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[Hemoglobinopathy C and splenomegaly in an Ivory Coast patient. Value of splenectomy].

We report the case of a young patient native of the Ivory Coast who suffered from homozygous hemoglobin C disease. He presented with the usual findings of this hemoglobinopathy: a moderate hemolytic anemia and a massive, painful and even disabling splenomegaly. Pain completely disappeared following splenectomy. However, postoperative course was complicated by portal venous thrombosis, which was medically treated. No deficiency of natural coagulation inhibitors could be demonstrated, so splenectomy was the only factor predisposing to thrombosis. We consider that in only very few cases of hemoglobin C disease, splenectomy (preceded by prophylactic antipneumococcic vaccine) may be indicated from pain and risk of spontaneous splenic rupture.

Abdominal Pain↗

[The treatment of splenic injuries from splenectomy to non-operative management: our experience on 429 cases].

BACKGROUND: From the first successful splenectomy performed in 1893 the trend, in the management of splenic injuries has been increasingly toward avoiding splenectomy in favor of splenic preservation, either operatively or nonoperatively. The aim of this study is to evaluate our experience in the management of splenic injuries. METHOD: 429 Patients who suffered splenic injuries from 1989 to 2001, were examinated retrospectively. RESULTS: 120 Patients were treated non operatively; 270 were treated with splenectomy and 39 with operative preservation. The mortality rate was 6.8% but no Patient treated nonoperatively or with surgical preservation died. CONCLUSION: The splenic preservation either operatively or nonoperatively is the treatment of choice of splenic injuries in all Patients irrespective of the grade of injury or the age of the Patient.

Adolescent↗

Subtotal splenectomy for treatment of retarted growth and sexual development associated with splenomegaly.

This communication presents a new alternative for the treatment of retarded growth and sexual development associated with spleno- megaly: subtotal splenectomy, preserving the upper splenic pole supplied only by the splenogastric vessels, to avoid adverse effects of total splenectomy. We performed this procedure associated with central splenorenal shunt or portal-variceal disconnection in 3 teenagers with portal hypertension due to Schistosomia-sis Mansoni, complicated by variceal bleedings. All of them presented retarded growth and sexual development. All patients had uneventful postoperative follow-up, and normal growth and sexual development after the surgery. Subtotal splenectomy should be considered for treatment dwarfism associated with splenomegaly.

Adolescent↗

[Splenectomy in four cases of hairy cell leukemia].

This paper reports the experimental diagnosis of hairy cell leukemia and the results of splenectomy in 4 cases. Three patients were examined using histochemical methods and anti-leukocyte differentiation monoclonal antibodies. All of them were diagnosed as hairy cell leukemia of B lymphocytes. The effects of splenectomy were found to be related to chemotherapy used before and after operation. Hairy cell leukemia complicated with megalosplenia or hypersplenism were indications for splenectomy.

Adolescent↗

[Indications and early results of splenectomy in hematologic diseases].

In this retrospective study, we present 245 patients with various hematological diseases, who had undergone splenectomy for diagnostic or therapeutic purpose in our Department during the last 20-year period (1971-1991). There were 138 men (56%) and 107 women (44%), with a mean age of 49 years. The hematological diseases, for which the splenectomy had been performed, were according to the frequency of admittance: hemolytic anemia, complicated or not by gallstone formation, Werlhoff disease (thrombocytopenic purpura), Hodgkin's disease, hairy-cell leukemia, chronic lymphatic leukemia, non-Hodgkin lymphoma. A drain was placed in the splenic bed in all patients. All patients received anticoagulant therapy and antibiotics as well. Pneumococcal vaccination had been done systematically during the preoperative period. All patients received prophylaxis with a Penicillin for two years postoperatively. During the immediate postoperative period the mortality (1.2% OPSI: 1 case) and the morbidity (3.5% OPSI: 1 case) rates were very low. In conclusion, splenectomy in patients with hematological diseases is a safe procedure, even in high risk patients, but it requires a preoperative preparation and a close cooperation between surgeon and hematologist during the peri- and postoperative periods. Additionally, we have to notice that the possibility of an acute serious infection exists for any patient during the rest of his life.

