Characteristics of large granular lymphocyte leukemia associated with variable common immunodeficiency disorders: A study of 12 cases.

OBJECTIVES: Common Variable Immunodeficiency Disorders (CVID) and Large Granular Lymphocytes leukemia (LGLL) exhibit diverse clinical manifestations including infections, dysimmunity, and lymphoproliferation. Recent decades have seen the discovery of new genes in the lymphopoiesis pathway, such as JAK-STAT. This case series supplemented by a literature review aims to describe clinical and biological characteristics of patients with both CIVD and LGLL. METHODOLOGY: Patients were included through a call for comments to French and Belgian centers and through a literature review via PubMed. Clinical characteristics were compared to two large French cohort involving CVID and LGLL patients. RESULTS: Twelve patients were included. In all cases, CVID precedes LLGL (median diagnosis delay for LLGL was 7 years). Most cases presented with splenomegaly and autoimmune cytopenia. Ten out of 12 patients underwent splenectomy during follow up. CONCLUSIONS: Patients with LGLL and CVID differ from patients without immune deficiency in term of clinical presentation and prognosis. We suggest CVID may act as a trigger of LGL lymphocytosis, due to endogenous and exogenous antigenic pressure leading to the selection of a dominant LGL clone and stimulation of the JAK-STAT pathway. The role of splenomegaly and splenectomy in LGLL onset warrant further investigation in future studies.

Abrupt elevation of tumor marker levels in a huge splenic epidermoid cyst, a case report.

Epidermoid cyst of the spleen is a rare disease, and relatively few cases were reported by literatures. Most published case reports provided inadequate information on the impact of splenic epidermoid cyst on tumor markers. A 32-year-old woman with a giant splenic epidermoid cyst was reported, for whom the serum concentration of a collection of tumor markers (CA19-9, CEA, CA125, CA242, and CA50) increased abruptly accompanied by left upper abdominal pain for 5 days. After comprehensive preoperative examination and multidisciplinary team discussion, we ruled out any concurrent malignancy and a laparoscopic total splenectomy was performed, during which the splenic cyst spontaneously ruptured unexpectedly. After surgery, the elevated serum tumor marker levels decreased sharply until reaching normal range 3 months later. Learning from the case, we conclude that interval monitoring of serum tumor markers is of critical value for patients with splenic epidermoid cyst. Abrupt elevation of tumor marker levels and abdominal pain may serve as signs of cyst rupture, which is strongly indicative of surgical intervention as soon as possible. Total removal of the splenic cyst is strongly suggested considering the recurrence and malignant potential of the splenic epidermoid cyst.

A Case Series of Isolated Primary Hydatid Cyst of the Spleen.

Hydatid disease is endemic mainly in Asia and other sheep-raising areas. In India, hydatid disease has been a very common disease because of its close association with livestock rearing. Hydatid disease is a parasitic infestation caused by Echinococcus granulosus. The usual location of infestation is in the liver sinusoids, lungs, and spleen. Hydatid disease in humans is rare, and a hydatid cyst of the spleen without involving the liver is very rare. The rarity of splenic hydatid disease may pose a diagnostic challenge for clinicians, especially in non-endemic areas. The diagnosis of hydatid disease is based on the epidemiological background of patients, clinical grounds, or noninvasive screening procedures. With this background, we aimed to study the presenting symptomatology and various clinical manifestations of isolated spleen hydatid disease and analyze the morbidity and mortality of hydatid disease. Different surgical modalities and their complications were studied. Three patients operated on for splenic hydatid at our institute were studied retrospectively.

Postpartum Spontaneous Rupture of a Primary Splenic Hydatid Cyst: A Case Report.

Hydatid disease is endemic in some regions of the world. Even in endemic regions, splenic hydatid cysts are rare, especially in pregnancy. The most serious presentation is intra peritoneal rupture, which is a surgical emergency. Exploration with splenectomy is the favoured management. We present a case of splenic hydatid cyst, detected late in pregnancy, with intraperitoneal rupture in the postpartum period. Cystic lesions located anywhere in the body in endemic regions could be hydatid cysts. Prompt treatment should be planned immediately on detection in order to prevent potentially serious complications like rupture.

