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Chirurgie (Heidelberg, Germany) Dec 2023Splenic surgery in hematological disorders requires a well-weighted decision on the indications because the medical treatment has rapidly changed in recent years due to... (Review)
Review
BACKGROUND
Splenic surgery in hematological disorders requires a well-weighted decision on the indications because the medical treatment has rapidly changed in recent years due to new pharmaceutical approaches.
OBJECTIVE
Summary of the indications, surgical procedures and perioperative management regarding operative interventions on the spleen in hematological disorders.
MATERIAL AND METHODS
Selective literature search and summary of reviews and guideline recommendations.
RESULTS
In hematological disorders surgical procedures of the spleen (splenectomy and partial splenectomy) are an important part of the repertoire in the treatment. In recent years the indications for surgery have become narrower because of new forms of medicinal treatment. Especially in hereditary spherocytosis, immune thrombocytopenia and symptomatic splenomegaly and hypersplenism it is still of importance. The minimally invasive splenectomy is regarded as the gold standard. The spleen has an important immune and sequestration function, therefore preoperative and postoperative infectious and thromboembolic events have to be anticipated and prevented. A close interdisciplinary cooperation with hematologists is essential for an optimal outcome of patients.
CONCLUSION
The minimally invasive splenectomy and partial splenectomy are part of the surgical repertoire in the diagnostics and treatment of hematological disorders. Because of novel medicinal approaches the therapeutic protocols are continuously changing. A close cooperation with hematologists is important for the optimal evaluation of the indications and the perioperative management.
Topics: Humans; Treatment Outcome; Spleen; Hematologic Diseases; Splenectomy; Splenomegaly
PubMed: 37947801
DOI: 10.1007/s00104-023-01979-7 -
Frontiers in Immunology 2023Elective splenectomy is the main treatment for a wide range of haematological diseases. Porto-spleno-mesenteric venous thrombosis represents one of the most severe...
BACKGROUND
Elective splenectomy is the main treatment for a wide range of haematological diseases. Porto-spleno-mesenteric venous thrombosis represents one of the most severe complications of this procedure. The aim of this study was to evaluate risk factors associated with development of porto-spleno-mesenteric venous thrombosis after elective splenectomy.
METHODS
All cases of elective splenectomy carried out from April 1 2017 to January 31 2023 were included in this single centre retrospective cohort study. Patients' demographics and perioperative data were analysed and correlated with the incidence of postoperative thrombosis. All patients underwent postoperative doppler ultrasound screening for thrombosis. Analysis was performed using SPSS 28, with p-value < 0.05 considered significant.
RESULTS
Twenty-two patients (10 women, 12 men) underwent splenectomy during the study period. Indications were: immune thrombocytopenia (n: 6), myeloproliferative disorder (n: 6), hereditary spherocytosis (n: 4), thalassemia (n: 1), lymphoma (n: 1), leukaemia (n: 1), other malignancies (n: 3). Six patients developed porto-spleno-mesenteric venous thrombosis and only 2 of them were symptomatic. Patients were treated with anticoagulation therapy with complete resolution. Analysis identified three main factors associated with thrombosis: spleen diameter (p = 0.03), myeloproliferative disorder (p = 0.02), intraoperative platelet transfusion (p = 0.002) and intraoperative red blood cells transfusion (p = 0.009).
CONCLUSION
Standardized postoperative screening allows prompt diagnosis and treatment of porto-spleno-mesenteric venous thrombosis even in asymptomatic cases. Patient with splenomegaly and affected by myeloproliferative disorder have a greater risk to develop this complication.
Topics: Male; Humans; Female; Spleen; Splenectomy; Retrospective Studies; Venous Thrombosis; Thrombosis; Myeloproliferative Disorders
PubMed: 37928557
DOI: 10.3389/fimmu.2023.1216283 -
Frontiers in Pediatrics 2023Hereditary Spherocytosis (HS) is a rare, congenital red blood cell disorder presenting with variable clinical manifestations ranging from mild hemolytic anemia to severe...
Hematological characteristics and hepatobiliary complications of hereditary spherocytosis in a tertiary care pediatric center: optimizing diagnosis and care through local and international networks.
BACKGROUND
Hereditary Spherocytosis (HS) is a rare, congenital red blood cell disorder presenting with variable clinical manifestations ranging from mild hemolytic anemia to severe anemia with hypersplenism and hepatobiliary complications.
METHODS
The objectives of the study were to evaluate the diagnostic pathway of HS, the presence and management of hepatobiliary complications in pediatric patients with HS followed in a tertiary care center. The demographic, clinical, hematological information were retrieved from medical records of patients having at least 1 hematology visit between 2010 and 2020.
