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Pros and cons of splenectomy in patients with myelofibrosis undergoing stem cell transplantation.

During fetal development, the spleen is a major hemopoietic organ. In the adult human, this task is relinquished to the bone marrow. However, under the stress of certain pathologic conditions, extramedullary hemopoiesis may again occur in the spleen. This is especially true for diseases of the marrow, in particular, myeloproliferative disorders such as agnogenic myeloid metaplasia, which is associated with severe fibrosis of the marrow space. At the same time, the spleen sequesters blood cells and contributes to peripheral blood cytopenias, which may improve following splenectomy. However, success is unpredictable, and the operative mortality of splenectomy is on the order of 10%. As a growing number of patients undergo hemopoietic stem cell transplantation as definitive therapy for myelofibrosis, the decision on splenectomy has additional ramifications since the spleen plays an important role in the kinetics of engraftment of donor cells and in immune reconstitution. We conclude from our analysis of available information that the benefit of splenectomy is difficult to predict, although after transplantation splenectomized patients have faster hemopoietic recovery. It appears that the most important indication for splenectomy in these patients is the relief of symptoms from massive spleen enlargement.

Adolescent↗

Role of splenectomy in the treatment of myelodysplastic syndromes with peripheral thrombocytopenia: a report on six cases.

Thrombocytopenia is generally of central origin in MDS, but can be due to peripheral platelet destruction in some cases. We studied platelet lifespan in 61 MDS cases with platelets < 70,000/mm3 and marrow blasts < 10%. Nine of them (15%) had a major platelet lifespan reduction (< 3.5 days), and were considered for splenectomy. Three of them were not splenectomized due to rapid death, patient refusal and older age plus liver predominance of platelet sequestration, respectively. The remaining six patients (two females and four males, median age 50 years, range 32 to 65) were splenectomized 3 to 21 months after diagnosis. Before splenectomy, five of them had RA and one had CMML. Platelets counts ranged from 5000 to 30,000/mm3 and did not durably respond to other treatments. Three of the patients has a relapse of platelet counts, concomitantly required platelet transfusion due to recurrent blending, whereas three had anemia (two required erythrocyte transfusion) and four had neutropenia. Three months after surgery, platelet counts ranged from 55,000 to 160,000/mm3 (> 100,000/mm3 in four cases), no patient required platelet or erythrocyte transfusion, but there was no effect on neutrophil counts. Three patients had a relapse of platelet counts, concomitant with progression to AML in two of them, whereas the third relapsing case achieved normal platelet counts with further danazol. One patient died with normal platelet counts 12 months after splenectomy (from sepsis, probably related to neutropenia rather than splenectomy). Two patients remained with normal platelet counts 10 and 52 months after surgery. Our findings suggest that the mechanism of thrombocytopenia should be studied more often in 'low risk' MDS (i.e. with low bone marrow blast counts) with thrombocytopenia, as about 15% of them appear to have peripheral platelet destruction. Some of those patients may benefit from splenectomy.

Acute Disease↗

Experience with laparoscopic splenectomy.

BACKGROUND/PURPOSE: The laparoscopic splenectomy (LS) often is adopted to treat children affected by hematologic diseases. Many of the pitfalls of LS are related to the 2 steps-dissection and extraction. Although various methods have been adopted, the conversion rate still is too high during the learning curve period. The authors analyse their experience in 54 laparoscopic splenectomies performed by their teams in 3 European countries. METHODS: From 1995 to 1999, 54 children underwent laparoscopic splenectomy, 4 of whom also underwent a concomitant cholecystectomy. There were 29 girls and 25 boys with ages ranging between 4 and 19 years (median, 8.1 years). All patients underwent an elective laparoscopic splenectomy: Thirty children had hereditary spherocytosis, 13 had an idiopathic thrombocytopenic purpura, 10 were affected by a beta thalassemia, and 1 child had sickle cell disease. RESULTS: Mean operating time was 140 minutes (range, 100 to 250 minutes). Hospital stay ranged from 2 to 6 days (median, 3 days). In 7 patients the spleen was removed through a 7-cm minilaparotomy; in another 46 cases the spleen was captured into an extraction bag, fragmented, and then removed through the umbilical or left orifice. There was one conversion to open surgery because of a camera failure during the operation. CONCLUSIONS: On the basis of our experience we believe that the operating time of LS is still too long compared with open surgery, and the extraction phase still not simple enough. A perfect control of hemostasis is fundamental because severe complications can arise from even a slight bleeding episode. It also is very important to search for and remove any accessory spleens. In our series this occurred in 7 patients, one of whom had 3 accessory spleens. The laparoscopic approach is today a good alternative to open splenectomy.