Adolescent↗

[Effect of splenectomy on intra- and extraperitoneal tumor growth with reference to the immune system in a rat model].

In this model, splenectomy does not significantly influence the intra- and extraperitoneal tumor growth. Although the spleen accounts 25% of the cellular immune capacity, we found no difference in pro- or antiinflammatory cytokines but a significant increase of the white blood count after splenectomy compared with the control group. The influence of splenectomy on growth of intraperitoneal disseminated tumor cells must be investigated in further studies.

Adenocarcinoma↗

Coronary artery bypass in two patients with immune thrombocytopenic purpura without preoperative splenectomy.

Two patients with severe coronary artery disease and idiopathic thrombocytopenic purpura underwent coronary artery bypass grafting without preoperative splenectomy. The patients' thrombocytopenia and bleeding were controlled with platelet transfusion alone. This report indicates that the use of cardiopulmonary bypass in patients with immune thrombocytopenia does not invariably mandate splenectomy, particularly in those in whom performance of a splenectomy may be associated with an increased risk of intraoperative cardiac morbidity.

Coronary Artery Bypass↗

Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura.

BACKGROUND AND OBJECTIVES: Acquired thrombotic thrombocytopenic purpura (TTP) is often due to autoantibodies inhibiting ADAMTS-13 activity resulting in impaired processing of very large von Willebrand factor multimers. TTP usually presents with an acute onset and a fulminant, sometimes fatal course. With appropriate treatment including plasma exchange, and fresh frozen plasma replacement, often supplemented by immuno-suppressive therapy, the acute episode generally resolves within days to weeks. DESIGN AND METHODS: We describe the clinical course of 3 patients with acquired TTP. One was refractory to PE, the other 2 relapsed after this treatment. All three were treated with splenectomy. ADAMTS-13 activity and inhibitor levels were monitored. RESULTS: ADAMTS-13 activity was initially < 5% in all 3 patients. After splenectomy the inhibitor against ADAMTS-13 disappeared rapidly in 2 patients and there was full recovery of ADAMTS-13 activity in all 3 patients. INTERPRETATION AND CONCLUSIONS: Splenectomy, by eliminating a source of pathogenic autoantibody production, can be a successful treatment for patients with relapsing or plasma-refractory acquired TTP due to autoantibody-mediated ADAMTS-13 deficiency.

ADAMTS13 Protein↗

[Laparoscopic splenectomy in pediatrics. Review of 72 cases].

Splenectomy in childhood is nearly always related to hematologic disorders such as hereditary spherocytosis, immune thrombocytopenic purpura, sickle cell anemia and others. In recent years, laparoscopic splenectomy evolved into a safe and effective procedure that requires less hospital stay, and benefits the patient with a best postoperative period and excellent cosmetic results. The authors reveal a 6 year experience in treating 72 children with different diseases that required laparoscopic splenectomy. After progressing in their training curve and with the acquisition of new technology for endoscopic thermocoagulation, the authors conclude that minimally invasive surgery is the procedure of choice for most surgical spleen diseases in children.

Adolescent↗

[Laparoscopic splenectomy through a lateral access].

The investigation included 35 patients with diseases of blood and spleen pathology (30) and wounds and traumas of the abdomen with injuries of that organ (5), treated by laparoscopic splenectomy through a lateral access. The technique and stages of the operation and advantages of the lateral access are described. The laparoscopic splenectomy was shown to be possible in 90% of cases if patients were selected with special reference to the main disease, size and degree of pathological alterations in the spleen. An adequate approach to selections of patients with injuries of the organ allowed to make operations in more than a third of the patients and casualties. When using the lateral access laparoscopic splenectomy is thought to be more convenient, gives better visualization of the anatomical structures and reduces risk of injury of adjacent organs, thus resulting in less frequency of complications and conversions.

Abdominal Injuries↗

[The clinical application of splenectomy in pyrexia of unknown origin with splenomegaly].

