Search

Myeloproliferative neoplasms can cause primary Budd-Chiari-Syndrome with acute or chronic liver failure necessitating liver transplantation. However, preventing the recurrence remains challenging and the need for post-transplant anticoagulant and cytoreductive treatment is not sufficiently clear. We analyzed the treatment regimens for all patients who presented to our department with PBCS from MPN between 2004 and 2021. Eight patients underwent liver transplantation - 6 of them due to an acute liver failure. Post-transplant, all patients received anticoagulant and 7 patients cytoreductive medication. The mean survival after transplantation was 13.25 years. Liver transplantation shows favorable long-term outcome when combined with post-transplant anticoagulant and cytoreductive treatment., Competing Interests: Declaration of competing interest The author Aysun Tekbaș and the coauthor Olga Morath receive fundings from the Interdisciplinary Center of Clinical Research of the Medical Faculty Jena, Germany., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) are characterized by excessive production of blood cells. Treatment of MPNs patients has an important effect thereby reducing morbidity and mortality., Objective: To evaluate the effect of cytoreductive treatment on some hematological and biochemical parameters in MPNs patients treated at a hemato-oncology Centre in Erbil, Iraq., Methods: A total of 185 patients diagnosed with PV, ET, and PMF (111 males and 74 females with a mean age of 50.8±3.2 years, range: 46-73) were assigned to receive MPNs treatment. Laboratory tests were performed before and after a median period from the initiation of MPNs treatment of 9.3 months (range 5-10 months)., Results: Significant differences were noted in Hemoglobin (P<0.003), Hematocrit (P<0.004), Neutrophil (P<0.001) and glutamate pyruvate transferase levels (P<0.01) in PV patients, Platelet count (P<0.002) in ET patients, and both white blood cell count (P<0.004) and Lactate dehydrogenase level (P<0.001) in PMF patients, while no significant differences were found in other parameters at the time of diagnosis and during therapy., Conclusion: Clinical and laboratory improvements were presented in MPNs patients. Regular follow up of patients are essential to ensure prescribed treatment in addition to the continual and long-lasting response to therapy and to prevent thrombosis., (© 2019 Salim et al.)

Purpose: Luminal gastrointestinal tract cancers (LGC) are common malignancies, and many patients can achieve long-term responses with surgery, cytotoxic and/or targeted therapies, and radiation. The long-term follow-up for patients with durable disease control has not been fully characterized, including subsequent malignancies. Such cases have not been comprehensively described., Patients and Methods: We identified patients evaluated for myeloid malignancies (MyM) who had a prior LGC at our institution over a 35-year period. Patient, disease, and treatment information was collected for analysis. Cytogenetic risk profiles were designated according to the Revised International Prognostic Scoring System for MDS and the European LeukemiaNet Guidelines for AML., Results: 66 patients were included in our cohort with 71 prior LGC diagnoses, including three patients with multiple LGCs. 31 cases were treated with surgery alone, and 37 patients received chemotherapy. The median age at diagnosis of MyM was 71.8 years (range, 36.2-90.5), with median duration between initiation of treatment of LGC and diagnosis MyM of 7.9 years (range 0.005-38.8). Intermediate or adverse (AML)/poor-very poor (MDS) cytogenetic risk was common, occurring in 43% of MDS patients and 100% of AML patients; deletion 5q was the most common cytogenetic abnormality overall. DNMT3A mutations were the most common molecular alteration (6 patients with 7 mutations)., Conclusions: Among patients with MyM following LGC, a high proportion harbored cytogenetic changes, many of which were adverse or poor-risk. Deletion 5q and mutated DNMT3A were the most common abnormalities identified., (Copyright © 2019. Published by Elsevier Ltd.)

