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Splenectomy: a strong risk factor for pulmonary hypertension in patients with thalassaemia.

OBJECTIVE: To determine the association between splenectomy and pulmonary hypertension in patients with thalassaemia with anaemia. DESIGN: Prospective cross-sectional study. METHODS: 68 patients with thalassaemia, who had a haemoglobin concentration of less than 100 g/l, were recruited into this study. Echocardiography was performed before clinical data were reviewed. Pulmonary artery pressure was estimated by measuring the systolic transtricuspid pressure gradient from tricuspid regurgitation and adding it to the right atrial pressure, which was estimated by the response of the inferior vena cava to inspiration. Pulmonary hypertension was defined as systolic pulmonary artery pressure > 35 mm Hg. History of splenectomy and other clinical data were compared between patients with and without pulmonary hypertension. RESULTS: 29 patients had pulmonary hypertension and 39 did not. Patients with pulmonary hypertension had significantly more nucleated red blood cells and higher platelet counts, and a higher prevalence of splenectomy (75.8% v 25.6%, odds ratio 9.1, 95% confidence interval 3.0 to 27.7). In multivariate analysis, splenectomy was the only factor significantly related to pulmonary hypertension. CONCLUSION: Splenectomy is a strong risk factor for pulmonary hypertension in patients with thalassaemia.

Adolescent↗

Implementing a policy for pneumococcal prophylaxis in a haematology unit after splenectomy.

People who have had a splenectomy for any reason are 40 times more likely to have an overwhelming infection, especially pneumococcal infection, and 17 times more likely to suffer fatal sepsis. The incidence of such life threatening infections is reduced by prophylactic immunisation with polyvalent pneumococcal vaccine and long term antibiotic prophylaxis or instituting prompt antibiotic treatment in the event of fever. This haematology unit agreed a policy of immunisation and antibiotic prophylaxis in June 1988 for all patients undergoing elective splenectomy. The success of this policy was audited in July 1993 by a retrospective analysis of patients' case notes. Seventy four patients were identified as having had a splenectomy, 54 (73%) before June 1988, of whom only 13 (24%) had received both pneumococcal immunisation and antibiotic prophylaxis before implementation of the agreed policy. At the time of audit, 46/74 (62%) patients were recorded as having received immunisation and 64/74 (86%) as receiving antibiotic prophylaxis or a supply of antibiotics to take in the event of a fever. All but one of the 20 patients who had a splenectomy after June 1988, since implementation of the agreed policy, received immunisation and antibiotic prophylaxis. The authors conclude that establishment of a formal agreed policy for pneumococcal prophylaxis for patients undergoing splenectomy has improved the quality of care.

Antibiotic Prophylaxis↗

Splenectomy and chronic thromboembolic pulmonary hypertension.

BACKGROUND: An increased prevalence of splenectomy has been reported in patients with idiopathic pulmonary arterial hypertension. Examination of small pulmonary arteries from these subjects has revealed multiple thrombotic lesions, suggesting that thrombosis may contribute to this condition. Based on these findings, we hypothesised that splenectomy could be a risk factor for chronic thromboembolic pulmonary hypertension (CTEPH), a condition defined by the absence of thrombus resolution after acute pulmonary embolism that causes sustained obstruction of the pulmonary arteries and subsequent pulmonary hypertension. METHODS: The medical history, clinical characteristics, thrombotic risk factors and haemodynamics of 257 patients referred for CTEPH between 1989 and 1999 were reviewed. In a case-control study the prevalence of splenectomy in patients with CTEPH was compared with that of patients evaluated during the same period for idiopathic pulmonary hypertension (n=276) or for lung transplantation in other chronic pulmonary conditions (n=180). RESULTS: In patients with CTEPH, 8.6% (95% CI 5.2 to 12.0) had a history of splenectomy compared with 2.5% (95% CI 0.7 to 4.4) and 0.56% (95% CI 0 to 1.6) in cases of idiopathic pulmonary arterial hypertension and other chronic pulmonary conditions, respectively (p<0.01). CONCLUSION: Splenectomy may be a risk factor for chronic thromboembolic pulmonary hypertension.

Case-Control Studies↗

Splenic vein thrombosis after splenectomy: frequency and role of imaging.

