Late postsplenectomy recurrence of thrombotic thrombocytopenic purpura responding to removal of accessory spleen

The present report describes a patient who experienced recurrence of thrombotic thrombocytopenic purpura 10 years after the initial episode. The patient had been successfully treated with steroids and splenectomy and had complete clinical and hematologic remission. Thrombotic thrombocytopenic purpura recurred 10 years later and did not respond to steroids and plasmapheresis. The presence of an accessory spleen was demonstrated by technetium scanning. Surgical removal of the accessory spleen resulted again in prompt and complete recovery.     

Phagocytosis of blood cells by splenic macrophages in thrombotic thrombocytopenic purpura.

A 43-year-old woman who developed thrombotic thrombocytopenic purpura was treated successfully with high doses of prednisone and a splenectomy. Imprints from the cut surface of the spleen showed marked phagocytosis of all the cellular elements of the blood. This observation suggests that hypersplenism, in addition to vascular occlusion, may play a major role in the physiopathology of some cases of thrombotic thrombocytopenic purpura.     

Thrombotic thrombocytopenic purpura—Remission following splenectomy: Report of a case and review of the literature

A case of thrombotic thrombocytopenic purpura in which sustained remission followed splenectomy is described. Strong serologic evidence of concurrent infection with Mycoplasma pneumoniae was found in this case. Previously reported cases in which thrombotic thrombocytopenic purpura has been associated with infections are reviewed. An approach to management of this unusual and frequently fatal condition, is suggested, based on a detailed review of the recent literature.     

Fate of therapy failures in adult idiopathic thrombocytopenic purpura

Of 38 adult patients with idiopathic thrombocytopenic purpura followed an average of more than 12 years, 15 suffered splenectomy failure or postsplenectomy recurrence of thrombocytopenia. Nine of the 15 also received immunosuppressive agents, and four of the nine failed such therapy. In eight of these 15 treatment failures normal or safe platelet counts were achieved in a subsequent three to 12 year period during which they received no therapy. The frequency of spontaneous recovery of satisfactory platelet levels in adults with idiopathic thrombocytopenic purpura in whom treatment failed may have negative implications for very vigorous or longstanding immunosuppressive therapeutic attempts in certain cases.     

Management of treatment failures in thrombotic thrombocytopenic purpura

The prognosis and optimal management of patients with thrombotic thrombocytopenic purpura (TTP) who fail initial therapy with plasmapheresis or splenectomy are unclear. We report our experience with eight patients with TTP who did not respond to initial therapy. Seven patients achieved complete remission when alternate therapy was started soon after the recognition of initial treatment failure. One patient who received no alternative therapy died of progressive TTP. Our cases combined with those in the literature indicate a 74% salvage rate for patients who fail initial treatment for TTP. The combination of splenectomy, dextran, and steroids appears to be an effective treatment for patients with TTP who fail to respond adequately to plasmapheresis.     

Thrombotic thrombocytopenic purpura associated with chronic HCV infection

BackgroundThrombotic thrombocytopenic purpura is a potentially lethal microvascular thrombotic disorder.Case presentationIn this study, we report a 32 years old woman who suffered from undifferentiated vasculitis with marked improvement on steroids and cyclophosphamide. Two years later, hepatitis C virus infection was discovered. Decision for interferon therapy was not recommended at this stage and the patient remained stable for the following 7 years. In January 2009, pegylated interferon and ribavirin were started due to worsening of her hepatitis; the treatment was stopped after 12 weeks due to the absence of any virologic response. Fourteen months later, she developed severe uncontrolled thrombotic thrombocytopenic purpura that led eventually to her death.ConclusionWe report this rare case of thrombotic thrombocytopenic purpura that may directly be related to chronic HCV infection rather than to interferon therapy.     