BCR-ABL1-negative myeloproliferative neoplasms (MPNs) are clonal stem cell disorders defined by proliferation of one or more myeloid lineages, and carry an increased risk of vascular events and progression to myelofibrosis and leukemia. Portal hypertension (pHTN) occurs in 7-18% of MPN patients via both thrombotic and nonthrombotic mechanisms and portends a poor prognosis. Transjugular intrahepatic portosystemic shunt (TIPS) has been used in the management of MPN-associated pHTN; however, data on long-term outcomes of TIPS in this setting is limited and the optimal management of medically refractory MPN-associated pHTN is not known. In order to assess the efficacy and long-term outcomes of TIPS in MPN-associated pHTN, we performed a retrospective analysis of 29 MPN patients who underwent TIPS at three academic medical centers between 1997 and 2016. The majority of patients experienced complete clinical resolution of pHTN and its clinical sequelae following TIPS. One, two, three, and four-year overall survival post-TIPS was 96.4%, 92.3%, 84.6%, and 71.4%, respectively. However, despite therapeutic anticoagulation, in-stent thrombosis occurred in 31.0% of patients after TIPS, necessitating additional interventions. In conclusion, TIPS can be an effective intervention for MPN-associated pHTN regardless of etiology. However, TIPS thrombosis is a frequent complication in the MPN population and indefinite anticoagulation post-TIPS should be considered., (© 2017 Wiley Periodicals, Inc.)

Background: The germline telomerase reverse transcriptase (TERT) rs2736100&#95;C variant was identified as a susceptibility factor for a variety of solid tumors and recently for myeloproliferative neoplasms (MPN)., Methods: LightCycler melting curve analysis was applied to detect risk alleles of TERT rs2736100&#95;C and Janus kinase 2 (JAK2) rs12343867&#95;C tagging 46/1 haplotype in 584 BCR-ABL1-negative MPN, 308 acute, and 86 chronic myeloid leukemia (AML and CML) patients and 400 healthy individuals., Results: TERT rs2736100&#95;C showed an increased allele frequency in BCR-ABL1-negative MPN patients compared with controls (62.7%±2.8% vs. 48.8%±3.5%, P < 0.0001) regardless of molecular background or disease type, but not in CML or AML. Combined TERT and JAK2 hetero- or homozygosity conferred even higher risk for classic MPN. Common complications (thrombosis, myelofibrosis, or leukemia) were not associated with the TERT variant; however, adverse survival was noted in TERT variant carrier polycythemia vera patients. MPN patients with the TERT CC genotype had a higher probability (44.4%) to die from solid tumors compared with TERT AC/AA individuals (5.3%; P = 0.004). TERT rs2736100&#95;C carriers had increased risk of solid tumors independently from cytoreductive treatment [3.08 (1.03-9.26), P = 0.045]., Conclusions: TERT rs2736100&#95;C polymorphism predisposes to the development of BCR-ABL1-negative MPN with the co-occurrence of solid tumors, especially with the usage of cytoreductive treatment., Impact: The high frequency of TERT variant in the classic MPN population highlights the importance of the avoidance of long-term cytoreductive treatment in MPN patients., (©2015 American Association for Cancer Research.)

