Splenectomy For Hereditary Spherocytosis Research Articles

The Use of Next-generation Sequencing in the Diagnosis of Rare Inherited Anaemias: A Joint BSH/EHA Good Practice Paper.

The Use of Next-generation Sequencing in the Diagnosis of Rare Inherited Anaemias: A Joint BSH/EHA Good Practice Paper.

Changing trends of splenectomy in hereditary spherocytosis: The experience of a reference Centre in the last 40 years.

We describe the clinical/haematological characteristics of 446 patients with hereditary spherocytosis diagnosed in the last 40years in a reference centre. The frequency of splenectomy decreased over time (44% before 1990 to 7% in 2011-2020), notwithstanding a confirmed good efficacy. Age at splenectomy progressively increased (63% in children before 1990 to 88% in patients aged ≥20years in 2011-2020). Our real-life experience showed that even a fraction of patients in the trait/mild categories (19/92, 21%) were splenectomised, whilst 30/78 (38%) in the moderate/severe groups were not. Overall, these data pinpoint to the increasing awareness about post-splenectomy thromboses and infections.

Reactive Thrombocytosis after Splenectomy in Hereditary Spherocytosis: Case Report and Literature Review.

Reactive thrombocytosis can be a significant complication after splenectomy and can range from mild (500,000-700,000/mm3), to moderate (700,000-900,000/mm3), severe (>900,000/mm3) and very severe thrombocytosis (>1,000,000/mm3).The use of low-dose hydroxyurea in patients with very severe thrombocytosis can reduce the platelet count to safe levels, and thus, the risk of developing thrombosis.

Long-Term Evaluation of the Outcomes of Subtotal Laparoscopic and Robotic Splenectomy in Hereditary Spherocytosis.

Hereditary spherocytosis (HS) is a common inherited disease affecting the erythrocyte membrane. Total splenectomy (TS) is effective in reducing hemolysis and decreasing the need of transfusions, but total removal of the spleen represents a potential risk factor for infectious and non-infectious complications. On the other hand, subtotal splenectomy (STS) could be an alternative therapy for HS. The aim of this study is to establish which surgical approach has the best outcome in HS. All patients (n = 63) receiving splenectomy for HS between 2002 and 2016 from one institution were retrospectively reviewed. Hemoglobin and reticulocytes levels during preoperative and postoperative follow-up periods were compared. Additionally, a meta-analysis was performed analyzing data regarding hemoglobin and reticulocytes levels from several available studies. At 1-year follow-up, our clinical data showed that mean hemoglobin levels increased after TS from (mean ± SD) 9.77 ± 1.82 to 11.88 ± 2.08g/dl, while after STS from 8.98 ± 1.66 to 11.87 ± 1.38g/dl. At 3-year and 5-year follow-up after TS, we observed an increase from 9.77 ± 1.82 to 13.59 ± 2.03 and 13.46 ± 1.64g/dl, respectively. At 3-year and 5-year follow-up after STS in our cohort, we observed an increase from 8.98 ± 1.66 to 13.21 ± 1.95 and 13.68 ± 1.65g/dl, respectively. The meta-analysis (for a follow-up period of 1year) showed that the hemoglobin levels increased with 2.61g/dl (95% CI 2.15-3.08g/dl; p < 0.001) after TS, and with 1.67g/dl (95% CI 1.25-2.10g/dl; p < 0.001) after STS. We conclude that subtotal and minimally invasive splenectomy could be considered as the first line of treatment in severe HS cases, especially in children.

