Uncommon Presentation of Hypersplenism in Adult Sickle Cell Disease Patients: A Rare Case Report.

BACKGROUND Hypersplenism, the rapid and premature destruction of blood cells, encompasses a triad of splenomegaly, cytopenias (anemia, leukopenia, or thrombocytopenia), and compensatory bone marrow proliferation. Secondary hypersplenism results from non-intrinsic splenic diseases, such as hemoglobinopathies. Sickle cell disease consists of a group of genotypes, where hemoglobin sickle C disease (HbSC) is the inheritance of hemoglobin S with hemoglobin C. Most homozygous genotypes undergo complete auto-splenectomy by age 6 years, whereas those with HbSC disease rarely do. We report a rare case of hypersplenism and massive splenomegaly in an adult with sickle cell disease, the HbSC genotype, requiring splenectomy. CASE REPORT A 41-year-old woman with known splenomegaly initially presented to the general surgery clinic for management of abdominal pain. She was found to have anemia, indicating cytopenia likely from hypersplenism. Consequently, she underwent splenic artery embolization, followed by an exploratory laparotomy and splenectomy, with an unremarkable postoperative course. CONCLUSIONS Acute splenic sequestration crisis can result from hypersplenism, a potentially fatal complication of sickle hemoglobinemia. The continuous cycle of sickled cell entrapment and stasis causes numerous splenic infarctions, forming splenic parenchymal scar tissue which reduces the spleen's size and functionality - the process of auto-splenectomy. Adults rarely experience these crises past adolescence, which are secondary to the scarring and atrophy from premature auto-splenectomy. Our patient's spleen measured 21.1 cm, larger than the average adult's spleen. In our case, adjunctive preoperative splenic artery embolization likely contributed to decreased intraoperative blood loss during splenectomy, mitigating the need for perioperative transfusions.

Efficacy of endoscopic therapy combined with partial splenic embolization versus Hassab's operation for patients with liver cirrhosis with esophageal variceal bleeding and hypersplenism: a multicenter cohort study based on propensity score matching.

BACKGROUND: The prognosis comparison between endoscopic therapy + partial splenic embolization (PSE) and Hassab's operation is unclear in the treatment of esophageal variceal bleeding in patients with liver cirrhosis. This study aimed to compare the outcome of endoscopic therapy + PSE (EP) with a combination of splenectomy + pericardial devascularization procedure, known as Hassab's operation (SH) for esophageal variceal bleeding in patients with liver cirrhosis with hypersplenism. METHODS: We enrolled 328 patients, including 125 and 203 patients who underwent EP and SH, respectively. Each group consisted of 110 patients after propensity score matching (PSM). Subsequently, we recorded and analyzed bleeding episodes and mortality in 6 months and 1, 2, and 5 years after therapies. RESULTS: The median follow-up time in the EP and SH groups was 53 and 64 months, respectively. Bleeding incidence 6 months after therapies in the EP group was lower than that in the SH group (1.8% vs. 10.0%, P = 0.010). Additionally, complications in the perioperative period were not significantly different (0% vs. 3.6%, P = 0.008). However, the bleeding rate between the two groups was not significantly different at 1, 2, and 5 years after therapies (7.3% vs. 12.7%, P = 0.157; 10.9% vs. 16.4%, P = 0.205; 30.6% vs. 31.8%, P = 0.801), as well as mortality rate (4.5% vs 7.3%, P = 0.571). CONCLUSION: Compared with SH therapy, the bleeding rate 6 months after EP therapy was lower, but the long-term bleeding rate was similar.

The Decline of Open, Laparoscopic, and Robotic Splenectomies: A Single Center Experience.