Splenomegaly and Response to Splenectomy in Immune Thrombocytopenia.

Background: Immune thrombocytopenia (ITP) is an acquired autoimmune disorder affecting patients of all ages and backgrounds. While current standards favor medical therapy in the frontline setting, splenectomy remains an integral part of treatment in refractory cases. Ideal parameters for patient selection for surgery remain elusive. Methods: Data for 40 adult patients undergoing splenectomy for ITP at a large urban center between 1 January 2010 and 1 July 2021 were collected and analyzed. Results: Most patients underwent uneventful laparoscopic splenectomy (95%). Complete or partial response at the time of last follow-up occurred in most patients (92.5%), with 60.0% requiring no additional medical therapy following surgery. Thrombosis was the predominant adverse event and the leading cause of death for two patients. Age and presence of splenomegaly appear to be associated with response to splenectomy. Conclusions: Splenectomy remains an effective therapy for selected patients with ITP. Predictors of positive response to splenectomy, such as younger age and the presence of splenomegaly, may help inform clinicians during patient selection for therapy. With strict attention paid to postoperative thromboprophylaxis, the diminishing use of splenectomy may not be warranted.

Laparoscopic Partial Splenectomy May Be Valuable for the Diagnosis of Malignant Lymphoma: A Case Report.

Background/Aim: Diagnosing primary splenic malignant lymphoma (PSML) is challenging due to the non-specific nature of splenomegaly, necessitating splenic biopsy for confirmation. However, performing partial splenic resection for diagnostic purposes is an elective procedure due to the risk of major hemorrhage. Despite the longstanding practice of splenectomy over the past few decades, it remains invasive and may result in severe early or late complications. Hence, we present laparoscopic partial splenectomy (LPS) in a patient suspicious of PSML for diagnostic purposes in this study. Case Report: An 81-year-old woman presented to our hospital with a one-month history of fever and dry cough. Atypical cells had been detected in her peripheral blood nine months ago. However, at that time, a bone marrow examination did not reveal any atypical cells. The laboratory tests revealed a soluble interleukin receptor-2 levels of 4,667 U/dl and atypical cells were also found in peripheral blood. Abdominal computed tomography showed splenomegaly without any other relevant findings. These findings are suspicious of PSML and LPS without vessel ligation was performed and a small fraction of the spleen from the inferior pole measuring 1.8×1.0 cm was resected. The operation lasted for 63 min with minimal estimated blood loss. Histopathological findings were compatible with the diagnosis of diffuse B-cell lymphoma. The postoperative clinical course was uneventful, and splenomegaly demonstrated improvement six months after the operation. Conclusion: LPS without vessel ligation for biopsy may be valuable for the diagnosis of malignant lymphoma, particularly when there are no swollen lymph nodes, as it offers a less invasive approach.

Recurrent Salmonella bacteraemia and right internal iliac artery endarteritis in a splenectomised patient.

A man in his mid-70s with a complex medical history, including splenectomy, presented with fever and rigours. Workup revealed Salmonella enterica serotype typhimurium bacteraemia and right internal iliac artery endarteritis. Two weeks following a 6-week course of antibiotics, he had a recurrence of Salmonella bacteraemia requiring an extended course of treatment.

Giant splenic artery pseudoaneurysm with arteriovenous fistula: diagnostic challenges and interdisciplinary treatment considerations.

We present a compelling case of an elderly male with a complex medical history who presented with sepsis secondary to a urinary tract infection. During admission, changes in his abdominal exam prompted imaging studies, which revealed a grade IV splenic laceration with a giant splenic artery pseudoaneurysm containing a suspected arteriovenous fistula component. Multidisciplinary discussion was had regarding patient management which resulted in the decision to perform an emergent splenectomy. Learning points from this case underscore the crucial role of interdisciplinary collaboration in the treatment of this pathology. Additionally, we discuss the decision-making process to support surgical intervention in the absence of clear guidelines in this exceedingly rare condition.

Spleen autotransplantation on liver versus greater omentum for simultaneous injuries of liver and spleen: an experimental study.