Modulation of Hemolytic and Hemoglobin/Heme Scavenging Profiles in Sickle Cell Anemia, Hereditary Spherocytosis and Paroxysmal Nocturnal Hemoglobinuria

Sickle Cell Anemia (SCA), Hereditary Spherocytosis (HS) and Paroxysmal Nocturnal Hemoglobinuria (PNH) can share some clinical complications, such as leg ulcers and priapism, due to hemolytic processes. Intravascular hemolysis (IVH) results in the release of cell-free hemoglobin (Hb) and heme into the circulation, potentially causing depletion of nitric oxide, oxidative stress and inflammation. We compared the hemolytic profile and heme/Hb scavenging profiles of patients with SCA on hydroxyurea therapy (SCAHU; 15-30 mg/kg/day for ≥ 2 months) or not (SCA), hereditary spherocytosis (HS), splenectomized HS (SHS) and paroxysmal nocturnal hemoglobinuria not on eculizumab therapy (PNH). Blood samples were collected from patients and from healthy volunteers (CON). Plasma levels of heme, haptoglobin (Hp) and hemopexin (Hpx) were quantified by colorimetric assay or ELISA. Fetal hemoglobin (HbF) was determined in the SCA groups by HPLC. Reticulocytes (RET) and cell counts were determined using an automated flow cytometry hematology system and serum levels of lactate dehydrogenase (LDH) and unconjugated bilirubin (UCB) were quantified by a modular colorimetric automation system. As expected, plasma heme was significantly elevated in the SCA group, compared to the CON group and with intermediate values in the SCAHU group (57.4±9.3, 24.5±2.9, 43.2±4.5µM, respectively, p<0.05, N≥10), correlating significantly and negatively with HbF levels in the SCA patients (r=-0.94, p<0.01). Accordingly, RET and UCB were significantly increased in SCA, compared to CON, with SCAHU presenting intermediate values (RET: 16.2±1.4; 1.9±0.1; 11.5±1.2%; UCB: 2.1±0.7; 0.2±0.0; 1.44±0.2 mg/dL; respectively, p<0.01, N≥10), indicating increased red cell turnover and heme catabolism in SCA. No significant elevations in plasma heme were observed in the HS and SHS groups, compared to CON (35.3±3.8 and 34.5±10.0µM; P>0.05, N≥10), although RET and UCB levels were significantly elevated in HS (8.27±1.07 % and 1.72±0.57 mg/dL, P<0.01, N≥13), but not SHS (2.80±0.17 % and 0.99±0.66 mg/dL, N≥11), perhaps indicating a redistribution of extravascular hemolysis (EHV) to low-level IVH in SHS. Surprisingly, plasma heme was not significantly elevated in PNH individuals studied (36.5±4.0 µM, N≥10), even though concentrations of plasma LDH were the highest in this patient group (1196±305 U/L; compared to 182±8, 352±73, 407±38. 211±19 and 182±28 U/L for CON, SCA, SCAHU, HS, SHS, respectively, P<0.01; N≥10) and LDH correlated significantly with plasma heme in this group (r=0.905, P<0.01 for PNH). RET and UCB presented and intermediate values in PNH (5.35±1.21% and 0.84±0.18 mg/dL, P<0.05, N≥10, compared to CON). To investigate whether hemolytic profiles may be modulated by the Hb and heme scavengers, Hp and Hpx, we measured these proteins in the circulations of patients. Hp was significantly depleted in SCA, SCAHU, HS and PNH (1.0±0.4, 1.0±0.6, 37.8±0.16, 12.3±10.2, respectively, compared to 142.9±20.6 mg/dL in CON, p<0.05; N≥10), while SHS presented similar levels to those of CON (189.9±38.8 mg/dL, N=5). In contrast, somewhat surprisingly, Hpx levels varied considerably in each patient group, with a significant depletion only observed in the SHS group (12.2±0.3, 15.9±5.4, 15.0±3.8, 6.0±1.5, 1.5±0.4, 17.8±5.0mg/dL for CON, SCA, SCAHU, HS, SHS, PNH, respectively; P<0.05, SHS compared to CON, N≥10). As such, plasma heme was slightly lower in PNH than in SCA, in association with very high plasma LDH, suggesting that either IVH is slightly less severe or that Hb/heme scavenging is more efficient in the PNH population studied than in SCA. While EHV may be abolished by splenectomy in HS, intermediate heme levels may suggest that some IVH, or reduced heme catabolism, may still occur in some of these patients. Interestingly, depleted Hp stores were coupled with maintained (or even elevated) Hpx levels in SCA and PNH, indicating that hemolytic processes may stimulate Hpx production, potentially via a heme-oxygenase-1 (HO-1)-dependent pathway. Furthermore, significantly diminished levels of Hpx in SHS suggest a role for the spleen in Hpx maintenance, possibly due to the importance of this organ for HO-1 activity. In conclusion, while parameters suggestive of hemolysis can be observed in SCA, HS and PNH, specific profiles of hemolytic markers and heme catabolism indicate that hemolytic events may be modulated differently in these diseases. DisclosuresConran:Bayer AG: Research Funding. Almeida:Jassen & Cilag: Other: Currently employed with.