Adolescent↗

Follow-up of partial splenectomy in children with hereditary spherocytosis.

BACKGROUND/PURPOSE: The aim of this report is to study the short- and long-term consequences of partial splenectomy (PS) in hereditary spherocytosis (HS). METHODS: The authors reviewed the files of 5 children who underwent PS from 1993 to 1998. The data collected included clinical presentation, age, gender, indication for the operation, radiographic examination, need for blood transfusion and hematologic values, description of surgical procedure, and early- and late-occurring complications. RESULTS: From 1993 to 1998, 5 children from one year, 9 months to 7 years of age underwent PS. Indications were hypersplenism and severe anemia. The average age at the time of the operation was 3 years, 5 months. The 2 youngest children (one year, 9 months and 2 years old) needed a second operation because of the recurrence of hypersplenism. CONCLUSIONS: Subtotal splenectomy seems to preserve the immune role of the spleen and can reduce the need for blood transfusion. But PS is associated with a regrowth of the splenic remnant in children suffering from chronic hemolysis with hypersplenism and seems to be effective for a relatively short period only, especially in young children. Thus, a second operation to perform a total splenectomy can be necessary. The decision to perform a PS on young children with HS as an alternative to total splenectomy with appropriate preoperative vaccination and postoperative prophylactic antibiotics therefore should be weighed carefully, keeping in mind, however, the benefit of postponing total splenectomy in these patients.

Blood Transfusion↗

Splenectomy in chronic myeloid leukemia and myelofibrosis with myeloid metaplasia.

Myelofibrosis with myeloid metaplasia (MMM) is a collective term that describes the related disorders AMM, PPMM, and PTMM. The chronic myeloid disorders include chronic myeloid leukemia, polycythemia vera, essential thrombocythemia, and agnogenic myeloid metaplasia (myelofibrosis). These disorders display varying propensities for pathologic enlargement of the spleen which can lead to mechanical discomfort, hypercatabolic symptoms, anemia, thrombocytopenia, and portal hypertension. Splenectomy has been found to be of little benefit in the early stages of chronic myeloid leukemia. Similarly, the benefit of splenectomy in advanced cases is limited to symptomatic palliation and treatment of delayed engraftment after allogeneic bone marrow transplantation. Although polycythemia vera and essential thrombocythemia are also characterized by splenomegaly, splenectomy is not considered a therapeutic option in the absence of transformation of the disease into myelofibrosis with myeloid metaplasia. Splenectomy has been studied most in myelofibrosis with myeloid metaplasia. Although there is no clear survival advantage to splenectomy in this disorder, the surgical procedure can result in substantial palliation of mechanical discomfort, hypercatabolic symptoms, portal hypertension, and anemia. However, the procedure is associated with an approximately 9% mortality rate, and the postsplenectomy occurrence of extreme thrombocytosis, hepatomegaly, and leukemic transformation is of major concern.

Humans↗

Recovery by splenectomy in patients with relapsed thrombotic thrombocytopenic purpura and treatment failure to plasma exchange.