PURPOSE: To assess the frequency of splenic vein thrombosis (SVT) after splenectomy and its consequences on patient treatment. MATERIALS AND METHODS: A group of 183 consecutive patients who underwent splenectomy were evaluated. Of these patients, 119 underwent postoperative ultrasound (US) or computed tomography. RESULTS: SVT was diagnosed in 13 of 119 patients in the first 2 weeks after surgery. In these 13 patients, splenectomy had been performed for hematologic disorders (n = 12) or trauma (n = 1). Seven of the 13 patients were asymptomatic. After anticoagulant therapy, follow-up US of 12 patients showed resolution of thrombosis with no complications in 10 patients; two patients developed cavernous transformation of the portal vein. CONCLUSION: Since only 65% of patients underwent imaging, the true frequency of SVT could not be determined; however, it occurred in at least 7% of patients who underwent splenectomy. Routine Doppler US should be performed after splenectomy to allow early anticoagulant therapy in patients with SVT.

Humans↗

Changes in serum thrombopoietin levels after splenectomy.

To clarify the role of thrombopoietin (c-Mpl ligand, TPO) in 'hypersplenic' thrombocytopenia, we used an enzyme-linked immunosorbent assay to examine changes in serum TPO levels accompanied with splenectomy in 6 patients with liver cirrhosis, 4 patients with gastric cancer, and 2 patients with lymphoid malignancies. We also measured serum levels of other thrombopoietic cytokines such as interleukin-6 (IL-6) and erythropoietin. Platelet counts reached a maximum at day 14 after splenectomy in all subjects. In patients with liver cirrhosis, a lower elevation of platelet counts was observed compared with that in patients with gastric cancer. Serum TPO levels gradually elevated after splenectomy and reached a maximum 3.5 days after splenectomy in noncirrhotic patients, whereas peak serum TPO levels were delayed until day 7 in the cirrhosis group. IL-6 and erythropoietin showed similar kinetics between cirrhotic and noncirrhotic patients. These findings suggest that transient thrombocytosis after splenectomy may be associated with an alteration in the site of TPO catabolism by platelets from spleen to the blood and that deterioration of TPO production may play a role in thrombocytopenia in liver cirrhosis.

Adult↗

Liver dysfunction following splenectomy in idiopathic myelofibrosis: a study of 10 patients.

The hepatic abnormalities that developed after the splenectomy in 10 subjects with idiopathic myelofibrosis were analyzed. In all patients in whom a liver biopsy was performed during the splenectomy, extramedullary hematopoiesis was demonstrated, consisting of dysmorphic megakaryocytes primarily localized in the sinusoids, often accompanied by erythroid precursors. Following splenectomy, a significant increase in both the liver size and serum levels of alkaline phosphatase, bilirubin or gamma-glutamyl transpeptidase was found within 6 months, whereas no such increase was observed in the serum aspartate transaminase and alanine transaminase concentrations. In addition, 2 patients developed acute liver failure leading to death at 3 and 4 weeks from splenectomy, respectively. In contrast with these findings, no hepatic alterations were observed in 10 chronic myeloid leukemia patients who were also submitted to splenectomy.

Adult↗

Improvements in host immunity by partially purified interleukin 1 in rats with portacaval anastomosis and splenectomy.

Despite the high incidence and severity of bacterial infections in individuals with chronic liver disease, the relative role of host immunity and the effects of immune stimulants have not been fully investigated. To study the role of the liver and spleen in reticuloendothelial system (RES) function and the host response to infection following portacaval anastomosis, 107 Sprague Dawley CD rats received a portacaval anastomosis either with or without an additional splenectomy, or a sham procedure. Animals that had undergone portacaval anastomosis and splenectomy were also administered a nonspecific host immune stimulant, interleukin 1, or saline and the effect on blood bacterial clearance and organ uptake examined. Three to four weeks following surgery, animals that received a portacaval anastomosis had a decreased ability to clear 59Fe-labeled Pseudomonas aeruginosa from the blood and an increased uptake of bacteria in the spleen (p less than 0.01) when compared to sham-treated animals. Rats that received a portacaval anastomosis and splenectomy showed further decreases in blood clearance and increased sequestration of bacteria in the liver (p less than 0.01). Rats with a portacaval anastomosis and splenectomy that received interleukin 1 treatment prior to Pseudomonas bacteremia showed significantly improved blood bacteria clearance (p less than 0.05) and a reduced bacterial sequestration in the liver (p less than 0.001) when compared to similar animals receiving saline. These findings suggest that portacaval anastomosis and splenectomy result in impaired immune function as reflected by blood bacteria clearance and changes in organ sequestration of bacteria. Secondly, pretreatment of those immunocompromised animals with interleukin 1 improves immune function.