Background: A considerable proportion of patients receiving liver transplants for Budd-Chiari syndrome (BCS) suffer from myeloproliferative neoplasms (MPN). This study evaluated the long-term prognosis of liver-transplanted patients with BCS secondary to MPN and the effect of immunosuppression on MPN progression., Methods: A total of 78 patients with BCS were evaluated between 1982 and 2013. Of those, 40 patients suffered from polycythaemia vera (PV) and essential thrombocythaemia (ET). One patient had primary myelofibrosis (PMF). All patients received the standard immunosuppressive regimen. We retrospectively evaluated the long-term survival, clinical course and laboratory parameters of patients with MPN., Results: Exactly 29/41 patients (71%) with MPN survived ≥ 3 years [mean age 36 ± 11 years; females n = 27 (93%)]. Mean follow-up after orthotopic liver transplantation (OLT) was 12.4 ± 7.3 years (range 3-28 years). Five- and 10-year survival rates were not significantly different in patients with and without MPN (P = 0.81 and P = 0.66 respectively) or in patients with PV and ET (P = 0.29 and P = 0.55 respectively). Thrombosis and bleeding developed in 7/29 (24%) long-term MPN survivors with no significant difference between ET and PV (P = 0.18). In the long-term follow-up, there was no evidence of progression to overt myelofibrosis or acute myeloid leukaemia (AML). In the uni- and multivariate Cox-regression analyses, MPN did not influence survival after OLT., Conclusions: Budd-Chiari syndrome patients with and without underlying MPN had similar long-term survival rates after OLT. There was no evidence of enhanced progression of MPN after OLT secondary to immunosuppressive therapy. However, major haemorrhage and recurrent thrombosis contributed to morbidity and mortality after OLT in those patients., (© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Increased bone marrow hemangioblast numbers, alterations in erythroid/myeloid lineages, increased reticulin, and greater circulating bone marrow progenitor cells are present in patients with pulmonary arterial hypertension (PAH). The data suggest that myeloid progenitors contribute to the pathogenesis of PAH, but there are little data on the prevalence of pulmonary vascular disease among the different forms of myeloid diseases. We hypothesized that there would be a higher prevalence of pulmonary vascular disease in myeloproliferative neoplasms that have high circulating progenitor cells, such as myelofibrosis and chronic myelogenous leukemia (CML), compared with those with low circulating progenitors, such as in aplastic anemia., Methods: Patients with myelofibrosis, CML, and aplastic anemia who underwent echocardiographic evaluation of cardiac function in preparation for bone marrow transplantation at the Cleveland Clinic between 1997 and 2012 were identified and their electronic medical records were queried for demographic data, blood cell counts, and pulmonary function tests. All echocardiograms were uniformly analyzed in a blinded fashion by an advanced sonographer and cardiologist for measures of right and left ventricular function and estimation of pulmonary vascular disease., Results: Gender and race distribution among disease groups was similar. Patients with myelofibrosis (n = 19) and aplastic anemia (n = 30) had increased right ventricle (RV) wall thickness compared with CML (n = 82) patients (aplastic anemia, 0.7 ± 0.1; CML, 0.5 ± 0.1; and myelofibrosis, 0.7 ± 0.1; p = 0.02). Patients with myelofibrosis had higher levels of estimated RV systolic pressure compared with the other groups (aplastic anemia, 29.9 ± 1.5; CML, 26.2 ± 1.1; and myelofibrosis, 36.7 ± 3.7 mm Hg; p < 0.01)., Conclusions: The findings suggest an important role for myeloid progenitors in the maintenance of pulmonary-vascular health, in which abnormal myeloproliferative progenitors are associated with RV pathology., (Copyright © 2015 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curable approach for myelodysplastic syndrome (MDS) and myeloproliferative neoplasms (MPN); however, the event-free survival rate of patients with pediatric MDS and MPN is still only approximately 60%. Although salvage HSCT is the only curative approach for patients with the failure of previous HSCT, its safety and efficacy have yet to be determined., Procedures: We retrospectively analyzed 51 pediatric MDS or MPN who received salvage HSCT for relapse or graft failure following HSCT using registry data of the Japan Society for Hematopoietic Cell Transplantation. The indications used for salvage HSCT were relapse in 22 patients and graft failure in 29 patients., Results: The overall survival (OS) rate for salvage HSCT in relapsed patients was 49.0 ± 10.8% at 3 years. The cumulative incidence of relapse following salvage HSCT was 29.8 ± 10.7% at 3 years, whereas the incidence of non-relapse mortality (NRM) was 28.6 ± 10.2%. No significant differences were observed in the OS after salvage HSCT between disease types. Twenty-four of 29 patients who received salvage HSCT for graft failure achieved engraftment, resulting in an engraftment probability of 81.5 ± 8.0% on day 100. The OS rate after salvage HSCT for graft failure was 56.8 ± 9.6% at 3 years., Conclusions: Second HSCT should be considered as a valuable option for the patients with relapse and graft failure in patients with pediatric MDS or MPN after HSCT, but high NRM is an important issue that needs to be addressed., (© 2014 Wiley Periodicals, Inc.)

Systemic mastocytosis is a neoplastic proliferation of mast cells that frequently presents with associated clonal hematological non-mast cell lineage disease. Myeloid and lymphoid neoplasms with abnormalities of the FGFR1 gene are a heterogenous group of rare and aggressive hematopoietic stem cell disorders. About a dozen of chromosome changes involving the FGFR1 gene, presenting as myeloid or lymphoid neoplasms, have been described in the literature. To date, only 2 cases of myeloid and lymphoid neoplasms with abnormalities of the FGFR1 gene have been reported in association with systemic mastocytosis, one with t(8;13) and one with t(8;17) involving the FGFR1 gene. Here we describe another case of myeloproliferative neoplasm with chromosome translocation t(8;19) involving FGFR1 gene associated with systemic mastocytosis.