Background: Splenectomy has been performed for various indications from haematological diseases to benign cysts and tumours, and for splenic traumatic injuries. However, there has been a steady decline in splenectomies in the last 20 years. The aim of this study is to establish the reasons behind this decline in splenectomy and to analyse them based on indication, type of splenectomy, and manner of approach (open, laparoscopic or robotic). Material and Methods: This is a retrospective study of a single centre experience of all the splenectomies, both total and partial, performed in the Department of General Surgery of Fundeni Clinical Institute (Bucharest) between 2002 and 2023. Only surgeries for primary splenic diseases were selected, splenic resections as part of other major operations were not included. Results: Between 2002 and 2023, 876 splenectomies were performed in the Department of General Surgery of Fundeni Clinical Institute (Bucharest). Most splenectomies (n=245) were performed for immune thrombocytopenic purpura (ITP), followed by benign tumours and cysts (n=136), lymphoma (n=119), hypersplenism due to cirrhosis (n=107) and microspherocytosis (n=95). Other indications included myelodysplastic syndrome (n=39), trauma (n=35), thalassemia (n=22), leukaemia (n=18) and also there were 60 splenectomies that were performed for hypersplenism of unknown cause. There were 795 total splenectomies (TS) and 81 partial splenectomies (PS). There was a decline in the number of splenectomies both TS and PS for all these indications, most notably in the case of ITP, microspherocytosis and hypersplenism due to cirrhosis with no splenectomies performed for these indications since 2020. Conclusion: With the development of new lines of treatment, advances in interventional radiology and in surgery with the spleen parenchyma sparing options, the need for total splenectomy has been greatly reduced which is reflected in the decline in the number of splenectomies performed in the last 20 years in our clinic.

Partial Splenic Embolization in Paediatric Sickle Cell Disease Patients with Hypersplenism.

PURPOSE: To assess the safety and efficacy of partial splenic embolization (PSE) to reduce the need of transfusions and improve hematologic parameters in patients with hypersplenism and sickle cell disease (SCD). MATERIAL AND METHODS: This prospective study includes 35 homozygous hemoglobin S patients with SCD and hypersplenism who underwent PSE from 2015 until 2021 in Kinshasa. Patients were evaluated, before and after PSE (1, 3 and 6 months), using clinical, laboratory and ultrasonographic methods. PSE was performed with the administration of gelatin sponge particles embolizing 60-70% of the splenic parenchyma. RESULTS: The mean age was 10 (± 4) years and (21/35, 60%) were male. After PSE Leucocytes decreased at 3 months (16 692.94 vs 13 582.86, p = 0.02) and at six months Erythrocytes increased 2 004 000 vs. 2 804 142 (p < 0.001), Platelets increased (168 147 vs. 308 445, p < 0.001) and Hemoglobin increased (5.05 g/dL vs. 6.31 g/dL, p < 0.001) There was a significant dicrease in the need of transfusions from 6 (2-20) before PSE to 0.06 (0-1) after PSE (p < 0.001). The most frequent complication was splenic rupture (4/35, 11.4%), seen only and in all patients with hypoechogenic nodules at baseline. CONCLUSION: PSE is a safe procedure in patients with SCD and hypersplenism, that do not have hypoechogenic nodules in the spleen. PSE improves the hematological parameters and reduces the frequency of blood transfusions.

Falsely low glycosylated haemoglobin levels probably secondary to hypersplenism in a patient with diabetes mellitus.

A man in his 70s presented with a history of low glycated haemoglobin (HbA1c) values despite a diagnosis of type 2 diabetes. His blood glucose readings ranged between 8 and 15 mmol/L, but his HbA1c values were below 27 mmol/mol. Initial investigations demonstrated evidence of reduced red blood cell lifespan as a cause of misleadingly low HbA1c values. Further investigation revealed chronic liver disease and splenomegaly, with hypersplenism being the probable cause of increased red blood cell turnover. HbA1c estimation was no longer reliable, so ongoing diabetic care was guided by home capillary blood glucose monitoring. Healthcare providers and clinical laboratorians need to be aware of the possible clinical implications of very low HbA1c values in patients with type 2 diabetes.

The role and evolution of partial splenic embolization over three decades: A multicentric retrospective single cohort study of 90 patients from French nationwide experience.