Purpose: In a simulated situation of simultaneous spleen and liver trauma, we aimed to compare the outcomes of treating both injuries with spleen autotransplantation on the omentum (SAO) alongside hepatorrhaphy versus spleen autotransplantation as a patch on the liver parenchyma. Methods: A total of 24 rats were separated into two groups: the SAO and the spleen autotransplantation on the liver. They underwent a uniform and simultaneous procedure involving full-thickness injuries to the left lobe of the liver and grade 4 spleen injuries. We measured hemoglobin, white blood cell (WBC), complement (C3 and C4), and immunoglobulin G, M, and A (IgG, IgM, IgA) levels before and 4 weeks after the surgery. We utilized Technetium-99m scintigraphy to evaluate the posttransplant splenic graft functions 4 weeks after the surgery. Results: The two groups had no significant difference in the hematologic and immunologic factors before surgery. However, both procedures significantly reduced hemoglobin, C3, IgG, and IgA levels (all P<0.05). WBC counts significantly increased in the SAO group, whereas the IgM level decreased after the intervention (P<0.05). WBC was increased in the SAO group, while IgM and IgA were decreased in the SAO group. The Technetium uptake was similar between the two groups (P=0.3). Conclusions: In simultaneous spleen and liver injuries, the autotransplantation of splenic into the liver parenchyma appears to be a promising surgical approach for preserving spleen function and hepatorrhaphy at the same time instead of doing them separately.

Clinical comparative study of laparoscopic partial splenectomy and open partial splenectomy.

Introduction: The aim of the article was too investigate and compare the feasibility, safety, and early postoperative recovery associated with laparoscopic partial splenectomy (LPS) and open partial splenectomy (OPS) in patients with benign splenic tumours and traumatic splenic rupture. Material and methods: A retrospective analysis was conducted on clinical data from 110 patients undergoing splenic resection at our hospital between March 2019 and May 2022. Among them, 35 patients underwent OPS, 25 underwent LPS for traumatic splenic rupture, while 50 patients with benign splenic tumours underwent either OPS (n = 20) or LPS (n = 30). Preoperative, intraoperative, and postoperative data were collected and compared. Statistical analysis was conducted using SPSS software. Results: There was no significant difference in the general data between the 2 groups of patients with benign splenic tumours and those with splenic trauma. Among patients with traumatic splenic rupture, the OPS group had a shorter operation time (p < 0.05). Regardless of whether they had traumatic splenic rupture or benign splenic tumours, the LPS group required less postoperative analgesia and had a shorter defecation recovery time (p < 0.05). Additionally, the LPS group displayed lower white blood cell count, white blood cell/lymphocyte ratio (WLR), neutrophil/lymphocyte ratio (NLR), monocyte/lymphocyte ratio (MLR), C-reactive protein (CRP), calcitonin (PCT), and interleukin-6 (IL-6) than the OPS group on the first and third days post-surgery (p < 0.05). Conclusions: In comparison to OPS, LPS presents significant advantages, including minimal surgical trauma, a reduced early postoperative inflammatory response, milder wound pain, and a faster recovery of gastrointestinal function.

Clinical Outcomes of Splenic Artery Embolization Versus Splenectomy in the Management of Hemodynamically Stable High-Grade Blunt Splenic Injuries: A National Analysis.