Thrombotic thrombocytopenic purpura is a serious, potentially fatal disease, and conventional plasma exchange appears to be the best initial therapy. Following this approach, survival in 90% of patients is available. In patients with relapse and treatment failure to plasma exchange, splenectomy is recommended. The rationale for splenectomy and the relevant pathomechanisms involved are obscure. In the present paper two patients with TTP are reported who first responded to conventional treatment strategies but later relapsed. Resumption of previous therapy was not able to continuously maintain normal platelet levels. Thus, splenectomy was considered to be indicated. In contrast to former reports, repeated cycles of conventional plasma exchanges were performed until a transient steady state (12 hrs) of the platelet counts occurred. Then splenectomy was performed immediately and, in contrast to former reports, no reinstitution of treatment was necessary after splenectomy. In addition no postoperative complications (bleeding, neurologic impairment) have been observed. This favorable outcome might be due to the strategy of repeated conventional plasma exchange procedures. The follow-up shows now event free disease for 2 years.

Adrenal Cortex Hormones↗

[Laparoscopic splenectomy for idiopathic thrombocytopenia].

BACKGROUND AND OBJECTIVE: Splenectomy is of great benefit for patients with idiopathic thrombocytopenia (ITP), when medical therapy has failed. It was the aim of this retrospective study to evaluate the results of laparoscopic splenectomy. PATIENTS AND METHODS: All patients with ITP, who had a laparoscopic splenectomy between 1992 and 1999, were included in this study. RESULTS: 53 patients with an average age of 41.8 years had a laparoscopic splenectomy: 48 of these operations were finished laparoscopically, while conversion to the open technique was necessary in five patients. The mean operation time was 95 minutes. In the last 38 cases it was 76 minutes. There were no deaths and no reoperations were necessary. The average postoperative hospital stay was 3.9 days. After a mean follow-up of 24 (1-75) months thrombocytopenia recurred in eight patients, of whom five received medical therapy. CONCLUSION: Splenectomy can be easily performed laparoscopically in patients with ITP and has a low morbidity. The results of therapy of ITP are good. Because of the quick convalescence the patients' comfort is better than with the conventional operation.

Adolescent↗

Laparoscopic splenectomy for haematological diseases: review of current concepts and opinions.

Laparoscopic splenectomy is now currently used by most surgeons in the treatment of many haematological diseases. The operative technique varies depending on the surgeon, but results are usually comparable among published series. We have reviewed 104 papers about laparoscopic splenectomy for haematological diseases and paid particular attention to surgical aspects and early postoperative results. We searched MEDLINE from January 1989 to April 1998, and of the 104 papers that we found 41 fulfilled our criteria of large series published in peer-reviewed journals that had been cited often. They usually compared laparoscopic and open splenectomy and focused on common problems (such as accessory spleens) and technical aspects (such as bleeding). Laparoscopic splenectomy is reported by most authors to be as safe and effective as open splenectomy for haematological diseases. It also has several advantages over the open approach, such as shorter and less complicated postoperative stay with better cosmetic results and more rapid return to full activities.

Hematologic Diseases↗

Effect of splenectomy on the susceptibility of mice inoculated with Diplococcus pneumoniae.

An experimental model is described which demonstrated increased susceptibility of mice to infection with D. pneumoniae following splenectomy. It was necessary to use small numbers of a particular strain of pneumococcus (D. pneumoniae type 6), intravenous infection and a particular strain of mouse (pathogen-free NCS strain). The increase in susceptibility persisted for at least 4 months after splenectomy. With modifications in experimental design such as use of large numbers of organisms, a different strain of pneumococcus, the intraperitoneal route of infection or a different mouse strain no increase or a much less impressive increase in susceptibility was demonstrated. Following intravenous injection of small numbers of D. pneumoniae Type 6 bacteremia tended to persist in all NCS mice. Multiplication of pneumococci subsequently occurred in a higher proportion of mice with splenectomy and at a more rapid rate than in control animals. Mice with splenectomy usually had more D. pneumoniae per ml of blood than per gram of any tissue. This suggested that in these mice multiplication of microorganisms occurs primarily in blood. In control mice higher concentrations of bacteria were present in spleen than in blood, and higher concentrations were found in blood than in other tissues. These results suggested that in normal mice infected intravenously with small numbers of D. pneumoniae Type 6, the spleen protects by removing and killing small but critical numbers of D. pneumoniae which are circulating in the blood. No evidence was found to suggest that the altered susceptibility is mediated by an effect of splenectomy on numbers of circulating leukocytes or on the antibacterial activity of mouse blood.