Topological Structures and Membrane Nanostructures of Erythrocytes after Splenectomy in Hereditary Spherocytosis Patients via Atomic Force Microscopy.

Hereditary spherocytosis is an inherited red blood cell membrane disorder resulting from mutations of genes encoding erythrocyte membrane and cytoskeletal proteins. Few equipments can observe the structural characteristics of hereditary spherocytosis directly expect for atomic force microscopy In our study, we proved atomic force microscopy is a powerful and sensitive instrument to describe the characteristics of hereditary spherocytosis. Erythrocytes from hereditary spherocytosis patients were small spheroidal, lacking a well-organized lattice on the cell membrane, with smaller cell surface particles and had reduced valley to peak distance and average cell membrane roughness vs. those from healthy individuals. These observations indicated defects in the certain cell membrane structural proteins such as α- and β-spectrin, ankyrin, etc. Until now, splenectomy is still the most effective treatment for symptoms relief for hereditary spherocytosis. In this study, we further solved the mysteries of membrane nanostructure changes of erythrocytes before and after splenectomy in hereditary spherocytosis by atomic force microscopy. After splenectomy, the cells were larger, but still spheroidal-shaped. The membrane ultrastructure was disorganized and characterized by a reduced surface particle size and lower than normal Ra values. These observations indicated that although splenectomy can effectively relieve the symptoms of hereditary spherocytosis, it has little effect on correction of cytoskeletal membrane defects of hereditary spherocytosis. We concluded that atomic force microscopy is a powerful tool to investigate the pathophysiological mechanisms of hereditary spherocytosis and to monitor treatment efficacy in clinical practices. To the best of our knowledge, this is the first report to study hereditary spherocytosis with atomic force microscopy and offers important mechanistic insight into the underlying role of splenectomy.

Prophylactic cholecystectomy during abdominal surgery

The presence of asymptomatic gallstones is no longer an indication for elective prophylactic cholecystectomy (PC) according to the recommendations of the 1991 French Consensus Conference on cholelithiasis. However, there may be potential benefits of performing prophylactic cholecystectomy during certain abdominal procedures for non-biliary disease; this remains a subject of debate. This debate has become livelier with the recent increase in bariatric surgery. Gastrectomy for cancer, small bowel resection, colonic resection, and splenectomy for hereditary spherocytosis as well as all bariatric surgical interventions can all alter the physiology of gallstone disease raising the question of the value of PC, but the specific morbidity of cholecystectomy must be kept in mind. The purpose of this study was to report epidemiological and pathophysiological data and the results from literature reports in order to assess the value of concomitant prophylactic cholecystectomy during various common surgical situations.

