoma in the splenic bed in another. With good perioperative management, CSC is both safe and effective for children with SCD. Both procedures can be performed safely through an upper midline or a LUQ transverse incision, even in the presence of massive splenomegaly.