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[Splenectomy in idiopathic thrombocytopenic purpura: short- and long-term results (author's transl)].

The results of splenectomy in 25 patients with chronic idiopathic thrombocytopenic purpura (ITP) are reported. Splenectomy was performed when the platelet count was consistently less than 30,000/mm3 in spite of glucocorticoid therapy over an observation period of at least six months. Following splenectomy, 13 patients showed complete remission, 9 partial remission, whilst in 3 cases the condition was unaffected by splenectomy. It is not possible to predict a successful response to splenectomy on the basis of preoperative laboratory findings. A rise in thrombocyte count to over 400,000/mm3 during the first 2 weeks after splenectomy makes complete remission very likely.

Adolescent

Clinical course and survival after elective splenectomy in 19 patients with primary myelofibrosis.

Nineteen patients with primary myelofibrosis and myeloid metaplasia (MMM), who fulfilled well-defined criteria, underwent elective splenectomy as soon as the diagnosis was confirmed. Nine patients developed postoperative complications such as intra-abdominal bleeding (three), rupture of the inferior vena cava (one), ascites (two), Australia antigen-positive hepatitis (three), mesenteric artery thrombosis (one) and pneumonia (one). One patient died within a month of the operation due to secondary intra-abdominal infection. The mean age of the patients at splenectomy was 56 years and the mean duration of their disease 2.4 years. The median actuarial survival after operation was 51 months. Although the series of patients is small, it seems that splenectomy did not have an adverse effect on life expectancy. The haematological status and the quality of life improved after splenectomy in 17 of 19 patients. The results warrant a further trial with elective splenectomy in an early stage of MMM.

Adult

Planned splenectomy in treatment of idiopathic thrombocytopenic purpura.

The results of a policy of treatment in idiopathic thrombocytopenic purpura based on previous observations on the natural history of the disease and its response to corticosteroids are described. The results of splenectomy were better when the history was less than 100 days. Three patterns of response to splenectomy were observed: complete remission, symptomatic remission, and relapse. The prognosis can be determined by the level of the platelet count six weeks after splenectomy. Corticosteroid treatment for more than three weeks before splenectomy noticeably increased the incidence of complications after operation. Splenectomy can safely be performed in pregnancy. The decision to operate should be made on the maternal condition and its response to corticosteroids.

Adolescent

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

A reappraisal of the role of splenectomy in children receiving renal allografts.

Four renal allograft recipients between 8 and 14 years of age died of acute fulminating infections less than 72 hours after the onset of symptoms. These patients all had undergone splenectomy at the time of related donor renal transplantation. Because the therapeutic value of splenectomy in renal transplantation has not been established, the role of adjunctive splenectomy is evaluated, and the relationship between splenectomy and overwhelming infection is discussed. As a result of these acute deaths in 4 of 62 children undergoing renal transplantation at this institution (6.4%), splenectomy in conjunction with renal transplantation in children has been discontinued.

Acute Disease

Effect of splenectomy alone and in combination with antilymphocyte serum on renal allograft rejection in the rat.

The effect of splenectomy alone and in combination with suboptimal doses of antilymphocyte serum (ALS) have been studied in the relatively weak (DA X Lewis)F1 to DA and the strong DA to Lewis renal allograft models. In the F1 to DA model, splenectomy alone produced a slight but significant prolongation of graft survival and in combination with a suboptimal dose of ALS gave excellent suppression of rejection. In the strong DA to Lewis model, splenectomy alone had no effect on graft survival and there were no additive effects with suboptimal doses of ALS. However, splenectomy alone produced a striking suppression of the lymphocytotoxic response to the kidney grafts in this combination. To elucidate the mechansim whereby splenectomy weakens the immune response, (DA X Lewis)F1 to DNA renal allografts were performed with the renal vein anastomosed to the recipient's portal vein. This procedure had no effect on graft survival.

Animals

Effect of splenectomy on the immune response of BALB/c mice bearing an immunoglobulin M plasmacytoma (TEPC-183).