INTRODUCTION: This study aims to compare the outcomes of splenic artery embolization (SAE) versus splenectomy in adult trauma patients with high-grade blunt splenic injuries. METHODS: This retrospective analysis of the American College of Surgeons Trauma Quality Improvement Program database (2017-2021) compared SAE versus splenectomy in adults with blunt high-grade splenic injuries (grade ≥ IV). Patients were stratified first by hemodynamic status then splenic injury grade. Outcomes included in-hospital mortality, intensive care unit length of stay (ICU-LOS), and transfusion requirements at four and 24 h from arrival. RESULTS: Three thousand one hundred nine hemodynamically stable patients were analyzed, with 2975 (95.7%) undergoing splenectomy and 134 (4.3%) with SAE. One thousand eight hundred sixty five patients had grade IV splenic injuries, and 1244 had grade V. Patients managed with SAE had 72% lower odds of in-hospital mortality (odds ratio [OR] 0.28; P = 0.002), significantly shorter ICU-LOS (7 versus 9 d, 95%, P = 0.028), and received a mean of 1606 mL less packed red blood cells at four h compared to those undergoing splenectomy. Patients with grade IV or V injuries both had significantly lower odds of mortality (IV: OR 0.153, P < 0.001; V: OR 0.365, P = 0.041) and were given less packed red blood cells within four h when treated with SAE (2056 mL versus 405 mL, P < 0.001). CONCLUSIONS: SAE may be a safer and more effective management approach for hemodynamically stable adult trauma patients with high-grade blunt splenic injuries, as demonstrated by its association with significantly lower rates of in-hospital mortality, shorter ICU-LOS, and lower transfusion requirements compared to splenectomy.

Splenic Hydatid Cyst Presenting As Non-cirrhotic Portal Hypertension: A Rare Case Report.

Hydatid cystic disease, also called cystic echinococcosis, arises from Echinococcus, a tapeworm infestation. It results in developing cysts primarily in the liver, although they can also occur in other organs. While the spleen is an uncommon site for cyst formation, it can still be affected. These infections are more prevalent in rural and underdeveloped regions, particularly among individuals involved in livestock rearing and animal care. The case we came across was of a 32-year-old female from a rural background engaged in animal handling and farming. She presented to our hospital with left hypochondriac pain, decreased appetite, and generalized weakness, but the patient had a history of two episodes of melena, which was self-limiting. Subsequent investigations revealed a diagnosis of splenic hydatid cyst with perisplenic collaterals and cystic compression of the splenic vein, causing symptoms of non-cirrhotic portal hypertension. Here, we present a unique case of splenic hydatid cyst leading to non-cirrhotic portal hypertension. This rare presentation poses diagnostic challenges and emphasizes the importance of considering parasitic infections in differential diagnoses.

A Case of Isolated Splenic Tuberculosis Presenting as a Splenic Abscess Complicated by Rupture Leading to Splenectomy in an Immunocompetent Male.

We report a rare case of splenic tuberculosis (TB) in a male patient with a competent immune system who had no previous record of pulmonary TB. A 56-year-old male patient came to our outpatient department complaining of upper abdominal pain with a few episodes of vomiting for three days. He had alcoholism, smoked for 15 years, and had no past history of diabetes mellitus, hypertension, TB, or HIV. An abdominal ultrasound and CT scan at admission showed pancreatitis with a splenic abscess. After five days of admission, the patient's vitals deteriorated, and he had severe abdominal pain. CT scan suggested a splenic abscess rupture with hemoperitoneum. An emergency exploratory laparotomy was performed, and a splenectomy was done due to the splenic abscess rupture. A cartridge-based nucleic acid amplification test from splenic intracapsular fluid detected a trace Mycobacterium tuberculosis complex. The patient was discharged after starting first-line antitubercular treatment for six months. After three months of follow-up, the patient was doing well with no complaints.

A Giant Non-Traumatic Splenic Pseudocyst Successfully Treated With Cyst Aspiration and Partial Cystectomy: A Case Report and Review of Literature.

Splenic cysts are extremely rare entities that typically result from prior abdominal trauma, infections, and degenerative diseases. They are divided into two categories: true cysts with epithelial lining, and false pseudocysts without epithelial lining, which is more common than true cysts. We describe here a case of a non-traumatic splenic pseudocyst in a healthy 29-year-old male patient, who presented with left upper quadrant abdominal pain. Physical examination revealed scaphoid abdomen and left hypochondrium fullness. The spleen was uniformly enlarged, smooth, and firm, with mild tenderness. Laboratory testing was normal. An abdominal CT scan showed a huge unilocular non-enhancing cyst occupying the upper part of the spleen, measuring around 16 × 18.5 × 20 cm. The patient was managed with cyst aspiration and partial cystectomy. The histopathological examination findings are consistent with splenic pseudocyst. A one-year follow-up period revealed no complications or recurrence. Spleen cysts are rare in clinical practice, posing challenges in diagnosis and treatment. Surgical options include partial or total splenectomy, cyst aspiration, percutaneous drainage, partial cystectomy, and marsupialization. The choice depends on the cyst's size, splenic coverage, and relation to the hilum. Recently, spleen-preserving approaches have been favored to avoid life-threatening sepsis. Non-traumatic splenic pseudocysts present significant diagnostic dilemmas, requiring histopathological examination for definitive diagnosis. Spleen-preserving management is highly recommended to reduce the risk of life-threatening sepsis.