BACKGROUND: Partial splenic embolization (PSE) has been proposed to treat the consequences of hypersplenism in the context of portal hypertension, especially thrombocytopenia. However, a high morbidity/mortality rate has made this technique unpopular. We conducted a multicenter retrospective nationwide French study to reevaluate efficacy and tolerance. METHODS: All consecutive patients who underwent PSE for hypersplenism and portal hypertension in 7 tertiary liver centers between 1998 and 2023 were included. RESULTS: The study population consisted of 91 procedures in 90 patients, with a median age of 55.5 years [range 18-83]. The main cause of portal hypertension was cirrhosis (84.6 %). The main indications for PSE were (1) an indication of medical treatment or radiological/surgical procedure in the context a severe thrombocytopenia (59.3 %), (2) a chronic hemorrhagic disorder associated with a severe thrombocytopenia (18.7 %), and (3) a chronic pain associated with a major splenomegaly (9.9 %). PSE was associated with a transjugular intrahepatic portosystemic shunt in 20 cases. Median follow-up after PSE was 41.9 months [0.5-270.5]. Platelet count increased from a median of 48.0 G/L [IQR 37.0; 60.0] to 100.0 G/L [75.0; 148]. Forty-eight patients (52.7 %) had complications after PSE; 25 cases were considered severe (including 7 deaths). A Child-Pugh B-C score (p < 0.02) was significantly associated with all complications, a history of portal vein thrombosis (p < 0.01), and the absence of prophylactic antibiotherapy (p < 0.05) with severe complications. CONCLUSION: Our results strongly confirm that PSE is very effective, for a long time, although a quarter of the patients experienced severe complications. Improved patient selection (exclusion of patients with portal vein thrombosis and decompensated cirrhosis) and systematic prophylactic antibiotherapy could reduce morbidity and early mortality in the future.

Multianticoagulant Pseudothrombocytopenia in a Patient with Primary Carcinoma of the Liver with Hypersplenism.

BACKGROUND: Pseudothrombocytopenia (PTCP) is a relatively rare phenomenon in vitro, the mechanism is not completely clear, and there is no unified solution for it. How to identify and solve PTCP accurately is a challenge for laboratory personnel. METHODS: According to the patient's clinical manifestations, thrombocytopenia caused by hypersplenism was excluded. PTCP was confirmed by platelet volume histograms, scattergrams and platelet clumps on the blood smears. Commonly used alternative anticoagulants such as sodium citrate or heparin were used for platelet counting. The corrective effect of the platelet count was not good, so non-anticoagulant blood was collected and tested immediately, and blood smears were used to count platelets manually. RESULTS: The PTCP of the patient could not be solved using sodium citrate and heparin anticoagulation. By collecting non-anticoagulant blood and testing immediately, the platelet count returned to normal (180 x 109/L), which is consistent with the results of manual counting on the patient's blood smears (175 x 109/L). CONCLUSIONS: When PTCP is confirmed, commonly used alternative anticoagulants can be used. If these do not work, non-anticoagulant blood can be collected and tested immediately, and blood smears can be used to count platelets manually.

The spleen is the graveyard of CD4+ cells in patients with immunological failure of visceral leishmaniasis and AIDS.

BACKGROUND: Visceral leishmaniasis (VL), or kala-azar, is a common comorbidity in patients with AIDS in endemic areas. Many patients continue to experiences relapses of VL despite virological control, but with immunological failure. These patients remain chronically symptomatic with hypersplenism, for example with anemia, leukopenia, and thrombocytopenia, and are at risk of severe co-infection due to low CD4+ count. Therefore, in this study, splenectomized patients with VL and HIV infection were investigated to understand why the CD4+ count fails to recover in these patients, evaluating the importance of spleen mass for hypersplenism and immunological failure. METHODS: From a retrospective open cohort of 13 patients who had previously undergone splenectomy as salvage therapy for relapsing VL, 11 patients with HIV infection were investigated. This study compared the patients' complete blood cell count (CBC) and CD4+ and CD8+ cell counts before and after splenectomy with respect to spleen weight. RESULTS: CBC was substantially improved after splenectomy, indicating hypersplenism. However, to the best of our knowledge, this is the first study to show that spleen mass is strongly and negatively correlated with CD4+ cell count (ρ = -0.71, P = 0.015). CONCLUSIONS: This finding was unexpected, as the spleen is the most extensive lymphoid tissue and T-lymphocyte source. After reviewing the literature and reasoning, we hypothesized that the immunological failure was secondary to CD4+ loss initially by apoptosis in the spleen induced by productive HIV infection and, subsequently, by pyroptosis sustained by parasitic infection in spleen macrophages.