Animals↗

Laparoscopic splenectomy.

PURPOSE: To study the safety and efficacy of laparoscopic splenectomy (LS) in patients with hematologic disorders requiring surgical intervention. PATIENTS AND METHODS: A series of 103 consecutive adult patients underwent LS between 1992 and 1997 at our teaching hospital. Data were collected prospectively. The indications for splenectomy included idiopathic thrombocytopenic purpura (ITP), hereditary spherocytosis, autoimmune hemolytic anemia, and thrombotic thrombocytopenic purpura. RESULTS: The mean spleen size was 14 cm (range 8.5-24 cm) and the mean weight was 263 g (range 40-210 g). Accessory spleens were detected in 12 patients with ITP and 17 patients in the study overall. In 12 patients, LS was combined with a laparoscopic cholecystectomy for gallstones. There were four conversions to open splenectomy, all for hemorrhage and all occurred in the first 50 patients. We have not converted a single patient in the last 2 years. The mean operative time was 161 minutes and was greater in the first 10 cases than the last 10. There were no deaths. Postoperative complications occurred in six patients, one necessitating a second procedure for a small-bowel obstruction. The average length of stay in the hospital was 2.5 days. After surgery, thrombocytopenia resolved in 84% of patients with ITP and anemia resolved in 92% of the patients with hereditary spherocytosis. After a mean follow-up of 38 months (range 2-565 months), four patients (6%) showed a relapse of ITP, three within 12 months of surgery. CONCLUSIONS: Laparoscopic splenectomy can be performed safely and effectively in a teaching institution. LS in comparison with open surgery offers the same efficacy in the control of hematologic disease with the additional benefits of a minimally invasive approach. Laparoscopic splenectomy should therefore be considered the technique of choice and should prompt earlier consideration of surgery for patients with selected hematologic disorders.

Adult↗

Laparoscopic splenectomy for immune thrombocytopenic purpura.

Splenectomy is an effective treatment for immune thrombocytopenic purpura (ITP). The recent advances in laparoscopic technique and technology have made laparoscopic splenectomy a viable option. Over 36 months we performed a total of 17 laparoscopic splenectomies, 15 of them for ITP and 2 for familial spherocytosis. We present our initial experience with laparoscopic splenectomy in 15 patients (age 16-71 years) with ITP. Operations were performed 2-24 months after the establishment of the diagnosis and initiation of appropriate therapy. Technically, the splenic artery was clipped first; the lower pole of the spleen and its posterolateral attachments were dissected using endoclips and electrocautery; the hilum and short gastric vessels were separated using an endostapler; the spleen was placed in a plastic bag, its opening pulled out through the umbilical incision, and the spleen fragmented and aspirated out of the bag. Operations lasted 100-300 min (mean 170 min). No patient required blood transfusion. The postoperative course was uneventful in all patients with minimal requirement of analgesia and early return to normal activity. Platelet counts returned to normal in all patients in a follow-up period of 2-36 months. Laparoscopic splenectomy is safe and effective for patients with ITP because of reduced operative trauma, less postoperative pain, cosmetic advantage, and possibly less postoperative complications.

Adolescent↗

Splenectomy for non-Hodgkin's lymphoma.