Splenectomy may be an effective therapeutic option for treating massive splenomegaly in patients with myeloproliferative neoplasms (MPNs). There are still limited data on its short- and long-term benefits and risks. Efficacy and short-term complications were analyzed in 94 patients with different MPNs who underwent splenectomy at M. D. Anderson Cancer Center. The long-term impact of splenectomy on overall survival (OS) and transformation free survival (TFS) was evaluated in 461 patients with myelofibrosis (MF) seen at M. D. Anderson, including 50 who underwent splenectomy during disease evolution. Splenectomy improved anemia and thrombocytopenia in 47% and 66% of patients, respectively. The most common complications were leukocytosis (76%), thrombocytosis (43%) and venous thromboembolism (16%). Post-operative mortality was 5%. Among patients with MF, splenectomy during disease evolution was associated with decreased OS (hazard ratio [HR] = 2.17, p < 0.0001) and TFS (HR = 2.17, p < 0.0001). This effect was independent of the Dynamic International Prognostic Scoring System. Splenectomy is a possible therapeutic option for patients with MF and other MPNs, and its greatest benefits are related to improvement in spleen pain and discomfort, anemia and thrombocytopenia. However, in patients with MF it appears to be associated with increased mortality.

Background: Splenectomy is a risk factor for both portal-vein and chronic thromboembolic pulmonary hypertension. The underlying mechanism is unclear, but may involve a hypercoagulable state., Methods: We describe 1 patient with polycythemia vera who developed extensive portal thrombosis of the portal, suprahepatic, and inferior cava veins, leading to right heart thromboembolism, with a resultant pulmonary embolism subsequent to splenectomy despite heparin prophylaxis., Results: In this patient, several mechanisms may have played a role, including perioperative stress, thrombocytosis, thrombophilia, and associated chronic liver disease. Nevertheless, combined treatment with intravenous heparin and thrombolysis and the myeloproliferative inhibitor hydroxyurea was associated with a favorable outcome., Conclusion: The risk of pulmonary thromboembolic complications and their management after splenectomies for hematologic disease warrant further study., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Special issues in myeloproliferative neoplasms (MPN) comprise clinical conditions with high relevance for the duration and quality of the patient's life, but with limited evidence to support sound diagnostic and therapeutic recommendations and a low probability of being solved by the current standard of clinical research. These issues include MPN in pregnancy and in children, abdominal vein thrombosis, bleeding complications, surgery, pruritus, and leukemic transformation. Practical suggestions to guide clinical decisions in these settings remain largely empirical, but recently developed guidelines based on experts' consensus may help to tackle these problems. This article reviews the state of the art regarding these issues, with special emphasis on experts' consensus recommendations.

Clinical care of patients with polycythemia vera, essential thrombocythemia and myelofibrosis (MF) requires not only a broad understanding of general treatment principles but also familiarity with the management of hydroxyurea-refractory disease complications. The latter include progressive splenomegaly, symptomatic portal hypertension (e.g. ascites, variceal bleeding), pulmonary hypertension, bone pain, intractable pruritus, constitutional symptoms (e.g. fatigue, night sweats) and cachexia (i.e. loss of lean body mass, general ill health, poor appetite). Some of these symptoms are directly or indirectly related to extramedullary hematopoiesis (EMH) and others to proinflammatory cytokine excess. Results from recent clinical trials of JAK inhibitors suggest remarkable activity in MF-associated constitutional symptoms, cachexia, pruritus and hydroxyurea-refractory splenomegaly. Involved-field radiotherapy is best utilized in the setting of EMH-associated symptoms, including ascites, bone (extremity) pain and pulmonary hypertension. Splenectomy is indicated in the presence of drug-refractory splenomegaly and frequent red cell transfusion requirement. Transjugular intrahepatic portosystemic shunt is used to alleviate symptoms of portal hypertension.