Thrombotic thrombocytopenic purpura: recovery after splenectomy associated with persistence of abnormally large von Willebrand factor multimers

A patient with thrombotic thrombocytopenic purpura (TTP) responded transiently to four courses of plasma exchange therapy, then subsequently had a sustained completed remission following splenectomy. The von Willebrand factor multimeric pattern during remission following each plasma exchange and during the entire postsplenectomy period showed abnormally large forms that were not present on presentation and with each clinical relapse. These findings support prior observations regarding the presence of abnormal von Willebrand factor multimers in relapsing TTP and suggest that the multimers contribute to platelet aggregation and the thrombotic lesions. The association of sustained remission and persistence of the abnormally large plasma multimers after splenectomy suggests that this response was not coincidental. This supports the concept that a subgroup of patients with TTP may exist in which the pathophysiology of disease is significantly modulated by the spleen and in which splenectomy may induce long-lasting remission.     

Response to plasma exchange and splenectomy in thrombotic thrombocytopenic purpura. A 10-year experience at a single institution.

BACKGROUND--This study was designed to assess the response of patients with thrombotic thrombocytopenic purpura to plasma exchange and to evaluate the role of splenectomy after relapse. METHODS--The records of all patients with thrombotic thrombocytopenic purpura who had plasma exchange as primary treatment at a single center during a 10-year period were retrospectively reviewed. Response to the initial course of plasma exchange was determined, and the clinical outcome was evaluated in patients whose conditions were either refractory to exchange, responded without relapse, or relapsed after initial response. The outcome of patients treated during relapse with splenectomy was evaluated. A literature review was conducted to determine the clinical outcome in patients treated similarly. RESULTS--Twenty-seven patients for whom data could be evaluated had been treated in the 10-year period. Twenty-one (78%) responded to the plasma exchange, but the conditions of six (22%) were refractory and these patients died. Eight patients (30%) had one or multiple relapses after initial response but had prolonged remissions after additional plasma exchange alone (two patients) or splenectomy (six patients). A review of 19 reports, including 224 patients with thrombotic thrombocytopenic purpura initially treated with plasma exchange, revealed similar findings, with initial response in 81%, refractoriness in 19%, and relapse after initial response in 27% of patients. CONCLUSION--Response to plasma exchange in thrombotic thrombocytopenic purpura is associated with an excellent prognosis, and most deaths occur in patients whose conditions are refractory. Relapses after initial response are frequent but can be managed successfully with additional plasma exchange or with splenectomy, which often induces long-term remissions.     

Twice daily plasma exchange in refractory thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) usually responds to daily single total plasma exchange (TPE) or to plasma infusion. However, some cases are refractory to total plasma exchange, and additional efficacious treatment may not be available. A 34-year-old white male diagnosed with TTP was found to be refractory to single TPE. Steroids and twice daily TPE, in addition to splenectomy and vincristine, worked well to prevent further clinical deterioration. Laboratory values were normalized at the completion of treatment protocol. Cases of TTP refractory to single TPE/day may benefit from early treatment of twice-daily TPE in addition to a combination therapy of steroids, splenectomy, and vincristine.     

Thrombotic thrombocytopenic purpura: Recovery after plasmapheresis,corticosteroids, splenectomy,and antiplatelet agents

A 68-year-old woman severely ill with thrombotic thrombocytopenic purpura (TTP) made a complete recovery after splenectomy was added to a program of corticosteroids, antiplatelet agents, and plasmapheresis. The authors review the subject of therapy of TTP. They advise high-dose corticosteroids, antiplatelet agents, and plasmapheresis, with splenectomy to be considered as an effective alternative.     

Treatment of Thrombotic Thrombocytopenic Purpura

Abstract. This report examines the results of treatment in 21 patients with thrombotic thrombocytopenic purpura (TTP) diagnosed over a 4-year time period (1976–1980) with a review of the possible role of each form of therapy in relationship to the various proposed pathogenic mechanisms. There was a 76.2% (16/21) overall survival with no significant difference in initial hematologic values in patients not surviving. Patients not surviving did not achieve a sustained platelet count greater than 150,000/μ1 at any time during their clinical course. 16 patients received steroids, antiplatelet agents and plasma exchange, with a total volume exchanged ranging from 20.8 to 1,455 ml/kg, as part of their treatment protocol. In this group of patients there was an 81.2% (13/16) survival, with 4 patients receiving additional therapy including splenectomy and/or vincristine. There was no correlation between the intensity of plasma exchange and the time to hematologic recovery. It is apparent that controlled clinical trials are necessary to better define the effectiveness of the present forms of therapy.     

