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Role of splenectomy in chronic lymphocytic leukaemia with massive splenomegaly and cytopenia.

Fourteen cases of chronic lymphocytic leukaemia (CLL) and massive splenomegaly associated with anaemia and/or thrombocytopenia (stage C) with or without mechanical distress underwent splenectomy. Eleven regressed to stage A and 2 to stage B. All 6 patients with mechanical distress were relieved of their symptoms. Among 13 patients with anaemia, haemoglobin (Hb) level was normalised in 3, improved in 8 and showed no change in 2 cases. Among 13 patients with thrombocytopenia, platelet count was normalised in 10, improved in 1 and was unchanged in 2 cases. Mean Hb level increased from 89 g/l to 125 g/l and mean platelet count from 77 x 10(9)/l to 241 x 10(9)/l (p < 0.001 for both). Duration of response lasted for a mean period of 42.1 (CI 20.8-63.5) months. Mean survival after splenectomy was 44.5 months (CI 21.2-67.7). There was no operative mortality but 4 patients had significant post-operative morbidity. It is concluded that splenectomy is beneficial in advanced cases of CLL with massive splenomegaly and cytopenia.

Anemia↗

Splenectomy in lymphoproliferative disorders: a report on 70 cases and review of the literature.

Between February, 1970 and September, 1991, we performed splenectomies on 70 patients with chronic lymphoproliferative disorders including primary leukemias: 19 B-cell chronic lymphocytic leukemia, 1 B-cell prolymphocytic leukemia, 22 hairy cell leukemias, 4 large granular lymphocytic leukemias, 1 T-cell prolymphocytic leukemia, and non-Hodgkin's lymphomas (NHL): 10 splenic lymphomas with villous lymphocytes, 4 follicular lymphomas, 5 mantle cell lymphomas, 3 lymphoplasmacytic and 1 large cell NHL. The primary indications for surgery in this series were therapy-resistant disease (40%) and therapeutic splenectomy (38%). Postsplenectomy, 70% of patients had a complete hematological response, 23% had a partial response, and 7% were nonresponsive. Median treatment-free survival correlated with the hematologic response postsplenectomy and the underlying diagnosis. Better treatment-free survivals were seen in patients with lesser degrees of anemia and thrombocytopenia. Overall, improvements were more pronounced in the B-cell than in the T-cell disorders. Indications for further therapy, postoperative morbidity and mortality, and survival times are discussed along with a review of the literature. These findings advocate a continuing role for splenectomy in symptomatic lymphoid malignancies running with splenomegaly and hypersplenism.

Adult↗

Resolution of autoimmune hemolytic anemia following splenectomy in CD3+ large granular lymphocyte leukemia.

A 24 year old female with a 4 year history of anemia and absolute lymphocytosis was evaluated and found to have T cell large granular lymphocyte (T-LGL) leukemia associated with autoimmune hemolytic anemia, neutropenia, mild thrombocytopenia and splenomegaly. In an effort to ameliorate her symptomatic cytopenias, she was treated with prednisone and subsequently methotrexate without success. In February 1993, she underwent splenectomy for symptomatic anemia. Splenectomy resulted in an increased hemoglobin concentration to normal levels, resolution of all laboratory evidence of hemolysis, and disappearance of thrombocytopenia. This response has been durable despite persistence of the abnormal LGL clone. We suggest that splenectomy may be an effective treatment for autoimmune hemolytic anemia and/or thrombocytopenia often associated with T-LGL leukemia. As this disease often exhibits a chronic clinical course with morbidity resulting from consequences of resultant cytopenias rather than visceral involvement with leukemic LGL, effective treatment of cytopenias despite persistence of the abnormal LGL clone is beneficial.

Adult↗

Prognostic factors of response to laparoscopic splenectomy in patients with idiopathic thrombocytopenic purpura.

Laparoscopic splenectomy (LS) has become the treatment of choice for patients with idiopathic thrombocytopenic purpura (ITP) who do not respond to medical treatment. The aim of this study was to identify factors predictive of outcome after LS for ITP. From May 1997 to December 2002, we performed 30 LS on patients with ITP. A positive response was defined as a postoperative platelet count greater than 50,000/micro L and no requirement for maintenance therapy. Chi-square testing was performed to determine the predictive effects of the following variables: age, sex, preoperative response to steroids or immunoglobulin, duration of disease, antiplatelet antibody, platelet associated antibody, and antinuclear antibody. LS was successfully performed in all patients. For a mean follow-up interval of 24.3 months, response to LS was 73.3%. Splenectomy for steroid nonresponders resulted in an inferior complete response rate (10 of 18, 55.6%) as compared with those that experienced relapse after steroid treatment (11 of 12, 91.7%) (p=0.042). The other significant predictor of outcome by univariate analysis was the time between diagnosis and surgery (p=0.049). The other variables showed no significant correlation with successful splenectomy. We conclude that LS can be performed safely with a satisfactory remission rate in patients with ITP who do not respond to medical treatment, and that the factors most frequently associated with surgical success are a response to steroid and disease duration.