RESULTS
Forty-two patients were enrolled, 23 M. Mean age at onset of symptoms was 2.8 years, at diagnosis was 3.5 years. Anemia was the first manifestation in 73%; suspect of HS arose for all patients in first or second level outpatient clinics. Only 64% of patients performed two confirmation tests in the reference center. 28/42 had familiarity for HS; of the 13/42 who did not, only 47% performed further analysis. Sixteen patients developed gallbladder stones (40%), visible at the first ultrasound (5.6 years). Hemolytic crises and parvovirus infections were more frequent in patients with stones (53.6% vs. 26.1% and 63.6% vs. 28.6%, respectively). 10/16 (62.5%) underwent elective cholecystectomy: 8 had concomitant splenectomy.
CONCLUSIONS
our study highlights the need to optimize the diagnostic pathway in networks of care involving general and specialized centers in order to reduce time to diagnosis and ensure that all patients receive confirmatory tests. A high frequency of hepatobiliary complications since young age was observed suggesting that screening with ultrasound should begin earlier.
PubMed: 37886235
DOI: 10.3389/fped.2023.1269645 -
Zhonghua Gan Zang Bing Za Zhi =... Sep 2023To understand the clinical and genetic characteristics of hereditary spherocytosis (HS) combined with cholestasis among pediatric patients. 12 cases of HS children...
To understand the clinical and genetic characteristics of hereditary spherocytosis (HS) combined with cholestasis among pediatric patients. 12 cases of HS children accompanied by cholestasis at Hunan Children's Hospital were selected as the research subjects between January 2013 and December 2022. Clinical data were collected. Whole-exome sequencing was performed by second-generation sequencing. Suspected pathogenic mutation sites were verified by Sanger sequencing. All pediatric patients were admitted to the hospital due to their yellow skin tone. Eight cases (66.67%) had a positive family history. The clinical manifestations were jaundice, splenomegaly (12/12), abdominal pain, anemia (4/12), and hepatomegaly (5/12). All pediatric patients had decreased hemoglobin, an increased reticulocyte ratio, total bilirubin and direct bilirubin, a positive erythrocyte fragility test, and remarkable spherical erythrocytes in their peripheral blood. Seven cases had elevated aminotransferase; four cases had severely elevated aminotransferase and bilirubin; eight cases had biliary calculi; and two cases had a dilated biliary tract. Liver pathological examination showed mild damage to the liver cells (G1S1) in three pediatric cases. Five children had a total of six unreported mutations: gene c.2431_2450del, c.4974-2A > G, c.2575G > A, and exon 22-35 deletion; gene: c.2379-2380delC; and c .6dupC. Children still had abnormal bilirubin levels following treatment. Two pediatric cases underwent splenectomy. Bilirubin and hemoglobin levels returned to normal after surgery. Children with HS may experience cholestasis, and those with poor treatment results may consider undergoing a splenectomy. Six new types of variants have expanded the HS gene mutation spectrum.
Topics: Humans; Child; Spherocytosis, Hereditary; Mutation; Cholestasis; Bilirubin; Transaminases; Hemoglobins
PubMed: 37872089
DOI: 10.3760/cma.j.cn501113-20230210-00048 -
Molecular Imaging and Radionuclide... Oct 2023A 7-year-old boy with known diagnosis of hereditary spherocytosis and ulcerative colitis was referred for F-fluorodeoxyglucose (F-FDG) positron emission...
A 7-year-old boy with known diagnosis of hereditary spherocytosis and ulcerative colitis was referred for F-fluorodeoxyglucose (F-FDG) positron emission tomography/computed tomography after detection of a 28 mm lesion suspicious for malignancy in spleen on upper abdomen magnetic resonance imaging (MRI). As an incidental finding, a moderately increased uptake of F-FDG was observed in periportal region with no definable mass. MRI revealed compatible findings with "periportal cuffing" as described on ultrasonography.
PubMed: 37870391
DOI: 10.4274/mirt.galenos.2023.53254 -
Journal of Pediatric Surgery Jan 2024Children undergoing splenectomy for hemolytic anemia often have cholelithiasis, which may or may not be symptomatic. It is unclear whether concurrent cholecystectomy...
PURPOSE
Children undergoing splenectomy for hemolytic anemia often have cholelithiasis, which may or may not be symptomatic. It is unclear whether concurrent cholecystectomy increases length of stay or morbidity after splenectomy. The purpose of this study was to compare morbidity among children undergoing laparoscopic splenectomy alone versus splenectomy with concurrent cholecystectomy in patients with hemolytic anemia.