Mice bearing TEPC-183, an immunoglobulin M(kappa)-secreting plasmacytoma, exhibit severe suppression of their immune responses to both thymus-dependent and thymus-independent antigens, 2,4-dinitrophenyl, and the type 3 pneumococcal polysaccharide SSS-III. This immunosuppression is not lifted by splenectomy of the tumor-bearing mice or prevented by removal of the spleen prior to tumor injection. On the contrary, splenectomy either before or after tumor implantation further accentuates the immunosuppressed state of tumor bearers and even depresses the immune response of normal mice. A secondary immune response of normal mice 34 to 51 days after splenectomy is still reduced. Thus, spleen cells may play a dual role. While splenectomy may remove a source of suppressor cells in tumor-bearing mice, it also eliminates a major source of antibody-producing cells and results in reduced immune responses of normal and TEPC-183-bearing mice. These findings have clinical relevance since splenectomy is used as a therapeutic and diagnostic procedure in neoplastic lymphoproliferative disorders.

Animals

Diagnostic and therapeutic aspects of splenectomy in syndromes associated with hypersplenia.

The value of splenectomy is assessed from diagnostic and therapeutic viewpoints in a series of 80 patients with various syndromes marked by hypersplenia. In the congestive type of splenomegaly, splenectomy resulted in complete normalization of the blood picture in all cases but one, and in primary splenic congestion it even proved curative in the majority of the cases. In leukaemia, non-Hodgkin's lymphomas, in myelofibrosis, and first of all in immuncytopenia, splenectomy was also of benefit, and had generally a palliative effect in non-autoimmune hypersplenia as well. In non-haematological syndromes associated with hypersplenia, namely, splenic tuberculosis, Boeck's sarcoid, SLE, haemorchromatosis and splenic vein thrombosis, splenectomy had generally a palliative, and combined with other therapeutic measures, a curative effect, depending on the primary disease. In a number of patients with hypersplenia associated with splenomegaly, it was only with the aid of splenectomy that the primary disease could be diagnosed.

Diagnosis, Differential

Elective splenectomy in the elderly patient.

This report reviews 10 years' experience with elective splenectomy in patients over 55 years of age. Of 55 patients, 25 were over the age of 65. The majority of splenectomies (41) were performed for hematological disorders exclusive of lymphoma, but including ITP, hypersplenism (both primary and secondary), Felty's syndrome and myeloid metaplasia. Sixty-eight per cent of these patients responded to splenectomy with sustained elevation of platelet counts while 70 per cent responded to sustained elevation of leukocyte counts. Sixteen of the hematological patients received a preoperative trial of steroid therapy in an attempt to alleviate leukopenia or thrombocytopenia with a 37.5 per cent (6 of 16) success rate. There was no correlation between the response to preoperative steroid therapy and response to splenectomy. These data correspond favorably to previous published material in the younger aged patients. There was no difference in the postoperative complication rate related to drainage or nondrainage of the splenic bed. However, eight other complications occurred for a morbidity rate of 14.5 per cent in addition to complications related to drainage of the splenic bed (8%). A 9 per cent (5 of 55) mortality rate was related to the patients' pre-existing diseases and appears acceptable in light of hematological response rate and quoted mortality rates for splenectomy over age 65.

Age Factors

Splenectomy for hematologic disease.

One hundred and thirty splenectomies were performed at the University Hospital in Birmingham for hematologic disorders during a 12 year period. There were seven deaths of which four were related to sepsis in patients with malignant growths which probably impaired immune competence. Splenectomy was found to be uniformly satisfactory for hereditary hemolytic anemia, for hypersplenism complicating rheumatoid arthritis and for the rare instance of primary hypersplenism. Splenectomy was usually salutary in didopathic thrombocytopenic purpura and in antoimmune hemolytic anemia. When done for diagnosis, splenectomy was definitive in about 50 per cent of the instances. Individual patients with thrombotic thrombocyeosinophilia syndrome benefited. Splenectomy for hypoplastic or aplastic anemia in an actual circulation offered only questionable benefit and is rarely necessary for hyperplenism secondary to portal hypertension.