Adolescent↗

Reduction of virus burden-induced splenectomy in patients with liver cirrhosis related to hepatitis C virus infection.

AIM: To examine the hepatitis C virus (HCV) levels and immunological markers in cirrhotic patients after splenectomy. METHODS: HCV RNA titers as well as cellular and humoral immune markers were determined in 20 cirrhotic patients after splenectomy and in 32 cirrhotic controls with an intact spleen. RESULTS: Serum HCV RNA titers were lower in the splenectomized patients than in the controls (186+/-225 x 10(3)copies/mL vs 541+/-417 x 10(3) copies/mL, P<0.01). HCV RNA was judged to have been spontaneously eradicated in 4 splenectomized patients, but in none of the controls. Natural killer cell activity was higher in the splenectomized patients than in the controls (41.2+/-19.3% vs 24.7+/-15.3%, P<0.01), and natural killer cell activity was negatively correlated to HCV RNA titers in the splenectomized patients except in those with serotype 2-related infection. The CD4/CD8 ratio was significantly lower in the splenectomized patients than in the controls. CONCLUSION: The findings suggest that splenectomy may diminish virus burden in cirrhotic patients with HCV infection at least in part, through augmentation of natural killer cell activity.

Aged↗

The effect of splenectomy on the pathophysiology and egg-specific immune response of Schistosoma mansoni-infected mice.

Splenic involvement in murine schistosomiasis mansoni is manifested by splenomegaly, hyperplasia of the lymphoid and mononuclear phagocytic elements, and strong immune responses of splenic lymphocytes to schistosome antigens. In the present study, groups of Schistosoma mansoni-infected mice were splenectomized or sham treated at 1, 4, and 8 weeks of the infection, and at 12 weeks changes in pathophysiology, humoral, and granulomatous responses as well as liver fibrosis were examined. Splenectomy had no influence on the body and liver weight or portal presure of the infected mice. Mice which underwent splenectomy at 8 weeks developed anemia. Whereas splenectomy performed at 8 weeks of the infection did not affect the anti-schistosome egg antigen humoral response of the animals, it caused significant enhancement in the liver granulomatous reaction. Increased granuloma size did not result in a concomitant increase in the extent of liver fibrosis as measured by hydroxyproline content. It is concluded that the spleen may play an important role in the immunoregulation of the egg-specific granulomatous inflammatory response.

Animals↗

Hematological and biochemical changes and results of aerobic bacteriological culturing in dogs undergoing splenectomy.

Changes in complete blood count (CBC), bone marrow, reticulocyte count, coagulation profile, biochemical analysis, and serum iron, transferrin, and immunoglobulin M (IgM) concentrations were measured three and 10 days after splenectomy in 12 dogs. Spleens were cultured aerobically for bacteria and submitted for histopathological evaluation in 23 dogs undergoing splenectomy. There were no consistent changes in red blood cell (RBC), white blood cell (WBC), or platelet counts; bone-marrow samples; or biochemical profiles. Serum iron, transferrin, and IgM concentrations remained normal. Eight (35%) bacterial cultures yielded growth. Five of the 23 dogs had pyrexic episodes two-to-five days after surgery. In contrast to previous reports done on healthy dogs, this study shows that dogs with splenic disease have no characteristic changes in hematological or biochemical parameters after splenectomy. Rather, the changes tended to reflect the primary disease process. Splenic vascular compromise or a decrease in processing of bacteria may have resulted in the bacterial growth. There was no direct correlation to pyrexic postoperative episodes.

Animals↗

Management of adult patients with persistent idiopathic thrombocytopenic purpura following splenectomy: a systematic review.