Splenomegaly is a common occurrence in the course of non-Hodgkin's lymphoma (NHL), sometimes leading to development of bulk symptoms or cytopenias. Splenomegaly may also be the primary manifestation of NHL. We reviewed our experience with diagnostic and therapeutic splenectomy for NHL over the past 3 years. In July of 1991, a prospective database had been established to evaluate elective splenectomy for hematologic disease; of 58 patients, 12 had NHL. Splenectomy was performed for diagnostic purposes, correction of cytopenias, and relief of bulk symptoms. Most patients had more than one indication for splenectomy. Operative hemorrhage requiring transfusion was seen only in patients with massive splenomegaly (> 1,500 g). Median postoperative hospital stay was 4 days. There was no operative mortality or major morbidity. Minor morbidity was seen in 17% of patients. A favorable hematologic response was seen in 80% of cytopenias at the 3-month postoperative interval. Splenectomy is safe and effective in appropriately selected patients with NHL.

Adult↗

Incidental splenectomy associated with Nissen fundoplication.

Despite the relative frequency of iatrogenic splenectomy, its effect on postoperative morbidity and mortality remains controversial. This retrospective study compares the postoperative morbidity and mortality among 72 patients who underwent a Nissen fundoplication for esophageal reflux and 25 patients who had iatrogenic splenectomy and Nissen fundoplication. The age range in both groups was comparable. In the Nissen fundoplication (N.F.) group the average postoperative hospital stay was 9.4 days and in the Nissen fundoplication and splenectomy (N.F. + S.) group postoperative hospital stay was 15 days. In the N.F. group nine patients (12.5%) experienced significant postoperative morbidity in comparison to 36% in the N.F. + S. group (statistically significant p less than 0.1%). In the later group the complications were of a more severe nature. No mortality occurred in the N.F. group and one patient died in the N.F. + S. group. Iatrogenic splenectomy in this patient could well have been a prime factor in precipitating his demise. This retrospective study stongly supports the view that iatrogenic splenectomy in association with Nissen fundoplication adds considerably to postoperative morbidity.

Adult↗

The role of splenectomy in multimodality treatment of thrombotic thrombocytopenic purpura.

Current treatment modalities for thrombotic thrombocytopenic purpura (TTP) include plasmapheresis (PP), splenectomy, steroids, dextran, other antiplatelet agents, and vinca alkaloids. Prior to the development of PP and use of multimodality treatment for TTP, mortality rates exceeded 50%. This report reviews 11 patients treated for TTP, demonstrates the successful use of splenectomy as salvage therapy, and defines our indications for splenectomy in the treatment of this disorder. Ten of 11 patients were initially treated with PP; three responded completely and one died of fulminant disease. Six patients had a transient partial response to plasmapheresis and were subsequently treated with splenectomy, steroids, and dextran-70. Initial plasmapheresis resulted in improvement in laboratory values and clinical status in those patients requiring splenectomy. Durable remission (6-48 months) was achieved in 91% of patients with minimal morbidity.

Adult↗

Postoperative complications after splenectomy for hematologic malignancies.

OBJECTIVE: The authors analyzed the frequency and character of postoperative complications after splenectomy in patients with hematologic malignancies, and correlated these findings with preoperative conditions that could have predicted their outcome. SUMMARY BACKGROUND DATA: Splenectomy is performed for hematologic malignancies for diagnostic and therapeutic indications. The role of splenectomy for lymphoproliferative and myeloproliferative malignancies is complex and sometimes controversial. METHODS: The medical records of 135 patients undergoing splenectomies for hematologic malignancies at Roswell Park Cancer Institute from January 1, 1984 to December 31, 1993 were reviewed retrospectively. These included Hodgkin's disease (HD), hairy cell leukemia (HCL), non-Hodgkin's lymphoma (NHL), chronic lymphocytic leukemia (CLL), chronic myelogenous leukemia (CML), and a miscellaneous group. RESULTS: The overall postoperative complication and mortality rates for all patients were 52% and 9%, respectively. The complication rate was 63% for patients whose spleens weighed greater than 2000 g, and 29% for patients whose spleens weighed less than 2000 g (p = 0.001). Seventy-three percent of the postoperative deaths were due to septic complications, only one of which was caused by an encapsulated organism. Complications occurred in less than 20% of patients with the diagnosis of HD and HCL; more than 50% of patients with NHL, CLL, and CML suffered postoperative complications. CONCLUSIONS: Splenectomy performed in patients with hematologic malignancies is a potentially morbid procedure. Splenic size was the only preoperative factor found to be predictive of postoperative complications. The complication rate differed significantly between the different diagnostic subgroups.