Temporal trends of splenectomy in pediatric hospitalizations with hereditary spherocytosis from 2000 to 2019: A national survey.

BACKGROUND: Total and partial splenectomy are used in pediatric patients with hereditary spherocytosis to resolve anemia and hemolytic complications. PROCEDURE: Data from the Healthcare Cost and Utilization Project's Kid's Inpatient Database was used to profile and describe temporal trends in pediatric (≤18 years) hospital admissions in the United States from 2000 to 2019 data release years. Survey sampling methods were used to produce national estimates. RESULTS: From 2000 to 2019, the use of splenectomy declined overall, from 427 to 206 weighted procedures (difference = 222, 95% confidence interval [CI]: 124-320; p < .0001); the risk of undergoing splenectomy during admission also declined from 56.7% to 38.7% (risk difference = 17.9 percentage points [p.p.], 95% CI: 9.7-26.1; p < .0001). Total splenectomy was mostly used. Age at time of splenectomy increased 10.2 years (difference = 1.6 years, 95% CI: 0.6-2.7; p = .0018). The risk of splenectomy increased with age until 10 years, then leveled off until 18 years. The proportion of children aged ≤5 years undergoing splenectomy decreased from 27.7% to 11.2% in 2019 (risk difference: 16.5 p.p., 95% CI: 7.3-25.7; p = .0004). The strongest clinical predictors of splenectomy, adjusting for patient- and hospital-level characteristics, were a co-diagnosis of symptomatic cholelithiasis (adjusted odds ratio [aOR] = 3.18, 95% CI: 1.92-5.28; p < .0001) and splenomegaly or hypersplenism (aOR = 2.52, 95% CI: 1.74-3.65; p < .0001). Risk of splenectomy with splenomegaly or hypersplenism increased over time. CONCLUSION: Splenectomy was delayed until age greater than 10 years. Older age, co-diagnosis with splenomegaly or hypersplenism, or symptomatic cholelithiasis were strongest clinical predictors of splenectomy. Conservative management of hereditary spherocytosis appears to be more common.

Study of platelet kinetics in immune thrombocytopenia to predict splenectomy response.

Despite the efficacy of splenectomy for chronic immune thrombocytopenia (ITP), its considerable failure rate and its possible related complications prove the need for further research into potential predictors of response. The platelet sequestration site determined by 111 In-labelled autologous platelet scintigraphy has been proposed to predict splenectomy outcome, but without standardisation in clinical practice. Here, we conducted a single-centre study by analysing a cohort of splenectomised patients with ITP in whom 111 In-scintigraphy was performed at La Paz University Hospital in Madrid to evaluate the predictive value of the platelet kinetic studies. We also studied other factors that could impact the splenectomy outcome, such as patient and platelet characteristics. A total of 51 patients were splenectomised, and 82.3% responded. The splenic sequestration pattern predicted a higher rate of complete response up to 12 months after splenectomy (p = 0.005), with 90% sensitivity and 77% specificity. Neither age, comorbidities, therapy lines nor previous response to them showed any association with response. Results from the platelet characteristics analysis revealed a significant loss of sialic acid in platelets from the non-responding patients compared with those who maintained a response (p = 0.0017). Our findings highlight the value of splenic sequestration as an independent predictor of splenectomy response.

The decreased risk of hepatocellular carcinoma in hepatitis B virus-related cirrhotic portal hypertension patients after laparoscopic splenectomy and azygoportal disconnection.