BACKGROUND: Treatment of chronic refractory idiopathic thrombocytopenic purpura is a dilemma because many patients have minimal symptoms, response to treatment is uncertain, and treatments may have serious adverse effects. PURPOSE: To determine the effectiveness of treatments for adult patients with idiopathic thrombocytopenic purpura who have not responded to splenectomy. DATA SOURCES: English-language reports from 1966 through 2003 that were retrieved from MEDLINE and Reference Update and bibliographies of retrieved articles. STUDY SELECTION: Articles reporting 5 or more total patients were reviewed to select eligible patients. Patients were eligible for inclusion if they were more than 16 years of age, had idiopathic thrombocytopenic purpura for more than 3 months, had a previous splenectomy, and had a platelet count less than 50 x 10(9) cells/L. DATA EXTRACTION: Patients were assessed for platelet count response, bleeding complications, duration of follow-up, and death. Complete remission was defined as a normal platelet count with no treatment for more than 3 months and for the duration of follow-up. DATA SYNTHESIS: 90 articles with 656 patients treated with 22 therapies met selection criteria. Azathioprine, cyclophosphamide, and rituximab had the most reported complete responses, but they were reported in only 41 to 109 patients. Reported complete response rates ranged from 17% to 27%, but 36% to 42% of patients had no response with these 3 treatments. Most reports described only platelet count responses; bleeding outcomes were reported in only 63 patients (10%). Only 111 (17%) of the 656 eligible patients had pretreatment platelet counts of less than 10 x 10(9) cells/L. No treatment method was reported in more than 20 patients. CONCLUSIONS: Evidence for the effectiveness of any treatment for patients with idiopathic thrombocytopenic purpura and persistent severe thrombocytopenia after splenectomy is minimal. Potentially effective treatments must be evaluated by randomized, controlled trials to determine both benefit and safety.

Adult↗

Felty's syndrome: granulocyte-bound immunoglobulin G and splenectomy.

A quantitative antiglobulin consumption technique was used to measure immunoglobulin G (IgG) present on human granulocytes. Granulocytes from 50 normal subjects had less than 20 X 10(-14) g IgG per cell. Patients with granulocytopenia due to bone-marrow failure, patients with IgG multiple myeloma, patients with splenomegaly, and patients with rheumatoid arthritis without granulocytopenia had granulocyte-bound IgG within the range of normal. Four patients with rheumatoid arthritis, splenomegaly, and severe granulocytopenia (Felty's syndrome) had granulocyte-bound IgG between 30 and 220 X 10(-14) g IgG per cell. One of these patients underwent splenectomy, after which his granulocyte-bound IgG fell to normal. Seven additional patients with Felty's syndrome who had previously undergone splenectomy had normal levels of granulocyte-bound IgG. Thus quantitation of granulocyte-bound IgG appears to be useful in defining patients with immunologically mediated granulocytopenia. Studies of patients with Felty's syndrome who have undergone splenectomy suggest that the spleen may produce this neutrophile-bound immunoglobulin.

Adult↗

[Laparoscopic splenectomy].

From 1995 to 1998, 14 patients have been treated with laparoscopic splenectomy. Seven patients had immune thrombocytopenic purpura (ITP), six hereditary spherocytosis and one chronic myelomonocytic leukaemia with trombocytopenia. 12 of the patients had normal or nearly normal sized spleen. Median duration of surgery was 156 minutes and the median postoperative hospital stay four days. All operations were completed laparoscopically. Three patients had postoperative fever without any sign of infection, one developed urinary retention and one was readmitted with pneumonia. The patient with chronic myelomonocytic leukaemia died 15 days postoperatively from an intracerebral bleeding. Two patients suffer from relapse of trombocytopenia, one is treated with steroids. Laparoscopic splenectomy can be performed safely in patients with normal sized spleen with all the advantages of minimal access surgery. However, problems related to identification of accessory spleens and splenectomy in patients with splenomegali, should be further evaluated.

Adolescent↗

Laparoscopic splenectomy: the latest modern technique.

BACKGROUND/AIMS: Recent advances in technical instruments have resulted in increased safety and simplicity in laparoscopic surgery. The purpose of this article is to introduce our latest operative techniques for laparoscopic splenectomy. METHODOLOGY: The patient is placed in the right semidecubitus position and the gastrosplenic ligament including the short gastric vessels was performed by using an ultrasonically activated scalpel. The splenic artery and vein were resected at the splenic hilum with an autosuture device. The electromechanical morcellator was used to remove the spleen. RESULTS: The laparoscopic splenectomy was successfully performed in all 74 patients from 1992-1997. There was no deaths related to the operation. Conversion to open surgery with a small incision of 5 cm was required in one patient with advanced liver cirrhosis and portal hypertension and 45 patients with portal hypertension. CONCLUSIONS: A laparoscopic splenectomy is considered to be a safe and feasable modality for the treatment for hematologic disorders of both the spleen and other benign tumors.