Splenectomy in Children With “Mild” Hereditary Spherocytosis

Splenectomy is considered the treatment of choice for patients with "symptomatic" hereditary spherocytosis (HS). Published guidelines offering recommendations for splenectomy in HS categorize patients primarily based on hemoglobin concentration. We performed a retrospective review of 64 children having splenectomy for HS at Children's Medical Center Dallas. On the basis of hemoglobin concentration alone, 16 children (25%) had mild, 38 (59%) moderate, and 10 (16%) severe HS. However, when reticulocyte count was used to categorize disease severity, only 3 patients (5%) having splenectomy would be considered mild, 17 (27%) moderate, and 42 (66%) severe. Despite otherwise having "mild" disease defined by near-normal hemoglobin levels, many children with nontraditional or subjective signs and symptoms related to hemolytic rate received a splenectomy, and, therefore, reticulocyte count might be a more reliable laboratory marker suggesting consideration of splenectomy. A validated assessment tool incorporating quality of life indicators in addition to the traditional medical indications for splenectomy in HS would be valuable in assessing indications for the procedure.

Laparoscopic versus robotic subtotal splenectomy in hereditary spherocytosis. Potential advantages and limits of an expensive approach

This study was designed to compare the laparoscopic subtotal splenectomy with the robotic approach in patients with hereditary spherocytosis. Thirty-two consecutive subtotal splenectomies by minimal approach in patients with hereditary spherocytosis were analyzed (10 robotic vs. 22 laparoscopic subtotal splenectomies). A significant difference was found for the robotic approach regarding blood loss, vascular dissection duration, and splenic remnant size. Follow-up for 4-103 months was available. Subtotal splenectomy seems to be a suitable candidate for robotic surgery, requiring a delicate dissection of the splenic vessels and a correct intraoperative evaluation of the splenic remnant. Robotic subtotal splenectomy is comparable to laparoscopy in terms of hospital stay and complication. The main benefits are lower blood loss rate, vascular dissection time, and a better evaluation of the splenic remnant volume.

Splenectomy for hereditary spherocytosis: complete, partial or not at all?

Hereditary spherocytosis (HS) is an hematologic disorder, common in the Northern European population, which is characterized by hemolytic anemia and splenomegaly. The pathophysiological bedrock of HS consists of the retention and damage of abnormal red cells, caused by an intrinsic erythrocyte membrane defect. Hemolysis within the spleen is the main detrminant of erythrocyte destruction in patients with HS. Splenectomy removes the primary ‘graveyard’ for spherocytes and, thus, eliminates anemia and hyperbilirubinemia and lowers the high reticulocyte number to nearly normal levels. Spleen removal is an effective therapeutic option but it is not without risks. The incidence and public health impact of spleen surgery in children with HS is substantial. Unfortunately, splenectomy is sometimes recommended erroneously for young children who have a mild clinical phenotype or is performed without relying on careful assessment of the risks and benefits. This article aims to present criticism and address controversy about HS management. Unclear and still controversial issues are tackled according to the author’s personal viewpoint, because strong evidence in different aspects of HS management is lacking.

Traditional laboratory measures of cardiovascular risk in hereditary spherocytosis

Individuals who have undergone splenectomy may be at an increased risk of arterial and venous thrombosis. We sought to determine if splenectomy affects surrogate laboratory measures of cardiovascular risk in persons with hereditary spherocytosis (HS). We conducted a prospective cross-sectional study of 21 children and 36 adults with HS. Fasting blood samples were collected for complete blood count and plasma lipid panel, homocysteine, lipoprotein (a), C-reactive protein, and fibrinogen. The variables were compared between the groups with and without prior splenectomy by Mann-Whitney tests. Subjects with prior splenectomy had higher hemoglobin, white blood cell and platelet counts and lower reticulocyte counts and total serum bilirubin concentrations (P < 0.001). Subjects not having had splenectomy had lower than normal levels of total and LDL-cholesterol (LDL-C). Total and LDL-C values were significantly higher, as were fibrinogen and homocysteine concentrations, in the post-splenectomy subjects than in individuals with intact spleen. Various lipid levels and other measures of cardiovascular risk are affected by splenectomy in persons with HS. Further investigations are indicated to more clearly define the balance of the potential benefits of hemolysis and anemia versus the deleterious effects of splenectomy in HS.