Adult

Immune thrombocytolytic purpura (ITP): a diagnostic and therapeutic survey of 86 cases with regard to the results of splenectomy and conservative therapy.

The therapeutic results in 86 cases of ITP seen over a period of 15 years are reported. Distribution of the haemorrhagic manifestations was in agreement with published data. On follow-up of the 40 splenectomized patients, the shortened platelet life-span, the reduced "recovery" as well as the increased rates of platelet turnover and platelet formation revealed a manifest tendency of normalization. One year after splenectomy the results were found "good" or "moderate" in 86%, more than two years after the intervention in 85%, of the cases. Retrospective analysis of the platelet-sequestration studies having shown "good" or satisfactory results in 93% of the splenic cases, this investigation is regarded as crucial to the indication of splenectomy. The absence of increased thrombocytopoiesis in approximately 15% of the cases is attributed to an antibody effect, reflected in a reduced polyploidy of the megakaryocytes. Normalization or increase in platelet count early after splenectomy is interpreted as a favourable prognostic sign. On the other hand, neither age of the patients nor the time of splenectomy was found to affect the success of the intervention. The results of corticosteroid treatment (Prednisolon 52%) lag far behind those of splenectomy. Ten patients were given combined immunosuppressive treatment.

Adolescent

Splenectomy, chemotherapy, and survival in Hodgkin disease.

The question was raised whether routine splenectomy might, by virtue of its effects on the receipt of subsequent chemotherapy, offer long-term benefits to patients with advanced Hodgkin disease. Therefore, we compared followup data from a group of patients who were receiving mechlorethamine hydrochloride, vincristine sulfate, procarbazine hydrochloride, and prednisone (MOPP regimen) and who had had splenectomies to a group of similarly treated, carefully matched control patients on the MOPP regimen who had not had splenectomies. Our results indicate no important difference in duration of survival or long-term remission status. There was only a suggestion that splenectomy in such patients may be followed by impaired resistance to subsequent infections. Therefore, although early splenectomy can be important in the assessment of splenic disease, it must not be considered to be a therapeutic procedure per se.

Dactinomycin

The influence of splenectomy on the invitro lymphocyte response to phytohemagglutinin and pokeweed mitogen in Hodgkin's disease.

Using tissue culture techniques, the 14C-thymidine incorporation of peripheral lymphocytes in 17 Hodgkin's patients was tested before and after splenectomy under stimulation with phytohemagglutinin and pokeweed. Incorporation under phytohemagglutinin stimulation about 10 days after splenectomy was not affected in Hodgkin's patients with pathologic Stages I and II, but was significantly (p less than 0.005) increased in those with Stages III and IV. The total PHA stimulation potency, i.e. the product of the lymphocyte count and PHA stimulation, increased slightly in both groups. Incorporation under pokeweed stimulation after splenectomy did not significantly differ from that before the operation in the two groups. Although the number of cases studied is rather small, it is concluded that splenectomy causes no demonstrable untoward effect on the cellular immunologic potency. The immunologic state is more likely to be favorably influenced than unfavorably.

Adolescent

Mesenteric venous thrombosis occurring late after splenectomy.

Two patients with mesenteric venous thrombosis which occurred late after splenectomy are reported. In the first case the thrombosis was seen, with preceding thrombocytosis, 3 years after splenectomy. In the second case the thrombosis occurred 2 years and 5 months postoperatively. Platelet counts had not been obtained before the mesenteric thrombosis in this case. In both instances persistent severe anaemia for several weeks preceded the symptoms and signs of mesenteric venous thrombosis. Both patients were treated by extended resection of the affected small bowel. Thus, dangerous thrombocytosis may occur in the presence of persistent severe anaemia even late after splenectomy. Thrombocytosis occurring immediately after splenectomy, which is almost inevitable, should be treated with heparin or other anticoagulants. In the late period the avoidance of persistent severe anaemia seems to be important in the prevention of thrombotic complications due to reactive thrombocytosis in splenectomized patients.

Adult

Platelet aggregability after splenectomy in patients with normosplenism and hypersplenism.