Plasma exchanges as treatment of severe acute immune thrombocytopenic purpura

INTRODUCTION: High dose steroids and intravenous immunoglobulins are the gold treatment of acute immune thrombocytopenic purpura, before splenectomy for severe and refractory forms of the disease. Authors report two cases of severe acute refractory immune thombocytopenia with a dramatic response to plasma exchanges. EXEGESIS: The first case was an idiopathic form, complicated by hemorragic peritoneal effusion. After failure of steroids, intravenous immunoglobulins and splenectomy and 2 courses of rituximab, plasmapheresis normalized in 3 days platelet count. In the second observation, ITP was associated to systemic lupus with antiphospholipids antibodies and multivisceral failure, despite steroids and intravenous immunoglobulins. After 3 plasma exchanges, platelet count was normalized, and the patient is under remission after 24 months follow-up. CONCLUSION: Plasmapheresis must be evaluated as an emergency treatment in refractory forms of acute immune thrombocytopenic purpura.     

High incidence of relapses in thrombotic thrombocytopenic purpura. Clinical study of 38 patients

Described in this study are 38 patients who received treatment for thrombotic thrombocytopenic purpura in 15 hospitals in Israel and the New York City area since 1977, when plasma therapy was introduced. Thirty-seven patients received plasma therapy and 30 survived. In 12 patients (37 percent of survivors), relapsing thrombotic thrombocytopenic purpura developed, manifested by thrombocytopenia and microangiopathic hemolytic anemia, and less frequently by neurological or renal abnormalities. Six patients had a second relapse, and two had five relapses. To assess the severity of the disease, a scoring system was designed based on the four major manifestations of thrombotic thrombocytopenic purpura listed above. The patients who died had a significantly higher score than those who survived. The initial episodes and the relapses of patients with relapsing thrombotic thrombocytopenic purpura were milder than those in patients who only had a single episode and survived. Two relapses, however, were fatal, demonstrating that relapsing thrombotic thrombocytopenic purpura is not a benign disorder. Infections, pregnancy and surgery were frequently associated with the initial episodes and the relapses. Hence, patients who recover from thrombotic thrombocytopenic purpura should be alerted to the possibility of relapse in association with these conditions.     

Refractory thrombotic thrombocytopenic purpura in a lupus nephritis patient

Thrombotic thrombocytopenic purpura is an uncommon disease and is rare in systemic lupus erythematosus. Rarely, patients do not respond to plasma exchange therapy, and treatment options are limited and often disappointing, thus it is a therapeutic challenge to clinicians. We report a patient with lupus nephritis who developed refractory thrombotic thrombocytopenic purpura and was subsequently treated with cyclosporin A. An immediate and sustained response was demonstrated, and she remained well; eventually she was able to be cyclosporin free after the acute episode. Cyclosporin A can be a safe and effective therapeutic option in patients with refractory thrombotic thrombocytopenic purpura.     

Low fetal morbidity in pregnancy associated with acute and chronic idiopathic thrombocytopenic purpura

Forty-six mothers with immune thrombocytopenic purpura (ITP) gave birth to 72 babies. Sixty-two babies were delivered vaginally and 10 babies by cesarean section. There was no mortality among mothers or babies. Eighteen infants were born thrombocytopenic (PLT < 100 ± 10⁹/l). Eleven infants had a platelet count of less than 50 ± 10⁹/l. All the severely thrombocytopenic babies (except 1) were born to post splenectomy thrombocytopenic mothers, regardless of steroid treatment during pregnancy. Five babies had clinical manifestations of bleeding; 3 had mild purpura, 1 severe gastrointestinal bleeding, and 1 intracranial bleeding. The latter 2 babies were born prematurely to the same mother who was severely thrombocytopenic despite splenectomy in childhood. In view of very low morbidity in babies of ITP mothers, we suggest that they be delivered vaginally. Cesarean delivery should be performed in selected cases where the mother is severely thrombocytopenic despite splenectomy or where prematurity or obstetrical complications are encountered. © 1994 Wiley-Liss, Inc.     