Animals↗

Effects of splenectomy on luteal function in pseudopregnant rabbits.

The involvement of the immune system in changes in luteal function was evaluated in rabbits. Pseudopregnancy was induced in 10 females and the spleens (considered to be the source of macrophages) of five were excised on day 7 of pseudopregnancy, while the five controls had sham splenectomies. Subsequent changes in serum progesterone concentrations were measured as an indicator of luteal function and luteolysis. A second pseudopregnancy was induced 31 days after splenectomy. The first pseudopregnancy was prolonged and during the second pseudopregnancy the serum progesterone concentrations on days 3 and 7 were much lower in the splenectomy group than in the control group. On day 14 of the second pseudopregnancy, the serum progesterone concentration markedly decreased in the control group while it remained almost at the level of day 7 in the splenectomy group. These results suggest that splenectomy suppresses the expression of luteal function and delays luteolysis in rabbits.

Animals↗

Life-threatening hypercoagulable state following splenectomy in ITP: successful management with aggressive antithrombotic therapy and danazol.

A life-threatening hypercoagulable state (HCS) is reported that developed after splenectomy in idiopathic thrombocytopenic purpura (ITP). A 50-year-old active male was rejected for blood donation because of an incidental finding of low platelet counts, 40,000/uL. The diagnosis was ITP. Although asymptomatic, he underwent splenectomy because of poor response to steroids and intravenous (IV) gamma globulin. One month after splenectomy, he suffered pulmonary emboli without deep venous embolism (DVT), followed by bilateral DVT, threatening amputation of the legs. Emergency thrombolysis, insertion of stent, and IV heparin saved his legs. Extensive workup for HCS was negative. IV heparin was withheld for colonoscopy for possible gastrointestinal neoplasm, at which time DVT recurred, necessitating another thrombolysis and heparin infusion. He was discharged on enoxaparin, antiplatelet therapy, and danazol. Platelet hyperactivation, characterized by high platelet microparticles (PMP) and CD62P, was present throughout his course of active ITP, resolving when ITP went into remission with danazol therapy. ITP has remained in remission for 4 years after stopping enoxaparin and danazol. In vitro, his plasma in active ITP induced activation of normal platelets, generating PMP and inducing CD62p-positive platelets and platelet aggregates; his plasma from remission had no effect. This indicates the presence of a platelet activating factor, possibly anti-platelet antibodies. Splenectomy may have allowed procoagulant PMP to accumulate to high levels resulting in HCS. We advise awareness of thrombotic complications post-splenectomy in the subset of ITP patients who are largely asymptomatic and exhibit persisting platelet activation.

Blood Coagulation Disorders↗

The paradoxical effects of splenectomy on tumor growth.

BACKGROUND: There is a vast and contradictory literature concerning the effect of the spleen and particularly of splenectomy on tumor growth. Sometimes splenectomy seems to inhibit tumor growth, but in other cases it seems, paradoxically, to facilitate both oncogenesis and the growth of established tumors. APPROACH: In this essay I have selected from this large literature a few papers that seem particularly instructive, in the hope of extracting some understanding of the rules governing this paradoxical behavior. CONCLUSION: In general, whether splenectomy enhances or inhibits tumor growth seems to depend primarily upon the ratio of spleen to tumor. Small proportions of spleen cells usually stimulate tumor growth, in which case splenectomy is inhibitory. Larger proportions of the same cells, especially if they are from immunized animals, usually inhibit tumor growth, in which case splenectomy results in tumor stimulation.

Animals↗

Interaction of splenectomy and perioperative blood transfusions on prognosis of patients with proximal gastric and gastroesophageal junction cancer.