Pulmonary Thromboembolism Following Splenectomy in Hereditary Spherocytosis

Hereditary spherocytosis is an inherited disorder characterized by presence of spherocytes. It usually manifests clinically as triad of anaemia, splenomegaly and jaundice. The recommended treatment in a symptomatic patient is splenectomy. Occurrence of pulmonary thromboembolism in hereditary spherocytosis following splenectomy is unusual. We report the case of a 30 year old male who underwent splenectomy for hereditary spherocytosis and subsequently developed pulmonary thromboembolism and right ventricular mural thrombosis six years later. His showed no other procoagulant disorders. The development of pulmonary thromboembolism and right ventricular mural thrombosis in a patient of hereditary spherocytosis after splenectomy is an interesting association. doi:10.4021/jmc2010.06.102e

Pulmonary Thromboembolism Following Splenectomy in Hereditary Spherocytosis

Hereditary spherocytosis is an inherited disorder characterized by presence of spherocytes. It usually manifests clinically as triad of anaemia, splenomegaly and jaundice. The recommended treatment in a symptomatic patient is splenectomy. Occurrence of pulmonary thromboembolism in hereditary spherocytosis following splenectomy is unusual. We report the case of a 30-year-old male who underwent splenectomy for hereditary spherocytosis and subsequently developed pulmonary thromboembolism and right ventricular mural thrombosis six years later. His showed no other procoagulant disorders. The development of pulmonary thromboembolism and right ventricular mural thrombosis in a patient of hereditary spherocytosis after splenectomy is an interesting association. J Med Cases. 2010;1(1):14-15 doi: https://doi.org/10.4021/jmc2010.06.102e

Medical and Quality of Life Indications for Splenectomy in Children with Hereditary Spherocytosis.

Abstract 5082 BackgroundSplenectomy has been considered the treatment of choice for patients with “symptomatic” hereditary spherocytosis (HS). However, given the long-term risks associated with asplenia, including sepsis and thrombosis, the risks and benefits of splenectomy in HS require ongoing analysis. To our knowledge, no comprehensive institutional review summarizing the medical and quality of life indications for splenectomy in children with HS has been reported to date. MethodsA retrospective record review was performed of 64 children, age ' 18 yrs, having splenectomy for HS at Children's Medical Center Dallas from January 1973 through March 2009. The following patient characteristics were abstracted: age at splenectomy, family history of HS and/or splenectomy, baseline hemoglobin concentration and reticulocyte count prior to splenectomy, degree of splenomegaly, total number of prior transfusions, hyperhemolytic events, and aplastic crisis. Patients were classified based on mean hemoglobin concentration as having mild (≥11.0 g/dL), moderate (8.0-10.9 g/dL), or severe HS ('7.9 g/dL) (Bolton-Maggs PH, et al. Br J Haematol 2004;126:455-74). The primary indication for splenectomy was characterized in each case as either a “quality of life (QOL) indication” or a “medical indication.” QOL indicators included jaundice, pallor, maxillary hyperplasia, poor growth, fatigue, exercise intolerance, and family history of splenectomy for HS which, having been deemed successful, fostered parental preference for splenectomy in their child. Medical indicators included baseline hemoglobin approaching transfusion-dependent threshold, multiple or severe hyperhemolytic events, severe aplastic crisis, symptomatic gallbladder disease, marked splenomegaly, and the need for numerous transfusions. Some patients had both medical and QOL indications but only one was designated as the primary indication for splenectomy based on documentation in the medical record. ResultsWhen classifying HS patients by hemoglobin concentration, 16 patients (25%) were mild, 38 (59%) moderate, and 10 (16%) severe. The following table summarizes the patient characteristics.Patient CharacteristicsMild HS (n=16)Moderate HS (n=38)Severe HS (n=10)Median age at splenectomy, yr11.410.34.7Family history of HS, n (%)16 (100%)25 (65.8%)6 (60%)Family history of splenectomy, n (%)14 (87.5%)18 (47.4%)5 (50%)Pre-splenectomy: Hemoglobin level (g/dL), median (range)11.6 (11.0-13.7)9.7 (8.2-10.9)7.4 (5.0-7.9)Reticulocyte count (%), median (range)9.8 (3.9-28.7)11.7 (1.1-21.1)12.4 (6.5-25.3)Splenomegaly (cm), median (range)3.5 (0-7.0)4.0 (0-12.0)4.7 (2.0-8.0)Despite having just mild HS, 16 patients had splenectomy, 6 for QOL indications. Moreover, 11 of 38 moderate HS patients had splenectomy for a primary QOL indication. Medical indications predominated in all 10 patients with severe HS. Of the 17 patients with QOL indications who had splenectomy, 8 (47%) also had documentation of medical indications. Among 45 patients with primary medical indications for splenectomy, 18 (40%) also had QOL issues contributing to the decision. Two patients' indications for splenectomy were unclear. ConclusionsAlthough splenectomy is generally felt to be unnecessary in patients with mild HS and many with moderate HS as well, our experience demonstrates that splenectomy is often performed primarily for QOL indications in both risk categories. A validated assessment tool that incorporates QOL indicators in addition to the traditional medical reasons for splenectomy in patients with HS would be of great value in an evidence-based assessment of the patient's need for splenectomy. We recommend inclusion of QOL indicators when considering splenectomy in patients with mild and moderate HS. DisclosuresNo relevant conflicts of interest to declare.