Thrombotic thrombocytopenic purpura unresponsive to plasma infusion and plasma exchange, but responsive to splenectomy

A 60-yr-old female presented with typical thrombotic thrombocytopenic purpura (TTP). She remained in coma with frequent seizures for 1 wk, with persisting severe thrombocytopenia and microangiopathic haemolytic anaemia, despite treatment with prednisolone, plasma exchange, fresh frozen plasma, sulphinpyrazone and dipyridamole. Splenectomy induced haematological improvement within 1 d, there was cessation of fitting after 2 d, and full neurological recovery ensued over 3 wk. Laboratory studies did not reveal the presence of a platelet-aggregating factor (PAF), stated to be present in some two-thirds of cases. While plasma exchange and plasma infusion are beneficial in many cases, splenectomy appears still to be of value in unresponsive disease.     

Accessory spleen in recurrent chronic immune thrombocytopenic purpura.

From 1969 to 1985 we discovered accessory spleens in 8 patients with chronic immune thrombocytopenic purpura (ITP) who relapsed or failed after splenectomy. Imaging of accessory spleen used a liver spleen scintigraphy with heat-treated RBC labeled with Tc-99m. Platelet kinetic studies with 51Cr or 111In, including sequestration index, were performed. Five patients had accessory splenectomy. Disappearance of bleeding symptoms was achieved in the 5 splenectomized patients but with only partial response of platelet counts. These results are discussed in the context of diagnosis and therapeutic management of accessory spleens in patients with chronic immune thrombocytopenic purpura who relapsed or failed after splenectomy.     

High-dose intravenous gamma-immunoglobulin infusion in the treatment of thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura has been treated with plasmapheresis, exchange transfusion, splenectomy, antiplatelet compounds, glucocorticoids, and vincristine sulfate infusion. We describe a patient with thrombotic thrombocytopenic purpura who has failed on treatment with plasmapheresis, antiplatelet compounds, glucocorticoids, and vincristine, was successfully treated with high-dose gamma-immunoglobulin infusion, and has been maintained in prolonged remission without further immunoglobulin infusion for more than 14 months at the time of this report.     

The response to high-dose intravenous immunoglobulin or steroids is not predictive of outcome after splenectomy in adults with autoimmune thrombocytopenic purpura

The response to high-dose intravenous immunoglobulin (IVIg) was recently reported to be predictive of outcome after splenectomy in patients with autoimmune thrombocytopenic purpura (AITP). We analysed the records of 75 adults with chronic AITP who received IVIg and subsequently underwent splenectomy. There was no significant difference in the response rate to splenectomy according to whether or not patients had responded to IVIg (81% v 67%, P = 0.36). Age, the time from diagnosis to splenectomy, and the response to steroids were also not significantly associated with outcome after splenectomy. These results indicate that the response to IVIg or steroids is not predictive of the efficacy of splenectomy.     

A dramatic response to rituximab in a patient with resistant thrombotic thrombocytopenic purpura (TTP) who developed acute stroke

Background Refractory condition can occur in 10–30% of all cases of thrombotic thrombocytopenic purpura despite increased frequency of total plasma exchange. Rituximab can affect the clinical outcome of the refractory cases. However, little is known about usefulness of rituximab on central nervous system involvement mimicking acute ischemic stroke. Methods We report the case of a woman with refractory thrombotic thrombocytopenic purpura who developed an acute onset right sided paralysis, dysarthria, and central facial paralysis, suggestive of cerebrovascular accident while under plasma exchange, corticosteroid, and vincristine therapy. Results After initiation of rituximab (375 mg/m² weekly for 4 weeks), a dramatic response occurred and the patient’s neurologic function recovered fully within days. Sustained remission was achieved, and the patient was well 1 year after her admission, while she was on azathioprine treatment. Conclusion This report suggests that rituximab can provide a good outcome of the dramatic central nervous system involvement in patients with thrombotic thrombocytopenic purpura.