Humans↗

[Massive thrombosis of the superior mesenteric artery following splenectomy. A coincidence?].

Postsplenectomy thrombocytosis is a well recognised complication about which there is little published information. Therefore, postoperative complications of this effect have not been emphasised. An experience with a case of superior mesenteric artery thrombosis and small intestinal ischemia following splenectomy is reported. We reviewed the literature, but we wasn't able to find any case, whereas the association between splenectomy and mesenteric or portal vein thrombosis is well known. Pathogenesis of postsplenectomy thrombocytosis is poorly understood. Theories to explain it include removal of the splenic sequestration effect or removal of a regulatory humoral factor produced by the spleen. Both mechanisms could be operative at the same time, explaining the observation that some patients develop thrombocytosis related complications soon after surgery, while others after a longer time. The literature on the thromboembolic risk of postsplenectomy thrombocytosis is inconclusive and no studies have established whether patients with thrombocytosis following splenectomy should be treated with anticoagulants or antiplatelet medications in order to prevent thrombotic complications. Certainly, a recommendation for the routine use of these drugs cannot be made on the basis of one observation, but the need for controlled studies must be stressed.

Aged↗

Hemodynamic responses to captopril during splenectomy in thalassemic children.

Splenectomy in beta-thalassemic children is frequently accompanied by perioperative hypertension which occasionally is followed by convulsion. The efficacy of captopril in attenuating the hypertensive response to splenectomy was investigated in 82 thalassemic children. The control group, consisting of 40 patients, received intravenous furosemide (1 mg/kg) preoperatively; whereas, 42 children were randomly allocated into 2 groups to receive oral captopril (0.7 mg/kg) or a combination of captopril (0.7 mg/kg) and furosemide (1 mg/kg) before the operation. Before anesthetic induction, both systolic and diastolic arterial pressures in the captopril and the combined groups were significantly lower than the furosemide group (P < 0.001), whereas, the heart rates in all groups were comparable. Changes in arterial pressure in response to the operation were significantly smaller in the combined group when compared with the other two groups (P < 0.001). Immediate postoperative hypertension requiring additional management occurred in 20 per cent of the furosemide group, and 14.3 per cent in the other two groups. One patient in the combined group had a convulsion in association with hypertension. The authors conclude that captopril combined with furosemide effectively controls intraoperative hypertension in thalassemic children undergoing splenectomy; however, postoperative hypertension remains common, and needs appropriate treatment immediately.

Administration, Oral↗

Laparoscopically assisted splenectomy following preoperative splenic artery embolization using contour emboli for myelofibrosis with massive splenomegaly.

Laparoscopically assisted splenectomy with an 8- to 10-cm left upper paramedian laparotomy was performed following preoperative splenic artery embolization using painless contour emboli (super absorbent polymer microsphere) with early successful results in two men (46 and 37 years old) with myelofibrosis accompanied by massive splenomegaly. Dissection around the lower part of the spleen and the hilum initially was performed intracorporeally with the usual laparoscopic view under 12 mm Hg pneumoperitoneum. The alternating changes of viewpoints between the direct view through an 8- to 10-cm incision and the usual laparoscopic view with or without application of a retraction method were effective for safe hilar devascularization. Preoperative splenic artery embolization at the distal site was effective for safe dissection around the enlarged spleen. The patients did not complain of pain before operation. Preoperative painless embolization and laparoscopically assisted splenectomy with small laparotomy promotes the feasibility and safety of minimally invasive splenectomy for myelofibrosis with massive splenomegaly.

Adult↗

Delayed growth and puberty in patients with Gaucher disease type 1: natural history and effect of splenectomy and/or enzyme replacement therapy.