Myeloablative conditioning followed by T-cell depletion of grafts and reduced intensity conditioning (RIC) has both been shown to decrease treatment related mortality (TRM). However in RIC the incidence of graft vs. host disease (GvHD) is high and patients with aggressive diseases tend to relapse. Following myeloablative conditioning, patients with chemotherapy-responsive hematological malignancies underwent transplantation from HLA identical siblings. GvHD prophylaxis was by ex viva T-cell depletion with alemtuzumab. The outcome of these patients was analysed. At transplantation, the median age of 81 consecutive individuals was 45 years (range 15-60). GvHD was seen in 10% and was commonly associated with infections resulting in one and 3 year TRM of 15 and 20.5%. Fifteen patients relapsed, 10 who had myeloproliferative syndromes or lymphoma and two with myeloma responded to DLI. For the whole group, median follow up is 777 (range 7-2702) days and 73% remain disease free. Cox regression analysis for survival showed that only occurrence of GvHD was a significant adverse factor. Age order than median was not associated with worse outcome. By reducing the incidence and severity of GvHD, T-cell depletion of grafts leads to greater tolerance to myeloablative chemotherapy, resulting in acceptable TRM. This strategy should be compared with the RIC approaches.

This study aimed to evaluate the outcome following myeloablative allogeneic hematopoietic stem cell transplantation (SCT) among patients older than 50 yr of age. A total of 215 patients with a median age of 57 yr underwent allogeneic hematopoietic SCT for early (41%) or advanced (59%) hematologic malignancies. After a median follow-up of 36 months a 10-yr survival estimate of 56 +/- 6% could be assessed for patients in early disease stages while patients with advanced diseases showed a significantly decreased survival probability of 31 +/- 5% (p < 0.0002). Transplant related mortality (TRM) at day 100 and 365 post-transplant was 13% and 30% for early but increased to 21% and 49% for advanced disease stages. As major determinants of TRM advanced disease stage (p < 0.0001) and occurrence of grades II-IV graft-vs.-host disease (GVHD) (p < 0.0001) were identified. These results show that hematopoietic SCT following myeloablative conditioning is also applicable to elderly patients whereas disease stage and high-grade GVHD represent the essential prognostic factors for outcome.

Hemopoietic cell transplantation (HCT) is presently the only therapy with curative potential for myelodysplastic syndromes (MDS) and myeloproliferative disorders (MPD). Among patients with less advanced MDS, 3-year survival figures of 65-80% are achieved with human leukocyte antigen (HLA)-identical related and unrelated donors. The probability of relapse is less than 5%. Among patients with advanced MDS (> or = 5% marrow blasts), about 35-50% of patients transplanted from related donors, and 25-40% transplanted from unrelated donors are surviving in remission beyond 3 years. The incidence of post-transplant relapse is 10-35%. Criteria of the International Prognostic Scoring System (IPSS) predict relapse and survival following HCT. In patients with myelofibrosis, allogeneic transplantation is successful in 50-80%, if performed during the fibrosis stage. The success rate declines to 25-40%, if the transplant is performed after leukemic transformation has occurred. About 40% of patients with chronic myelomonocytic leukemia survive in remission after transplantation. Results obtained with low/reduced-intensity conditioning regimens are encouraging because of a low incidence of early mortality. However, retrospective analyses comparing low intensity and conventional conditioning regimens have yielded inconclusive results regarding long-term outcome. Co-morbid conditions present at the time of transplantation have a major negative effect on transplant outcome. Controlled prospective trials are needed.

Objective: To define indications and outcome in haematologic cases undergoing splenectomy., Study Design: A retrospective review of clinical records from consecutive patients having open or laparoscopic removal of the spleen in an academic centre in the private sector. Endpoints were survival, operating time, spleen size, histopathology, requirements for blood or related products complications and average costs., Results: In the total group (n = 69) there were two deaths. Referrals were for immune thrombocytopaenia (41%), acquired haemolytic anaemia (10%), myeloproliferative syndrome (9%), acute or chronic leukaemia (19%), lymphoma (13%) and a miscellaneous group (8%), comprising cholelithiasis, aplasia or as a diagnostic procedure for otherwise unexplained splenomegaly. An open midline approach was predicated by spleens greater than twice normal size and a history of any bleeding disorder. Here the mean operating time was 83 min (range 40-295) whereas for laparoscopy this was 251 min (range 181-272)., Summary: Careful stratification between the two options facilitated optimum haemostasis and consequently reduced requirement for packed red cells and platelets. Neither underlying pathology nor the choice of treatment influenced morbidity or mortality. Overall local experience is consistent with published international standards of surgical practice. Outcome is directly proportional to the number of each procedure carried out by a single team, observance of consistent protocols for preoperative evaluation and standardized proactive management through the recovery period.