PURPOSE: To assess the interaction of splenectomy and perioperative allogeneic blood transfusions on the prognosis of patients undergoing a potentially curative resection of proximal gastric and gastroesophageal junction (GEJ) cancer, because reports from the transplantation literature demonstrated that the immunosuppressive effects of transfusions are dependent on the presence of an intact spleen. PATIENTS AND METHODS: Between July 1, 1985, and July 30, 2001, 240 patients underwent complete resection (R0) of a proximal gastric or GEJ cancer (Siewert type II or III). Clinical and pathologic factors were collected in a prospective database. The survival data were modeled using the method of Kaplan and Meier and analyzed by the log-rank test and Cox regression. RESULTS: The median follow-up of the patients was 25 months (40 months for survivors). The median relapse-free survival was 30 months, and the median disease-specific survival was 45 months. Univariate analysis suggested an interaction of splenectomy and perioperative transfusion in their effect on relapse-free survival. Patients who received a perioperative transfusion but did not undergo splenectomy demonstrated the worst prognosis on multivariate analysis independent of other prognostic factors. In patients who underwent splenectomy, perioperative transfusion had no effect on relapse-free survival on multivariate analysis. CONCLUSION: Our study suggests an interaction of blood transfusion and splenectomy in their effect on survival paralleling the findings in the transplantation literature. The adverse effect of allogeneic blood transfusion on prognosis in patients with gastric cancer seems to be associated with the presence of an intact spleen and is abrogated by its absence.

Adenocarcinoma↗

Blastic transformation after splenectomy in a patient with nonvillous splenic marginal zone lymphoma with p53 overexpression: a case report.

A 61-year-old man with no subjective symptom was admitted to our hospital for further examination of the causes of anemia (hemoglobin, 9.5 g/dL) and thrombocytopenia (platelets, 9.2 x 10(4)/microL), which had been pointed out in a medical checkup half a year previously. A bone marrow examination showed 73% lymphoid cells. Immunophenotyping of these cells were CD19+CD20+CD3-CD5-CD10-CD23-, and light chain restriction (kappa) was positive by fluorescence-activated cell sorting analysis. A computed tomography scan showed mild splenomegaly. To confirm the diagnosis histologically, we performed a splenectomy. Finally, we diagnosed the patient's disease as nonvillous splenic marginal zone lymphoma (SMZL). A month after the splenectomy, the white blood cell count was remarkably increased to 7 x 10(4)/microL with the blastic transformation of lymphoid cells. We first treated the patient with fludarabine and then with the CHOP regimen (cyclophosphamide, hydroxydaunomycin, vincristine [Oncovin], and prednisone), but the disease was so refractory that the patient died of the disease 13 months after the splenectomy. Immunohistochemical staining and a molecular examination for p53 were carried out with specimens from the splenectomy. We found overexpression of the p53 protein in lymphoid cells and a point missense mutation in codon 280 at exon 8 that changed AGA (Arg) to AGT (Ser). This case may indicate the existence of a more aggressive subset of SMZL, suggesting a reconsideration of the roles of splenectomy and p53 overexpression in the diagnostic and therapeutic approaches to patients with SMZL.

DNA Mutational Analysis↗

Laparoscopic splenectomy: present status and future perspective.

Laparoscopic splenectomy has become widely accepted as the approach of choice for the surgical treatment of benign and malignant hematologic diseases. Advances in technology have led to better outcomes for the procedure, and have allowed surgeons to apply the technique to disease processes that were at one time felt to be contraindications to laparoscopic splenectomy. However, challenges still remain. There is a steep learning curve associated with the procedure. The development of cost-effective laparoscopic simulators to target the skills required for laparoscopic splenectomy and other laparoscopic procedures is essential. The advent of devices which isolate and seal the large blood vessels that surround the spleen have reduced intra-operative bleeding and minimized conversions to open splenectomy. Improvements in optics and instrumentation, as well as robotic technology, will continue to define the frontier of minimally invasive surgery, and further facilitate the acceptance of laparoscopic splenectomy for the treatment of benign and malignant hematologic diseases.

Animals↗

[Intraoperative and postoperative complications of splenectomy].