Risks and benefits of splenectomy versus no splenectomy for hereditary spherocytosis – a personal view

Splenectomy is indicated in hereditary spherocytosis to relieve symptoms due to anaemia or splenomegaly, reverse growth failure or skeletal changes due to over-robust erythropoiesis, and prevent recurrent gallstones. A life-long risk of bacterial infection has been recognised for many years as a concomitant cost of splenectomy. The scope of this risk has expanded to include a number of organisms beyond the triad of pneumococcus, meningococcus, and haemophilus influenzae. Recently, it has been demonstrated that splenectomy also confers a significant risk of delayed adverse vascular events in patients with hereditary spherocytosis, just as it does in patients undergoing splenectomy for other indications. Further, these same studies demonstrated a benefit of avoiding splenectomy: hereditary spherocytosis patients with a spleen have significantly fewer adverse vascular events than unaffected family members, probably because of the protective effect of chronic, mild anaemia. Accordingly, this review marshals the evidence favouring a conservative approach to splenectomy in spherocytosis.

Splenectomy in hereditary spherocytosis: Review of 1,657 patients and application of the pediatric quality indicators

The objective of the present study is to profile the outcome and safety of pediatric patients undergoing splenectomy with hereditary spherocytosis (HS) using a nationwide sample and the Agency for Healthcare Research and Quality (AHRQ) Pediatric Quality Indicators (PDIs). A retrospective cross-sectional descriptive analysis of a non-overlapping combination of the National Inpatient Sample (NIS), and Kids' Inpatient Database (KID) databases (1988-2004) were performed. These combined databases contain information from nearly 93 million discharges in the United States. Children with an age at admission of <18 years of age and HS (ICD-9 diagnosis code of 282.0) who underwent total splenectomy (ICD-9 procedure code of 41.5) were identified. Variables of gender, race, co-existing diagnoses, hospital type, and charges adjusted to 2006 dollars, length of stay, inpatient mortality, and complications were collected. PDIs were identified for each patient by linking the data obtained from the NIS and KID databases with the PDIs using the AHRQ Quality Indicators Wizard. Splenectomy for HS was associated with low morbidity and mortality. Accompanying cholecystectomy and/or appendectomy appeared to be safely performed at the same operation. Of the 13 PDIs identified by AHRQ as potentially avoidable adverse events, none were observed to occur in more than 1% of the patients. Based on the results of this study, splenectomy in patients with HS appears safe and to result in a minimal number of potentially preventable complications as identified by the AHRQ PDIs. We have successfully demonstrated use of the indicators to aid in the analysis of a specific surgical procedure within a subset of the pediatric population.