BACKGROUND: Posthepatitic cirrhosis is one of the leading risk factors for hepatocellular carcinoma (HCC) worldwide, among which hepatitis B cirrhosis is the dominant one. This study explored whether laparoscopic splenectomy and azygoportal disconnection (LSD) can reduce the risk of HCC among patients with hepatitis B virus (HBV)-related cirrhotic portal hypertension (CPH). METHODS: A total of 383 patients with HBV-related CPH diagnosed as gastroesophageal variceal bleeding and secondary hypersplenism were identified in our hepatobiliary pancreatic center between April 2012 and April 2022, and conducted an 11-year retrospective follow-up. We used inverse probability of treatment weighting (IPTW) to correct for potential confounders, weighted Kaplan-Meier curves, and logistic regression to estimate survival and risk differences. RESULTS: Patients were divided into two groups based on treatment method: LSD (n = 230) and endoscopic therapy (ET; n = 153) groups. Whether it was processed through IPTW or not, LSD group showed a higher survival benefit than ET group according to Kaplan-Meier analysis (P < 0.001). The incidence density of HCC was higher in the ET group compared to LSD group at the end of follow-up [32.1/1000 vs 8.0/1000 person-years; Rate ratio: 3.998, 95% confidence intervals (CI) 1.928-8.293]. Additionally, in logistic regression analyses weighted by IPTW, LSD was an independent protective predictor of HCC incidence compared to ET (odds ratio 0.516, 95% CI 0.343-0.776; P = 0.002). CONCLUSION: Considering the ability of LSD to improve postoperative survival and prevent HCC in HBV-related CPH patients with gastroesophageal variceal bleeding and secondary hypersplenism, it is worth promoting in the context of the shortage of liver donors.

Case report: Successful pregnancy complicated with non-cirrhotic portal hypertension in a lady who suffered from postpartum hemorrhage previously.

RATIONALE: Non-cirrhotic portal hypertension (NCPH) is characterized by the absence of cirrhotic modification of the liver and the patency of the portal and hepatic veins. When compared to the general population, NCPH is associated with an increased risk of maternal and perinatal morbidity and mortality during pregnancy. NCPH was present in the majority (74.1%) of pregnant women with portal hypertension. One (25%) out of every 4 pregnancies was complicated by variceal hemorrhage while pregnant. So far, there is still no consensus in the world about the treatment of this rare condition. PATIENT CONCERNS: We have specifically illustrated a rare instance where the patient was diagnosed with NCPH and hypersplenism at the age of 8 and experienced a 3 L massive hemorrhage during labor induction as a result of her first pregnancy loss due to hypertension. DIAGNOSES AND INTERVENTIONS: The diagnosis of threatened preterm labor with cervical dilatation, gestational diabetes mellitus, massive splenomegaly with hypersplenism, portal vein hypertension, and parenchymal damage of kidney with impaired renal function led to the cesarean delivery of the second pregnancy at 29+3 weeks gestation without splenectomy after been evaluated by multispecialty team. OUTCOMES: She and her child were both in generally good condition 3 months after the operation. LESSONS: Preconception counseling, ongoing follow-up, and monitoring are crucial in pregnant women with NCPH. A multidisciplinary team approach, with timely intervention and intensive monitoring, can help achieve optimal maternal-perinatal outcomes in pregnancies complicated with portal hypertension. Our case provided a successful treatment, but more guidelines for the management of NCPH are needed.

Risk factors for portal vein system thrombosis after partial splenic embolisation in cirrhotic patients with hypersplenism.

AIM: To determine risk factors for portal venous system thrombosis (PVST) after partial splenic artery embolisation (PSAE) in cirrhotic patients with hypersplenism. MATERIALS AND METHODS: Between March 2014 and February 2022, 428 cirrhotic patients with hypersplenism underwent partial splenic artery embolisation and from these patients 208 were enrolled and 220 were excluded. Medical records of enrolled patients were collected. Computed tomography (CT) images were reviewed by two blinded, independent radiologists. Statistical analyses were performed by using SPSS. RESULTS: Progressive PVST was observed in 18.75% (39/208) of cirrhotic patients after PSAE. No significant differences in peripheral blood counts, liver function biomarkers, and renal function were observed between the patients with progressive PVST and the patients without progressive PVST. The imaging data showed significant differences in PVST, the diameters of the portal, splenic, and superior mesenteric veins between the progressive PVST group and non-progressive PVST group. Univariate and multivariate analysis demonstrated portal vein thrombosis, spleen infarction percentage, and the diameter of the splenic vein were independent risk factors for progressive PVST. Seventeen of 173 (9.83%) patients showed new PVST; the growth of PVST was observed in 62.86% (22/35) of the patients with pre-existing PVST. Spleen infarction percentage and the diameter of the splenic vein were independent risk factors for new PVST after PSAE. CONCLUSION: The present study demonstrated portal vein thrombosis, spleen infarction percentage, and the diameter of the splenic vein were independent risk factors for PVST after PSAE in cirrhotic patients with hypersplenism.