BACKGROUND: Growth retardation in childhood was only recently recognized as a prominent feature of Gaucher disease type 1, but there are few data on both the pubertal development and the final outcome of growth and sexual maturation. OBJECTIVE: To investigate the natural pattern of growth and puberty in patients with Gaucher disease type 1 and the effect of splenectomy and enzyme replacement therapy. METHODS: We retrospectively analyzed growth and puberty in 57 patients with Gaucher disease type 1; 52 were followed since childhood and/or prepuberty and 42 have reached sexual maturity and final height. In the analysis we considered severity of disease, time of splenectomy, and start of enzyme replacement therapy. RESULTS: Deceleration of growth at age 3-5 years was observed in 30 of 57 patients followed since early childhood while untreated: height-SDS decreased from -0.34 +/- 0.42 at age 0-3 years to -1.93 +/- 0.95 (P < 0.01) at age 7-10 years and was more pronounced with severe disease. A high prevalence (59.6%) of delayed puberty, which was more frequent with severe disease, was observed in 47 patients followed before and throughout puberty. No primary endocrine pathology was found. All patients, untreated as well as treated, with growth and pubertal delay had a spontaneous catch-up, achieved full sexual maturation, and most (83.3%) reached a final height within the range of parental height-standard deviation score. Splenectomy (partial and/or total) performed in 20 patients while still growing had a beneficial effect on growth, which was temporary in some and did not affect puberty. ERT improved growth in 11 patients who started therapy before puberty, as evidenced by a progressive increase in the height-SDS, and seemed to normalize the onset of puberty. CONCLUSIONS: Growth retardation in childhood and delay of puberty are characteristic of Gaucher disease type 1 and are more frequent with severe disease. There is a spontaneous catch-up later in life and most patients reach a final height within their genetic growth potential. Enzyme replacement therapy apparently normalizes growth and possibly also the onset of puberty.

Adolescent↗

[Esophageal-gastric hemorrhage in patients with portal hypertension treated by splenectomy].

The authors report their observations over 336 patients previously subjected to splenectomy for the portal hypertension syndrome. Prior to surgery 66.4% of patients showed esophageal varices, 43.7% - bleeding. After the operation venous varices were noted in 48.2% of patients, bleeding - in 34% of all patients. The causes of hemorrhage occurred in later terms after splenectomy are analysed. A conclusion is made concerning the way how to minimize a probability of hemorrhage recurrence following splenectomy.

Esophageal and Gastric Varices↗

[Phagocytosis in patients with splenectomy].

Phagocytosis was evaluated in nine patients with splenctomy undergoing renal transplant, eight renal transplanted patients without splenectomy, three immunodepressed patients with terminal renal failure, four patients with splenectomy and six healthy volunteers. For phagocytosis evaluation, neutrophils adhered to glass were used. They were cover with 4% latex and albumin containing culture medium with or without phagocytosis stimulating tetrapeptide extracted from IgG in each case. Phagocytosis was decreased in all groups studied. Immunodepressed patients showed the highest diminishment of phagocytosis and patients with splenectomy and immunodepression the lesser. It was speculated that such a finding is due to a decrease of tetrapeptide in serum.

Acute Kidney Injury↗

[Laparoscopic splenectomy with a postero-lateral approach in patients with idiopathic thrombocytopenic purpura].

UNLABELLED: The aim of the study is presentation of operative technique and early results of laparoscopic splenectomy in 15 cases of idiopathic thrombocytopenic purpura (ITP) patients resistant to typical steroid therapy. There were 7 women and 8 men in age 16-58 years (mean 32.6 years). Preoperative investigations revealed accessory spleens in two patients. Platelets count at the time of qualifying patients to splenectomy varied from 7-35 g/L and after preoperative treatment (prednisone 1 mg/kg--6 patients, methylprednisone 25 mg/kg--5 patients, immunoglobulins 2.0/kg--2 patients, immunoglobulins and methylprednisone--2 patients) varied from 36-205 g/L. Splenic length was from 8-17 cm. The lateral approach and 4-5 ports were utilized in all cases. Pneumoperitoneum with value of 12-14 mmHg of carbon dioxide was created using an open technique. First step of the procedure was to free spleno-colic ligament, then spleno-reno attachment. In all cases the hilus artery, vein and short gastric vessels were clipped and divided by Endo GIA stapler 30 or 60 mm and clips. Spleens were removed from the abdomen cavity in the plastic bag after manual/forceps crushing inside the bag. Intraoperative bleeding occurred in 2 patients (20%). Blood loss was about 800 and 1000 ml respectively. The mean operative time was 170 min (140-300 min). Spleen weight was 210 g average. In 3 cases conversion to open approach was needed because of injury of splenic vessels (2 pts.) and difficulty in removing spleen--in 1 patient. Post operative course was uneventful in every 10 patients. Mean hospital stay was 5.8 days. Increase in platelet count was observed in 14 patients on the second postoperative day to 60-291 g/L. One patient did not response as expected. No early postoperative complications were observed. CONCLUSION: Laparoscopic splenectomy may be considered as a safe and feasible procedure in selected patients with ITP without long-term improvement after typical conservative treatment.

Adolescent↗