Adolescent↗

The effect of splenectomy on antibody response to lipopolysaccharide (E. coli) immunization.

The influence of splenectomy on the antibody response to lipopolysaccharide (LPS: E. coli 0128:B12) was investigated in mice. Splenectomy had little effect on the primary response to the LPS. However, the level of IgG anti-LPS antibodies of splenectomized mice was significantly lower than that of sham-operated mice when the mice were immunized 1, 3, and 7 days after the operation and reimmunized 7 days after the first immunization. There was no significant difference in those immunized 30 days after the operation and reimmunized 7 days later. In mice immunized before splenectomy and reimmunized 30 days after splenectomy, the level of IgG anti-LPS antibodies was low, even in the mice splenectomized 30 days after primary immunization. Our results indicate that splenectomy impairs the antibody response to lipopolysaccharides.

Animals↗

Efficacy and safety of pneumococcal revaccination after splenectomy for trauma.

OBJECTIVE: To assess the outcome of patient education after splenectomy and vaccination and to determine the safety and efficacy of pneumococcal revaccination 2 or more years after primary vaccination. MAIN OUTCOME MEASURES: Titers to serotype no. 6 and no. 23 pneumococcus and cutaneous and systemic reaction to revaccination. RESULTS: A total of 112 consecutive postsplenectomy patients receiving pneumococcal vaccine were identified; 45 were contacted and offered revaccination; 24 patients demonstrated a lack of understanding of the postsplenectomy state (unaware of splenectomy n = 2, unaware of splenectomy risk n = 8, unaware of vaccine n = 23); 3 patients had infections requiring hospitalization (pneumonia, strep throat and tonsillitis, pneumonia and bacteremia); 40 patients agreed to revaccination, and 33 patients returned for follow-up titers; 16 of 33 (48%) demonstrated at least a two-fold increase in at least one titer. Only 15% described the revaccination as worse than a tetanus shot. CONCLUSIONS: (1) Despite physician-patient conversations, pamphlets, and Medic Alert bracelets, patient retention was poor. (2) All splenectomy patients should be revaccinated and reeducated between two and six years after splenectomy. (3) Revaccination after two years was well tolerated. (4) There were no fatal episodes of pneumococcal sepsis in over 200 patient years.

Adult↗

Prognostic factors for splenectomy response in adult idiopathic thrombocytopenic purpura.

The prognostic factors for splenectomy response were evaluated in 40 adult patients with idiopathic thrombocytopenic purpura (ITP). We defined the corticosteroid response on the basis of the platelet count after seven to ten days of therapy, rather than at some time after discontinuing corticosteroid agents, as previously reported in the literature. Initial corticosteroid response was statistically significant in predicting splenectomy response; age and sex were marginally significant; duration of symptoms and spleen weight were not predictive. Of 30 patients with a minor or complete response to corticosteroids, 28 (93%) responded to splenectomy; of ten patients unresponsive to corticosteroids only four (40%) responded to splenectomy (P less than .001). Eighty-two percent of the patients in the complete responder group are projected to be continually relapse-free at 96 months. Only one of the patients in this group died; the nonresponders to splenectomy had a median survival of only ten months.

Actuarial Analysis↗