Comparison of splenic embolization and splenectomy for traumatic splenic rupture: a meta-analysis.

INTRODUCTION: This study aims to assess the safety and clinical efficacy of percutaneous splenic embolization (PSE) and splenectomy as approaches to treating cases of traumatic splenic rupture (TSR). MATERIAL AND METHODS: Eligible articles published throughout August 2023 were identified. Endpoints compared between PSE and splenectomy patient groups included operative time, intraoperative hemorrhage, duration of hospitalization, postoperative complication rates, and measures of immune function. RESULTS: Thirteen studies, involving 474 and 520 patients in the PSE and splenectomy groups respectively, were incorporated into this meta-analysis. As compared to the splenectomy group, individuals treated via PSE exhibited a significant reduction in pooled operative time (p < 0.00001) and hospitalization duration (p < 0.00001), with corresponding reductions in rates of intraoperative hemorrhage (p < 0.00001), total complications (p < 0.0001), incisional infection (p < 0.0001), ileus (p = 0.0004), and abdominal infection (p = 0.02). The immune status of these PSE group patients was also improved, as evidenced by significantly higher pooled CD4+ (30 days), CD4+/CD8+ (30 days), and CD3+ (30 days) values (p < 0.0001, 0.0001, and 0.0001, respectively). CONCLUSIONS: Compared to splenectomy, PSE-based TSR treatment can significantly reduce operative time, rate of postoperative complications, and incidence of intraoperative hemorrhage, while improving post-procedural immune functionality.

Importance of CD71+ Erythrocyte Cell Levels in Prognosis in Patients With ß-Thalassemia.

BACKGROUND/OBJECTIVES: CD71+ erythroid cells (CECs) are immature red blood cells (proerythroblasts, erythroblasts, and reticulocytes). CECs play an important role in the development of sepsis and cancer by causing immunosuppression. We examined the CEC levels in the peripheral blood of beta thalassemia (ßThal) patients and investigated the relationship between CECs and the clinical status of the patients, especially splenectomy. METHODS: Sixty-eight patients with ßThal (46 splenectomized and 22 nonsplenectomized) and 15 healthy controls were included in this study. The hemogram parameters, ferritin, and CECs (flow cytometry method) were measured. RESULTS: It was observed that the CEC level in the patient group was significantly higher than the control group (p < 0.05). CEC levels were found to be significantly higher in patients with splenectomy than in patients with nonsplenectomy (p < 0.05). CEC levels were higher in patients with nontransfusion-dependent ßT (NTD-ßThal) than in patients with transfusion-dependent ßT (TD-ßThal) (p < 0.05). CEC levels were found to be significantly higher in patients with splenectomy than in patients with nonsplenectomy in both TD-ßThal and NTD-ßThal groups (p < 0.05). There was a moderate-negative correlation was detected between CECs and Hb levels (r = -0.467; p < 0.05). CONCLUSIONS: High CEC levels in ßThal patients develop as a result of ineffective erythropoiesis. We think that keeping CEC levels under control is important for prognosis, especially in patients with splenectomy.

Splenic abscess and infective endocarditis.