METHODS
We retrospectively evaluated children with hemolytic anemia undergoing non-traumatic laparoscopic splenectomy in the National Surgical Quality Improvement Program-Pediatric database (2012-2020). Outcomes were compared for patients undergoing splenectomy alone (n = 1010) versus splenectomy with cholecystectomy (n = 371). Pearson's Chi-square and Student's t-tests were utilized as appropriate. Propensity score-matching was completed, controlling for eight demographic and clinical variables.
RESULTS
1381 patients were identified, 73.1% undergoing splenectomy alone and 26.9% splenectomy with cholecystectomy. Splenectomy with cholecystectomy patients were older (10.9 years vs. 8.4 years, p < 0.01), more likely to have hereditary spherocytosis (56.1% vs. 40.8%, p < 0.01), less likely to have sickle cell disease (12.1% vs. 33.5%, p < 0.01), more likely ASA class 1 or 2 (49.3% vs. 42.1%, p < 0.01), and had similar preoperative hematocrit levels (29.6 vs. 29.3, p = 0.33). The splenectomy with cholecystectomy group was less likely to receive preoperative blood transfusions (13.5% vs. 25.4%, p < 0.01). There were 360 pairs selected on propensity score-matching, and splenectomy with cholecystectomy was associated with increased operative time (182 min vs. 145 min, p < 0.01) and decreased occurrences of a postoperative transfusion (4.2% vs. 8.9%, p = 0.01). Length of stay after surgery (2.5 days vs. 2.3 days, p = 0.13), composite morbidity (3.9% vs. 3.4%, p = 0.69), and 30-day readmission rates (3.3% vs. 7.4%, p = 0.08) were all similar.
CONCLUSIONS
Splenectomy with cholecystectomy is associated with similar postoperative morbidity, length of stay and readmission rates compared to splenectomy alone. These data support the safety of concurrent cholecystectomy with splenectomy for children with cholelithiasis in the setting of hemolytic anemia.
TYPE OF STUDY
Retrospective Cohort Study.
LEVEL OF EVIDENCE
Level III.
Topics: Humans; Child; Retrospective Studies; Splenectomy; Cholecystectomy; Anemia, Hemolytic; Laparoscopy; Morbidity; Cholelithiasis; Postoperative Complications; Cholecystectomy, Laparoscopic
PubMed: 37833213
DOI: 10.1016/j.jpedsurg.2023.09.010 -
Frontiers in Genetics 2023Hereditary spherocytosis (HS), the most common inherited hemolytic anemia disorder, is characterized by osmotically fragile microspherocytic red cells with a reduced...
Hereditary spherocytosis (HS), the most common inherited hemolytic anemia disorder, is characterized by osmotically fragile microspherocytic red cells with a reduced surface area on the peripheral blood smear. Pathogenic variants in five erythrocyte membrane structure-related genes (Spherocytosis, type 1; MIM#182900), (Spherocytosis, type 2; MIM#616649), (Spherocytosis, type 3; MIM#270970), (Spherocytosis, type 4; MIM#612653) and (Spherocytosis, type 5; MIM#612690) have been confirmed to be related to HS. There have been many studies on the pathogenic variants and mechanisms of HS, however, studies on how to manage the transmission of HS to the next-generation have not been reported. In this study, we recruited a patient with HS. Targeted next-generation sequencing with a panel of 208 genes related to blood system diseases detected a novel heterozygous variant in the : c.300+2dup in the proband. Sanger sequencing of variant alleles and haplotype linkage analysis of single nucleotide polymorphism (SNP) based on next-generation sequencing were performed simultaneously. Five embryos were identified with one heterozygous and four not carrying the variant. Single-cell amplification and whole genome sequencing showed that three embryos had varying degrees of trisomy mosaicism. One of two normal embryos was transferred to the proband. Ultimately, a healthy boy was born, confirmed by noninvasive prenatal testing for monogenic conditions (NIPT-M) to be disease-free. This confirmed our successful application of PGT in preventing transmission of the pathogenic variant allele in the HS family.
PubMed: 37795245
DOI: 10.3389/fgene.2023.1221853 -
Microscopy and Microanalysis : the... Apr 2023In hereditary spherocytosis (HS), genetic mutations in the cell membrane and cytoskeleton proteins cause structural defects in red blood cells (RBCs). As a result, cells...