OBJECTIVE: To observe the clinical value of splenectomy for pathologic diagnosis in fever of unknown origin with splenomegaly only. METHODS: The pathologic findings of 35 patients with fever of unknown origin and splenomegaly treated by splenectomy, admitted in to the department of hematology in our hospital since 1996 were studied retrospectively. For these patients, there were no other positive signs except splenomegaly and the routine tests could not help us make the etiological diagnoses. RESULTS: In these 35 patients, there were 17 cases of non-Hodgkin's lymphoma (48.6%), 5 cases of Hodgkin's disease (14.2%), 2 cases of malignant histiocytosis (5.7%), 5 cases of connective tissue disease (14.2%), 2 cases of chronic congestive splenomegaly (5.7%), 1 case of hemophagocytic syndrome (2.9%), 1 case of remote spleen infarction (2.9%), 1 case of tuberculosis of spleen (2.9%) and 1 case of spleen angiosarcoma (2.9%). CONCLUSION: When only splenomegaly is found in patients with fever of unknown origin, it is necessary to persuade the patients to accept diagnostic splenectomy for pathological as soon as possible, otherwise, the diagnosis and treatment may be delayed.

Adolescent↗

[Laparoscopic splenectomy: the learning process].

This is a report of our initial experience with laparoscopic splenectomy in children. From August 1997 to March 1999, 19 patients underwent laparoscopic splenectomy for various hematologic disorders. The ages ranged from 3 to 13 years. A four port technique was used to perform the procedure. All 19 procedures were completed successfully laparoscopically. The average surgical blood loss was 60 cc. The average operative time was 138 min and the length of stay in the hospital 2.5 days. One patient required a reintervention because of postoperative bleeding. We discuss the learning curve in our laparoscopic splenectomy cases.

Adolescent↗

An unusual association between splenectomy and Kikuchi's disease.

Kikuchi's disease is a clinico-pathologic entity of unknown etiology characterized by subacute inflammatory process of lymph nodes. It affects mostly women around the age of 30 years. It is usually a self limiting illness characterized by pyrexia, neutropenia, and cervical lymphadenopathy. We report a case of Kikuchi's disease in a patient with past history of splenectomy. A 35-year-old otherwise healthy female patient presented with 15 days history of fever, night sweats, and right cervical lymphadenopathy. She was on no medication and had no contact with animals or patients with tuberculosis. Her past history revealed splenectomy for thrombocytopenia 14 years before presentation. Lymphoma was suspected and she was referred for a cervical lymph node biopsy. The final histopathology diagnosis revealed subacute necrotizing lymphadenitis consistent with Kikuchi's disease. This is the first case of Kikuchi's disease presenting in a post splenectomy patient.

Adult↗

Impaired regulation of erythrocyte autoantibody production after splenectomy.

C3H mice were given 4 i.p. injections, eac of 2 X 10(8) WAG rat RBC, at weekly intervals. The production of erythrocyte autoantibodies elicited by the cross-reacting rat RBC was assessed using the average direct Coombs' test (DCT) score. Autoantibody production reached higher levels and persisted significantly longer in mice splenectomized 15 days before the first injection of rat RBC. This increased production of autoantibodies was not prevented by injecting each splenectomized mouse i.v. with 5 X 10(7) syngeneic spleen cells immediately after splenectomy. Similarly, splenectomy of mice already DCT+ significantly prolonged autoantibody production which was not prevented by injections of 10(8) cells prepared from the spleens removed at splenectomy. Transfer of spleen cells from mice already DCT+ to mice before the injections of rat RBC were started in the recipients caused a significant reduction in the amount of RBC autoantibodies produced. This suppression of autoantibody production was greater in unsplenectomized mice than in splenectomized mice. The results show that the spleen is involved in the regulation of these erythrocyte autoantibody responses. It is hypothesized that, in addition to the cellular component of the spleen, the splenic architecture and/or environment contributes to the regulation of autoantibody responses.

Animals↗

Splenic syndrome in patients at high altitude with unrecognized sickle cell trait: splenectomy is often unnecessary.