The relationship between platelet count and aggregability was serially evaluated after splenectomy in patients with normosplenism and hypersplenism, and the results were compared with those found in patients undergoing only upper abdominal surgery. The preoperative count and aggregability of platelets were significantly suppressed in patients with cirrhosis of the liver, idiopathic portal hypertension, and prehepatic portal obstruction. However, the platelet aggregability of these patients markedly increased in accordance with the platelet counts after splenectomy. In the patients with normal splenic function preoperatively, splenectomy caused a simultaneous increase in platelet count and aggregability. The platelet function was not always reflected by the count in the patients with idiopathic thrombocytopenic purpura. On the other hand, in the control patients undergoing only laparatomy the platelet count substantially increased two weeks after operation, but platelet aggregability did not differ from the preoperative value. The present results suggest that additional factors for thromboembolism such as hypotension, acidosis, or stagnant blood flow should be avoided during the peak period of reactive thrombocytosis after splenectomy, and that an appropriate use of anticoagulants or inhibitors of platelet aggregation is recommended if and when necessary.

Adenosine Diphosphate

Splenectomy for complications of chronic granulocytic leukaemia.

13 patients with chronic granulocytic leukaemia (C.G.L.) which was unsatisfactorily controlled underwent splenectomy. 3 out of 4 patients with hypersplenism did well, as did 5 out of 8 patients in whom the C.G.L. had undergone metamorphosis to a refractory phase. In a case of C.G.L. complicated by severe myelofibrosis the need for transfusion was reduced but survival was short. Splenectomy should be considered when C.G.L. in its chronic phase is complicated by hypersplenism, and may be considered as a part of the treatment after C.G.L. has undergone metamorphosis to a refractory phase. However, a favourable outcome is unlikely for patients over 65 years, and in the presence of coexistent illnesses, rapidly progressive metamorphosis to an acute phase, or severe bone-marrow failure from any cause. In C.G.L., elective splenectomy early in the chronic phase must be clearly distinguished from splenectomy performed at a later stage when the disease is not well controlled.

Adolescent

Splenectomy for haematological cytopenias in patients with malignant lymphomas.

Haematological cytopenias caused by increased splenic activity occurred in 86 patients with malignant lymphomas (50 patients with stage III or IV Hodgkin disease [H.D.] and 36 with non-Hodgkin lymphomas). Splenectomy was performed in 84 of these 86 patients. There was complete or partial correction of the haematological defect in 90% of the H.D. patients and 89% of the non-Hodgkin patients. Chemotherapy or radiotherapy was started after the haematological defect had been corrected by splenectomy, and as a result 79% of the H.D. patients and 75% of the non-Hodgkin patients had a complete or partial remission of their disease. Increased splenic activity should be considered as a cause of a haematological cytopenia in patients with malignant lymphomas even in those with bone-marrow involvement, since splenectomy is accompanied by excellent results in terms of disease remission with subsequent therapy. 2 patients with H.D. (4%) and 5 with non-Hodgkin lymphomas (14%) died in hospital after splenectomy. Thus the operation was associated with acceptable mortality and morbidity despite the advanced stage of disease in most cases.

Anemia

The born-again spleen. Return of splenic function after splenectomy for trauma.

We assessed splenic activity after splenectomy by interference phase microscopical examination of circulating red cells. Normal eusplenic children had a low number (less than 1 per cent) of red cells with surface indentations or "pits". About 20 per cent of red cells of children who had electively been subjected to splenectomy for hematologic indications were "pitted". Thriteen of 22 children who had had emergency splenectomy because of traumatic injury had a low percentage of "pitted" red cells, suggesting a return of splenic function. In five of these children a 99mTc sulfur colloid scan demonstrated multiple nodules of recurrent splenic tissue. In contrast to the prevailing opinion that splenosis is rare, we have found it to be a frequent occurrence. Return of splenic function may, in part, account for the low frequency with which overwhelming bacterial sepsis and meningitis have been documented after splenectomy for traumatic indications.

Adolescent