Spleen is being surgically removed because of trauma, in diagnostic and-or therapeutical purposes because of the benignant and malignant diseases. The percentage of morbidity during and after splenectomy is relatively low. During surgery might occur bleeding, trauma of the pancreatic tail, stomach, lineal flexure of the colon, left hemidiafragm, left suprarenal gland and upper pole of the left kidney, which must be correspondingly reclaimed during the same intervention. In the early postoperative period, postoperative bleeding, subfrenic abscess, pulmonal atelectasis, bronchopneumonia and left pleural extravasations might occur. Especially is important notification of these events in due time and adequate conservative and surgical treatment. After splenectomy, there is an increase of the number of trombocytes, which might lead to the tromboembolic complications. In the prevention of these complications in the postoperative period prolonged antiagregation therapy is suggested. Postsplenectomy sepsis is very late, general complication of splenectomy, which occurs because of the lower immunity in the child age. To prevent these complications, partial splenectomies, reimplantations of the spleen, prolonged application of the penicillin medicines after splenectomy and antipneumococcal vaccine are performed.

Humans↗

Late infections following splenectomy in Hodgkin's disease.

Here 145 reported post-splenectomy infections in 115 patients with Hodgkin's disease are reviewed. Such infections can occur at any age (median age 19.8 years) and the interval from splenectomy to the infection is quite variable (median 21.9 months). Most infections are present clinically as pneumonia, septicemia, meningitis, or a combination thereof, with the most common offending organism being pneumococcus. The infection can be fulminant and even fatal. It is recommended that the spleen should only be removed when essential. A staging laparotomy and splenectomy seem justifiable only in cases of Hodgkin's disease where a change of staging would lead to a change of planned therapy. Prophylactic penicillin should be administered at least for 3 years and possibly indefinitely since post-splenectomy infections can occur many years later. Pneumococcal vaccine should also be given before any chemotherapy or radiotherapy is initiated. Prompt and aggressive treatment should be given when post-splenectomy infection is diagnosed.

Adolescent↗

Durable remission after splenectomy for Waldenström's macroglobulinemia with massive splenomegaly in leukemic phase.

A patient with M-proteinemia (IgM, kappa type), lymphocytosis, anemia, and massive splenomegaly, was diagnosed as having Waldenström's macroglobulinemia (WM). Since this case was refractory to chemotherapy, splenic irradiation was performed, which effectively reduced the serum IgM level, spleen size, and lymphocyte counts; however, its effect was transient. Splenectomy was then carried out. The spleen contained abundant IgM-producing lymphocytes, and after splenectomy, the serum IgM values decreased and the peripheral blood counts returned to near normal. The transient increases of serum IgM occurred during two infectious episodes postoperatively. The patient has now been in a satisfactory remission for six years after splenectomy. The removal of an IgM-producing/secreting site and release from hypersplenism may be the major mechanisms involved in achieving the durable remission after splenectomy. In individual cases of WM with massive splenomegaly, we recommend splenectomy as part of the management of this disorder.

Humans↗

Splenectomy does not cure the thrombocytopenia of systemic lupus erythematosus.

Fourteen patients with systemic lupus erythematosus had splenectomies done between 1960 and 1982 for treatment of severe thrombocytopenia. Thrombocytopenia persisted or recurred within 1 month postoperatively in five patients and within 6 months in three others. Three patients had late recurrence (18, 30, and 54 months after splenectomy); in two it was probably related to withdrawal of immunosuppressive agents or corticosteroids. Median lowest platelet count before splenectomy and median platelet count at relapse or failure of splenectomy were both 8000/microL. Only two patients maintained normal platelet counts without need for corticosteroids or other treatment. These results differ from those in patients with idiopathic thrombocytopenic purpura. Other treatments should be tried before splenectomy is done for thrombocytopenia in patients with systemic lupus erythematosus.

Adult↗

Splenectomy in the chronic phase of chronic granulocytic leukemia. Effects in 32 patients.

Splenectomy in the chronic phase of chronic granulocytic leukemia was done in 32 patients, in 24 as part of a trial of elective splenectomy in early disease and in 8 because of thrombocytopenia preventing adequate therapy of the chronic phase. Patients with splenectomy had a median survival after diagnosis of 60 months. However, survival of patients operated on within 1 year of diagnosis was no different (median, 44 months) from that recorded in 120 consecutive Philadelphia chromosome-positive patients treated during the same time period (median, 42 months). After blastic transformation of disease, splenectomy patients survived slightly longer, responded more frequently to platelet transfusions, and avoided the morbidity of massive splenomegaly seen in 30% of patients treated with spleens retained. Response to blastic phase chemotherapy, however, was not improved. Prophylactic splenectomy in the chronic phase of disease did not influence survival but may ameliorate some complications of the terminal stages of illness.

Adult↗