BACKGROUND: The health risks associated with sickle cell trait are minimal in this sizable sector of the world's population, and many of these patients have no information about their sickle cell status. Splenic syndrome at high altitude is well known to be associated with sickle cell trait, and unless this complication is kept in mind these patients may be subjected to unnecessary surgery when they present with altitude-induced acute abdomen. METHODS: Four patients were admitted to the surgical ward with a similar complaint of acute severe left upper abdominal pain after arrival to the mountainous resort city of Abha, Saudi Arabia. All were subjected to splenectomy because of lack of suspicion regarding sickle cell status. RESULTS: Histologic examination of the spleen showed all patients had sickle cells in the red pulp. On further assessment all were found to have sickle cell trait with splenic infarction. In a similar study of 6 patients with known sickle cell disease who had comparable problems when they travelled to the Colorado mountains, all made an uncomplicated recovery with conservative management. CONCLUSIONS: In ethnically vulnerable patients with splenic syndrome, sickle cell trait should be ruled out before considering splenectomy. These patients could respond well to supportive management, and splenectomy would be avoided.

Abdomen, Acute↗

Results of emergency surgery in patients with Moschowitz's disease refractory to hematological treatment: is splenectomy always advisable?

BACKGROUND: Patients with thrombotic thrombocytopenic purpura (TTP), Moschowitz's disease, run a high risk of perioperative bleeding and need intensive hematologic support. In some patients, TTP is associated with cancer but the surgical role in these patients is still unclear. To illustrate the surgical problems and outcome we present the case histories of three patients with TTP observed in our emergency department. MATERIALS AND METHODS: Two patients had TTP secondary to cancer and one patient with primary TTP (no evidence of neoplasia) had emergency operation for gastric hemorrhage, occlusion and TTP unresponsive to plasmapheresis. RESULTS: The first two patients who had not radical resection of cancer and no splenectomy, died for TTP complications. The third patient who underwent emergency splenectomy, had an uneventful postoperative course and TTP completely regressed. CONCLUSIONS: These case reports suggest that patients with TTP should be screened to rule out cancer. In patients with acute cancer-related complications emergency surgery should aim to resect the cancer. An associated splenectomy may increase the effectiveness of postoperative hematologic therapy.

Adult↗

[Laparoscopic splenectomy for epithelial cyst of the spleen].

Splenic cysts of all types are a relatively rare entity. The origin of epithelial cysts of the spleen is controversial, most probably congenital in origin. Generally, epithelial cysts of the spleen are asymptomatic and discovered incidentally. We present the case of a 40-year-old woman with a 3.5 cm splenic cyst diagnosed four years before. The cyst raised progressively in dimension, the actual size being of 6.5 cm. Computed tomography scan and magnetic resonance showed a central splenic cyst in close relations with main splenic vessels. An initial indication of partial splenectomy was established, eventually a total splenectomy being performed by laparoscopy due to close relations of the cyst with the main hilar splenic vessels. The hemostasis was assured with the aid of Ligasure Atlas 10 mm instrument. The pathology examination revealed a multilocular splenic cyst with a cuboidal epithelial lining. Central localization of splenic cysts represents an indication for total splenectomy. Laparoscopy provides a minimal access method of obtaining pathological confirmation of diagnosis, reduction of cyst complications, and a short hospital stay. This article discusses different aspects of epithelial cysts related to pathology, diagnostic and indications for operative treatment, a review of the literature being also presented.

Adult↗

Splenectomy in idiopathic hypersplenism.

The therapeutic effects of splenectomy in 15 patients with idiopathic hypersplenism were studied. The mean age was 43 years (range, 5-72 years). The male to female ratio was 1:1.14. The response to splenectomy was: in thrombocytopenia, complete response (CR) in 71%, partial response (PR) and no response (NR) in 14% each of patients; in anemia, CR in 83%, PR in 0%, and NR in 16% of patients; and in leukopenia, CR in 78% and PR and NR in 11% each of patients. However, the morbidity (27%) and mortality (20%) observed was quite high. Thus, we conclude that splenectomy is an excellent treatment to improve the hematological parameters in patients with idiopathic hypersplenism, but elderly patients and patients with multisystem disease should not have this surgery.

Adolescent↗