According to the classification of the World Health Organization, the designation myelodysplastic/myeloproliferative disorder, unclassifiable may be applied to cases that have clinical, laboratory, and morphologic features that support a diagnosis of a myelodysplastic syndrome (MDS) as well as a myeloproliferative disorder (MPD), but that do not meet the criteria for any of the other entities included in the MDS/MPD category [3]. In this paper we report on two Caucasian patients with unclassifiable myelodysplastic syndromes with proliferative characteristics. Both patients were suffering from thrombocytopenia and splenomegaly and underwent splenectomy. The weight of the spleen specimens was more than 2000 g. Histopathology findings revealed a marked infiltration of the spleen with extramedullary hematopoiesis. After surgery, one patient showed a rapid increase of platelets in peripheral blood and developed severe thrombocytosis. In the other case, the patient was suffering from a decrease of platelets and died in hypovolemic shock caused by gastrointestinal bleeding. In summary, these two cases demonstrate the difficulties of prognosis and treatment in patients with mixed myelodysplastic/myeloproliferative disorders. Additionally, we indicate the potential positive outcome of splenectomy as ultima ratio in patients with these hematological features and severe thrombopenia.

Background: Laparoscopic splenectomy is emerging as the standard for treatment of benign splenic disorders. Since splenectomy is indicated relatively infrequently, issues arise concerning training of surgeons to perform laparoscopic splenectomy. Our experience with 50 laparoscopic splenic procedures is reported with emphasis on the learning curve at an academic institution., Materials and Methods: Data were prospectively collected on 50 consecutive patients undergoing attempted or successful laparoscopic surgical procedures on the spleen at Northwestern Memorial Hospital or The Chicago Health Care System, Lakeside Division, from April 1993 to April 1998, and on 5 patients undergoing open splenectomy from April 1993 to October 1995. Outcomes including conversion rate, operative time, day feedings were tolerated, and length of hospital stay was examined and correlated with the number of attempted cases., Results: Laparoscopic splenectomy progressed from an operation requiring two advanced laparoscopic surgeons to one performed by carefully supervised senior residents. Success rates increased from 60% initially to greater than 95% recently. Likewise, operative time decreased significantly from 195 to 97 min, while length of stay declined from 2.5 to 1.5 days. High success rates, low operative times, and short length of stays were achieved during the last 20 patients while surgical residents were taught to perform the procedures. The reasons for improvement are multifactorial including use of the harmonic scalpel, a change to the lateral position, and increasing experience with the procedure., Conclusions: Laparoscopic splenectomy is a safe and effective procedure that reduces postoperative length of hospital stay. It can be performed successfully in most patients with operative times comparable to those of open splenectomy. Moreover, the procedure can (and should) be taught to residents once they master basic and advanced laparoscopic skills., (Copyright 1999 Academic Press.)

There has been an increase in the indications for splenectomy for hematologic diseases. It is a consequence of expanding the list of disorders and liberalizing the indications for splenectomy for many diseases. Hereditary spherocytosis is the most frequently encountered congenital anemia for which splenectomy is curative. Splenectomy is generally advised for warm antibody acquired hemolytic anemia if conventional medical therapy fails. Idiopathic thrombocytopenic purpura (ITP) remains the most common hematologic disorder for which splenectomy is therapeutic. A variety of thrombocytopenias are improved by splenectomy. The myeloproliferative disorders constitute a spectrum of disease potentially improved by splenectomy, but preoperative management should be modified in this group. Splenectomy in patients with chronic leukemias and lymphomas are often palliative and facilitate chemotherapy. There are no specific requirements preoperatively other than the administration of pneumococcal and Hemophilus influenzae vaccines. Platelets are not administered to patients with ITP. An integral part of the procedure is the search for accessory spleens. The postoperative complication of overwhelming postsplenectomy sepsis must be considered; it occurs more frequently in association with specific basic diseases and can be obviated by appropriate treatment.