Evaluation of splenic infarction ratio and platelet increase ratio after partial splenic artery embolization.

OBJECTIVE: The spleen is part of the lymphatic system and is one of the least understood organs of the human body. It is involved in the production of blood cells and helps filter the blood, remove old blood cells, and fight infection. Partial splenic artery embolization (PSE) is widely used to treat pancytopenia and portal hypertension. The efficacy of PSE for improving thrombocytopenia has been well demonstrated. In this study, we evaluated the splenic infarction ratio and platelet increase ratio after PSE. METHODS: Forty-five consecutive patients underwent PSE from January 2014 to August 2022. We retrospectively evaluated the splenic infarction volume and ratio after PSE and analyzed the relationship between the splenic infarction ratio and platelet increase ratio after PSE. RESULTS: The platelet increase ratio was correlated with the splenic infarction ratio after PSE. The cutoff value for the splenic infarction ratio with a two-fold platelet increase was 63.0%. CONCLUSION: We suggest performance of PSE in patients with a splenic infarction ratio of 63% to double the expected platelet count.

Changes in Hepatic Function After Splenectomy for Hepatolenticular Degeneration, Cirrhosis, and Hypersplenism.

BACKGROUND: Patients with hepatic reticulum degeneration (HLD) may eventually develop complications of cirrhosis with splenomegaly and hypersplenism, requiring splenectomy to alleviate hypersplenism and complete lifelong copper therapy. The purpose of this study is to investigate the effect of splenectomy on liver function in patients with hypersplenism. METHODS: A retrospective systematic analysis was conducted on the liver function indicators of 220 HLD patients who underwent splenectomy from January 2015 to January 2018 before surgery and on days 1, 3, 5, 7, and 14 after surgery. Among them, 30 patients were followed up for 6 months. RESULTS: The Child score increased on the 1st day after surgery and gradually decreased after the 1st day. The level of alanine aminotransferase (ALT), aspartate aminotransferase (AST), and total bilirubin (TB) increased 5 days after surgery (P < .01) and decreased on the 14th day after surgery (P < .01); the level of albumin (ALB) decreased on the 1st, 3rd, and 5th day after surgery (P < .01) and increased on the 14th day (P < .01). The follow-up results of the patient for 6 months showed that the levels of ALT and AST decreased, while the levels of ALB increased 6 months after surgery. CONCLUSION: Splenectomy is proved to be beneficial for the improvement of liver function in HLD patients combined with hypersplenism, which realize a lifelong anti-copper treatment.

The Role of the Spleen in Portal Hypertension.

As liver disease progresses, intrahepatic vascular resistance increases (backward flow theory of portal hypertension) and collateral veins develop. Adequate portal hypertension is required to maintain portal flow into the liver through an increase in blood flow into the portal venous system (forward flow theory of portal hypertension). The splenic artery resistance index is significantly and selectively elevated in cirrhotic patients. In portal hypertension, a local hyperdynamic state occurs around the spleen. Splenomegaly is associated with a poor prognosis in cirrhosis and is caused by spleen congestion and by enlargement and hyperactivation of splenic lymphoid tissue. Hypersplenism can lead to thrombocytopenia caused by increased sequestering and breakdown of platelets in the spleen. The close relationship between the spleen and liver is reflected in the concept of the hepatosplenic axis. The spleen is a regulatory organ that maintains portal flow into the liver and is the key organ in the forward flow theory of portal hypertension. This review summarizes the literature on the role of the spleen in portal hypertension.