Partial Splenectomy for Hereditary Spherocytosis—What Do We Know and What Do We Need to Know?

Partial splenectomy is an alternative to total splenctomy for the treatment of children with hereditary spherocytosis (HS) and other congenital hemolytic anemias. Total splenectomy effectively controls the symptoms of the disease, but places children at risk for overwhelming postsplenectomy sepsis (OPSI), as well as other severe complications. A growing body of data suggests that partial splenectomy provides acceptable hematological outcomes compared with total splenectomy while preserving splenic function. In this article we briefly review the rationale for partial splenectomy, parameters to evaluate splenic function, and recent clinical data on outcomes after partial splenectomy. Although many questions remain regarding the role of partial splenectomy, it may be a promising alternative to total splenectomy in children with HS and other congential hemolytic anemias.

Delayed adverse vascular events after splenectomy in hereditary spherocytosis.

It is probable that the variety and frequency of delayed adverse vascular events after splenectomy are underappreciated. Splenectomy is performed for a wide variety of conditions, and delayed postsplenectomy hazards are not often studied. To estimate the relative risk of adverse vascular events in members of hereditary spherocytosis families who have or have not had a splenectomy. Members of families in which hereditary spherocytosis exists were systematically questioned about adverse vascular events. The cumulative incidence of arterial and venous events at age 70 years was greater in persons who had undergone a splenectomy for spherocytosis (arterial, 22% females, 32% males; venous, 20% females, 19% males) than in affected persons who did not undergo splenectomy (arterial, 3% females, 2% males; venous, 6% females, 4% males) or non-affected family members (arterial, 10% females, 17% males; venous, 4% females, 12% males). Affected subjects who undergo splenectomy are at greatly increased risk of arterial events as compared to affected subjects who do not undergo splenectomy [arterial, hazard ratio (HR) 7.2, 95% confidence interval (CI) 2.8-17.2; venous, HR 3.3, 95% CI 1.1-9.8]. There is a significant, long-lasting, increased risk of adverse arterial and venous thromboembolic events after splenectomy performed for hereditary spherocytosis. A review of the literature indicates that this is also true when splenectomy is performed for several other indications.

A laparoscopic approach to partial splenectomy for children with hereditary spherocytosis

Partial splenectomy is sometimes used for children with hereditary spherocytosis (HS) to reduce hemolysis while retaining some splenic immune function. Previous reports have described a partial splenic resection through a laparotomy incision. Whereas laparoscopic total splenectomy for HS is well-established, laparoscopic partial splenectomy (LPS) has not been described. The authors have developed a novel LPS technique that combines the benefits of partial splenectomy with those of a laparoscopic approach. A chart review was conducted for three children with HS who underwent LPS, with approximately one-fourth of the spleen left on the basis of the short gastric arterial supply. The mean preoperative spleen size was 17.6 cm. The mean preoperative hemoglobin count was 100 g/l, and the postoperative hemoglobin count was 133 g/l. All three patients reported reduced malaise and increased energy levels. There was no recurrent anemia at the 1- to 2-year follow-up evaluation. The LPS procedure is a safe and effective approach to HS that resolves anemia, potentially retains some splenic immunity, and confers the benefits of a minimal access technique.

Near‐total splenectomy for hereditary spherocytosis: clinical prospects in relation to disease severity

We prospectively studied the efficacy of near total splenectomy (NTS) for managing hereditary spherocytosis (HS) based on haemoglobin (Hb), total bilirubin and splenic remnant regrowth in 30 children receiving NTS for HS between November 1996 and December 2004 (mean followup 3.6 years). Patients were classified into three severity groups. At followup, mean Hb had increased by 2.9-5.0 g/dl (P = 0.110) and bilirubin dropped by 15.4-56.4 mumol/l. Remnant regrowth was moderate, within the weight-specific norm. Amongst spleen-preserving techniques, NTS shows markedly lower rates of recurrent haemolysis, remnant regrowth and secondary operations, thus potentially benefiting all clinical forms of HS.