Objective: To assess the effect of certain risk factors on the outcome of elective splenectomy., Design: Retrospective cohort study., Setting: University hospital, Norway., Subjects: 135 patients who underwent elective splenectomy from 1978 to 1992 for autoimmune diseases (n = 58), myeloproliferative disorders (n = 58), and other diagnoses (n = 19)., Main Outcome Measures: Morbidity and mortality, and the effect on outcome of age, malignancy, platelet count, treatment with steroids, and blood transfusion during the operative period., Results: Three patients died, one from each diagnostic group; all had been treated with steroids. The overall complication rate was 31% (42/135). Most of the complications (31, 74%) were infective (pneumonia n = 23, abscess or sepsis n = 8). Nine patients required reoperation (haemorrhage, n = 6, and wound dehiscence, n = 2, and bowel obstruction, n = 1). Neither treatment with steroids nor severe thrombocytopaenia were risk factors for any complication, but age (p = 0.05) and a diagnosis of myeloproliferative disease (p = 0.08) had an important though not significant role. Blood transfusion during the operative period was the only independent risk factor (p = 0.001), and further analysis showed that transfusion of blood had a dose dependent effect on the incidence of infective complications (chi-square for linear trend 14.21 p < 0.001)., Conclusion: To reduce the complication rate of elective splenectomy blood transfusion during the operative period should be avoided.

A 15-year study of perioperative complications was carried out in 142 adults who underwent splenectomy for hematologic disease at the University of Alberta Hospital in order to obtain recent statistics on morbidity and mortality. The patients were grouped into four diagnostic categories: idiopathic thrombocytopenic purpura (71 patients), lymphoproliferative disorders (34 patients), myeloproliferative disorders (12 patients) and miscellaneous disorders (25 patients). Splenectomy was carried out for therapeutic reasons in 93% of patients and to establish a diagnosis in 7%. The overall complication rate was 22% (31 of 142) and the death rate was 6% (7 of 142). Infection accounted for 42% of the complications. Steroid or antibiotic therapy preoperatively did not significantly affect the infection rate. Drains, if removed within the first week, also did not affect the postoperative infection rate. Spleen size and the interaction between diagnosis and the presence of thrombocytopenia were predictors of the need for intraoperative transfusion.

The effect of splenectomy on the course of the chronic myeloproliferative syndromes was retrospectively analysed and compared in 96 patients who underwent this procedure and 195 who did not. The operation had a 4% mortality and a 47% morbidity rate, mainly attributable to haemorrhage, infection and respiratory complications. While splenectomy conferred benefit in certain selected cases with hypersplenism, there was no significant overall improvement in the postoperative haematological values and no influence on the rate of blastic transformation. Splenectomy did not improve survival in the group as a whole, or in any subset (P greater than 0.5). In the non-splenectomy group, only a minority of patients experienced massive progressive splenomegaly and in most individuals the spleen size varied little from the time of presentation to death. Splenectomy in patients with the chronic myeloproliferative syndrome carries a significant mortality and has a high morbidity, does not reduce the rate of blastic transformation and has no influence on survival. The majority of patients on conventional therapy do not experience the discomfort of massive splenomegaly during the course of their disease and the routine use of this operation is inappropriate.

Review of splenectomies carried out for haematological disease over a ten-year period, at a district hospital, shows that the indications for splenectomy have changed substantially over this time. Fewer patients with idiopathic thrombocytopenic purpura now require splenectomy, however its role in the management of lymphoproliferative disorders has expanded. Splenectomy remains an important therapeutic option for a range of haematological disorders: this series shows it to be a safe and effective operation in selected patients, although it is not without both short and long-term sequelae.