In hereditary spherocytosis (HS), genetic mutations in the cell membrane and cytoskeleton proteins cause structural defects in red blood cells (RBCs). As a result, cells are rigid and misshapen, usually with a characteristic spherical form (spherocytes), too stiff to circulate through microcirculation regions, so they are prone to undergo hemolysis and phagocytosis by splenic macrophages. Mild to severe anemia arises in HS, and other derived symptoms like splenomegaly, jaundice, and cholelithiasis. Although abnormally shaped RBCs can be identified under conventional light microscopy, HS diagnosis relies on several clinical factors and sometimes on the results of complex molecular testing. It is specially challenging when other causes of anemia coexist or after recent blood transfusions. We propose two different approaches to characterize RBCs in HS: (i) an immunofluorescence assay targeting protein band 3, which is affected in most HS cases and (ii) a three-dimensional morphology assay, with living cells, staining the membrane with fluorescent dyes. Confocal laser scanning microscopy (CLSM) was used to carry out both assays, and in order to complement the latter, a software was developed for the automated detection of spherocytes in blood samples. CLSM allowed the precise and unambiguous assessment of cell shape and protein expression.
Topics: Membrane Proteins; Erythrocytes; Microscopy, Confocal; Cell Membrane; Cell Shape
PubMed: 37749743
DOI: 10.1093/micmic/ozac055 -
Cureus Aug 2023Introduction The utilization of robot-assisted technique for splenectomy has recently gained popularity especially in patients undergoing splenectomy for hematological...
Introduction The utilization of robot-assisted technique for splenectomy has recently gained popularity especially in patients undergoing splenectomy for hematological indications owing to its magnification of and easy manipulation of internal abdominal organs. Moreover, robotic splenectomy emerged as an essential teaching module before approaching more complex robotic procedures. Methods A total of 43 elective splenectomies were performed for hematological indications in Department of Surgical Gastroenterology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER) between January 2018 to July 2023 of which 14 patients underwent robotic splenectomy. All patients underwent lateral approach of robotic splenectomy with a modification of avoiding the lesser sac dissection. Prospectively maintained data were retrospectively analyzed and results were recorded in terms of intra-operative time taken, blood loss, need for blood and blood product transfusion and postoperative morbidity and mortality. Results The indications for patients who underwent robotic splenectomy include idiopathic thrombocytopenic purpura in eight patients, autoimmune hemolytic anemia in three patients, Evans syndrome in one patient and hereditary spherocytosis in two patients. The median splenic diameter was 14.8cm and the median platelet count before the operation was 10,800 cells/cubic millimeter (7000-3,20,000). The mean operative time was 92 minutes and blood loss was 40ml. The median duration of hospital stay was 2.4 days. All 14 patients had therapeutic success and there was no procedure-related mortality or morbidity. Conclusion Robotic splenectomy using the modified lateral approach can safely be performed with comparable operative time, blood loss and overall morbidity. However further studies are mandatory to confirm the advantage of this modified technique of lateral approach of robotic splenectomy.
PubMed: 37736466
DOI: 10.7759/cureus.43820 -
European Journal of Haematology Nov 2023Subtotal or total splenectomy are recommended in severe and should be considered in intermediate forms of hereditary spherocytosis (HS). Data on laparoscopic subtotal...
INTRODUCTION
Subtotal or total splenectomy are recommended in severe and should be considered in intermediate forms of hereditary spherocytosis (HS). Data on laparoscopic subtotal splenectomy (LSTS) in HS patients are sparse.
METHODS
Thirty three patients with HS (median age 10.7 years (yrs), range 1.8-15.5) underwent LSTS. Baseline and follow-up investigation included haematological parameters, microscopic analysis of pitted erythrocytes (pitE), and B-cell subpopulations assessed by flow cytometry. Results were compared to those of non-splenectomised HS patients, HS patients after total splenectomy (TS), and healthy individuals.
RESULTS
After LSTS, haemoglobin levels were normalised in all patients. During median long-term follow-up of 3.9 yrs (range 1.1-14.9), only four patients presented mild anaemia. Despite re-growing of the remnant spleen none of the patients required a second surgical intervention. As compared to TS, PitE in LSTS patients were significantly lower and indicated normal to only moderately decreased spleen function. Relative but not absolute IgM memory B-cell counts were reduced in both LSTS and TS patients.
CONCLUSIONS
LSTS is effective for the treatment of patients with HS. A small remnant spleen is sufficient to provide adequate phagocytic function and to induce a pool of IgM memory B-cells.
Topics: Humans; Child; Splenectomy; Spleen; Spherocytosis, Hereditary; Laparoscopy; Immunoglobulin M
PubMed: 37700575
DOI: 10.1111/ejh.14077