OBJECTIVE: To determine the background, bacteriological, clinical and radiological findings, associated lesions, treatment and outcome of splenic abscesses (SAs) in infective endocarditis (IE). METHODS: Retrospective study (2005-2021) of 474 patients with definite IE. The diagnosis of SA was made in 36 (7.6%) patients (31, 86.1%, males, mean age = 51.3) on abdominal CT. RESULTS: The main implicated organisms were Streptococcus spp (36.1%), Enterococcus faecalis (27.7%), Staphyloccus spp (19.4%). Rare agents were present in 10 patients (27.8%). Pre-existing conditions included a prosthetic valve (19.4%), previous IE (13.9%), intravenous drug use (8.4%), diabetes (25%) alcohol abuse (13.9%), liver disease (5.5%). Vegetations ≥ 15 mm were present in 36.1%. Common presentations were abdominal pain (19.4%) and left-sided pleural effusion (16.5%). SA were more often small (50%; 7 multiple) than large (36.1%; 1 multiple) or microabscesses (13.9%, 3 multiple). Associated complications were extrasplenic abscesses (brain, 11.1%; lung, 5.5%; liver, 2.8%), infectious aneurysms (16.7%: 3 intracranial, 1 splenic, 1 hepatic, 1 popliteal), emboli (brain, 52.8%; spleen, 44.4%, 5 evolving to SA; kidney, 22.2%; aorta, 2.8%), osteoarticular infections (25%). Twenty-eight (77.8%) patients only received antimicrobials, 7 (19.4%) underwent splenectomy, after cardiac surgery in 5. One had percutaneous drainage. The outcome was uneventful (follow-up 3 months-14 years; mean: 17.2 months). CONCLUSION: In SA-IE patients, the prevalence of vegetation size, Enterococcus faecalis, rare germs, diabetes, osteo-arthritic involvement and cancer was higher than in non-SA patients. Some SAs developed from splenic infarcts. IE-patients with evidence of splenic emboli should be evaluated for a possible abcedation. Cardiac surgery before splenectomy was safe.

Splenectomy as a part of cytoreductive surgery in ovarian cancer: systematic review and meta-analysis.

OBJECTIVE: The role of splenectomy on cytoreductive surgery in patients with ovarian cancer remains controversial. We conducted this meta-analysis to evaluate the safety and impact of survival outcome of splenectomy in patients with ovarian cancer. METHODS: In this meta-analysis we analyzed studies published in PubMed, Embase, Cochrane Central Register of Controlled Trials (CENTRAL), International Clinical Trials Registry Platform (ICTRP), and Clinical Trials. gov that appeared in our search from inception to November 10, 2023. RESULT: This meta-analysis included 10 studies, totaling 6297 patients, comprising one prospective and nine retrospective analyses. The results indicated no significant disparity in overall survival and mortality (OR 1.14, 95% CI 0.69 to 1.87, p=0.62) between the splenectomy cohort and the no splenectomy (required) cohort. Furthermore, relative to the no splenectomy (required) cohort, the splenectomy group showed a heightened incidence of overall post-operative complications (odds ratio (OR) 1.66, 95% CI 1.65 to 2.61, p=0.03), an extended duration of hospitalization (mean difference (MD) 2.88 days, 95% CI 2.09 to 3.67), an increased interval from surgery to the initiation of adjuvant chemotherapy (MD 4.44 days, 95% CI 2.41 to 6.07, p<0.0001), and a greater probability of undergoing reoperation (OR 4.7, 95% CI 1.91 to 11.55, p=0.0007). However, concerning the occurrence of specific post-operative complications such as anastomotic leakage (OR 0.97, 95% CI 0.33 to 2.84, p=0.95), pancreatic fistula (OR 3.25, 95% CI 0.63 to 16.7, p=0.16), abdominal abscess (OR 1.75, 95% CI 0.25 to 12.33, p=0.57), sepsis (OR 1.46, 95% CI 0.77 to 2.77, p=0.25), and thrombotic events (OR 1.82, 95% CI 0.93 to 3.57, p=0.08), no significant differences were observed between the two cohorts. CONCLUSION: Splenectomy does not impact the overall survival and mortality of patients with ovarian cancer. Thus, it can be considered an acceptably safe procedure to obtain optimal cytoreduction. However, caution should be taken when selecting patients for splenectomy because it is associated with an increased incidence of overall post-operative complications, prolonged hospital stays, delayed initiation of adjuvant chemotherapy, and an increased probability of requiring subsequent surgical interventions.