A patient with polycythemia rubra vera and myeloid metaplasia required splenectomy because of local symptoms attributable to a massively enlarged spleen. Preoperative embolization of the splenic circulation with inert materials was performed to reduce splenic size and vascularity in order to permit safe splenectomy. This case illustrates the utility of preoperative splenic embolization when routine splenectomy is contraindicated for technical reasons. The hazards of this technique and guidelines for its clinical use are outlined.

Factor XIII A subunit (FXIIIA) is found in plasma, platelets, and monocytes. The hemopoietic contributions to FXIIIA in these components were studied in patients transplanted with marrows from donors with different FXIIIA phenotypes. In three patients with successful engraftment (by DNA genotyping, red cell phenotyping, and cytogenetic studies) platelet and monocyte FXIIIA changed to donor phenotypes with hematologic recovery. Thus, FXIIIA in platelets and monocytes is synthesized de novo and/or from their progenitor cells. Plasma FXIIIA phenotype change after transplantation was more complex. Patient I changed from phenotype 1-1 (one electrophoretically fast band) to 1-2 (three bands) in 115 d; patients 2 and 3 did not change completely from phenotype 1-2 to 1-1 in up to 458 d, but did show enrichment of the fastest band. Thus, while there is a definite contribution of donor hemopoiesis to plasma FXIIIA, another source of recipient FXIIIA appears to be present to delay or prevent the phenotype change.

32P is effective therapy for polycythemia and primary thrombocytosis. The Polycythemia Vera Study Group is comparing radioactive phosphorus with alkylating agents to determine relative efficacy. Less well investigated is the effectiveness of 32P vs. busulfan in chronic granulocytic leukemia. Endolymphatic administration of radiopharmaceuticals may play a role in the therapy of infradiaphragmatic lymphoma. Among the radionuclides that have at times been used in hematology are 32P, 198Au 24Na, 76As, 89Sr, 52Mb, 54Mn, 91Y, 95Zr, 95Cb, 111Ag, 109Pd, 131I, 185W, and 192Ir. As stated, 32P has proven single most efficacious agent. The hematologic diseases that have been treated include both malignant and benign conditions. Among the malignant conditions are polycythemia vera, agnogenic myeloid metaplasia, thrombocythemia, leukemia, Hodgkin's disease, and multiple myeloma. Hemophilia, and Osler--Weber--Rendu disease are among the benign entities in which the agents have been tried. Polycythemia and thrombocythemia remain those in which the greatest success has been achieved.

A 16 year review of 391 splenectomies performed at New England Medical Center was done to evaluate the morbidity and mortality of patients with drained splenic weights greater than 1,000 grams. Thirty-six met the criteria for study. Twenty men and 16 women with an average age of 55.4 years were identified. Myeloproliferative disorders were the most predominant cause of massive splenomegaly. Pancytopenia and hemolytic complications of the disease processes were the most acute indications for operations. The average time between diagnosis and operative intervention was 42 months. An average of 10 units of blood products were required to correct preoperative coagulopathy. Eleven of 36 patients had postoperative complications. Eight of 21 with drains and an equal number of patients with preliminary splenic arterial ligation had complications. Eight-one per cent of all complications were infection related. Complication increased the length of stay 11 days. The 30 day mortality rate was 11.1 per cent. Sepsis was the major cause of mortality. Closed drainage system provided no demonstrable benefit nor appeared to be the cause of sepsis. No episodes of pulmonary embolic phenomenon or peripheral venous thrombosis were demonstrated. Elective splenectomy in patients with smaller spleens was performed without operative mortality and with 3 per cent morbidity rate. For patients with massive splenomegaly, the average survival time was 28.5 months. The majority of these patients died from complications of the disease. Preoperative coagulopathy, failure to demonstrate a hematologic response to splenectomy and reoperation were clear predictors for decreased long term survival periods.(ABSTRACT TRUNCATED AT 250 WORDS)

During the past two years, postoperative thromboses of parts or all of the splanchnic venous circulation have developed in four of 30 patients with myeloproliferative disorders who have undergone splenectomy at our institution. The patients' courses were marked by platelet abnormalities, both quantitative and qualitative. In an attempt to avoid this complication, aspirin and low-dose heparin sodium have been administered and there has been no evidence of thrombosis in the four patients so managed.