Thrombotic thrombocytopenic purpura and systemic lupus erythematosus.

We report two patients with systemic lupus erythematosus who subsequently developed thrombotic thrombocytopenic purpura. In each case the coexistence of these two conditions was confirmed by pathological findings. Both patients responded to treatment, but one eventually died. A review of the literature suggests a possible relationship between the two disorders.     

Thrombotic thrombocytopenic purpura and systemic lupus erythematosus.

Thrombotic thrombocytopenic purpura (TTP) is a rarely seen complicating systemic lupus erythematosus (SLE). The diagnosis of TTP in a setting of SLE is challenging since both share common features including thrombotic microangiopathy. We report two cases of SLE with TTP one with a good response when cyclophosphamide was given early with plasmapheresis and steroids; the other with a poor outcome in a patient given cyclophosphamide late in the course of the disease.     

Thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus

Thrombotic thrombocytopenic purpura (TTP) occurring in patients with systemic lupus erythematosus (SLE) is rare and can be difficult to diagnose because of overlapping features of the two disorders. The aim of this study is to further characterize this uncommon association in terms of presenting features, diagnostic difficulties and treatment outcome. This is the largest series from a single centre with 6 patients diagnosed over a 6-year period. Two thirds of the patients had a simultaneous diagnosis of TTP and SLE. Half of the patients had a positive Coombs test along with clear features of TTP. Five patients received plasmapheresis as initial treatment while 1 patient received plasma infusions only. Four out of 5 patients responded to plasmapheresis and only 1 patient required cytotoxic therapy. TTP in association with SLE appears to be underdiagnosed and a positive Coombs test is not against the diagnosis of TTP in this setting. Most of the patients respond well to plasmapheresis. In case of a poor response, cytotoxic drugs should be considered early.     

Thrombotic thrombocytopenic purpura as an etiology of thrombocytopenia in systemic lupus erythematosus: case report

Thrombotic thrombocytopenic purpura (TTP) is an unusual complication of systemic lupus erythematosus (SLE). Although the reported association between SLE and TTP is increasing, a few cases do improve without plasmatherapy. We report a case of TTP which was successfully treated without plasmatherapy, which might be underestimated as an etiology of thrombocytopenia in SLE. TTP should always be considered as a concomitant disease when Coombs' negative hemolytic anemia or thrombocytopenia is seen in SLE patients.     

Thrombotic thrombocytopenic purpura as an etiology of thrombocytopenia in systemic lupus erythematosus: case report

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an unusual complication of systemic lupus erythematosus (SLE). Although the reported association between SLE and TTP is increasing, a few cases do improve without plasmatherapy. We report a case of TTP which was successfully treated without plasmatherapy, which might be underestimated as an etiology of thrombocytopenia in SLE. TTP should always be considered as a concomitant disease when Coombs' negative hemolytic anemia or thrombocytopenia is seen in SLE patients.     

Thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus. Case report and literature review from the plasmapheresis era.

Although thrombotic thrombocytopenic purpura (TTP) has been described in patients with systemic lupus erythematosus (SLE), the relationship between these 2 diseases is controversial. We recently treated a patient with longstanding SLE who developed TTP. The patient responded to therapy with aggressive plasmapheresis. Review of the literature revealed that TTP may occur in the setting of either active or inactive SLE. Survival in SLE associated TTP correlated with the use of plasma therapy (plasma infusion or plasmapheresis) rather than with the activity of the underlying autoimmune disease. We conclude that TTP and SLE represent distinct clinical entities that may occur together in an immunologically predisposed host. The use of plasma therapy appears to have had a significant impact on survival in these patients.     

Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: disease activity and the use of cytotoxic drugs

Thrombotic thrombocytopenic purpura (TTP) is a rare and occasionally fatal haematologic disorder that can coexist with systemic lupus erythematosus (SLE) and other autoimmune diseases. We identified all cases of TTP seen in our institution over a 3 year period using a computerized database. We found that SLE activity (measured by the SLE Disease Activity Index) and TTP activity ran a parallel course in three patients with coexistent SLE and TTP. TTP in these three patients, although refractory to plasmapheresis, responded to cytotoxic therapy. These observations further support an autoimmune contribution to the pathogenesis of some cases of TTP. A literature review revealed that mortality in SLE patients with more severe, refractory TTP treated with plasmapheresis and cytotoxics, may not be higher than in patients responding to plasmapheresis alone (who are likely to have milder disease). These data suggest that cytotoxics may have a role in treatment of patients with active SLE and TTP refractory to plasmapheresis.     

Thrombotic thrombocytopenic purpura in systemic lupus erythematosus and antiphospholipid antibodies: effective treatment with plasma exchange and immunosuppression.

We describe 2 patients with systemic lupus erythematosus (SLE) with neurological complications and thrombocytopenia, who, upon further investigation, were found to have thrombotic thrombocytopenic purpura. While neither patient responded to treatment with corticosteroids, both responded to plasma exchange and intravenous cyclophosphamide. This highlights the need to carefully consider this diagnosis in patients with SLE presenting with neurological complications and thrombocytopenia.     

Thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus successfully treated with plasma exchange and corticosteroids

Thrombotic thrombocytopenic purpura (TTP) is a rare dynamic life-threatening disease with systemic formation of platelet thrombi in the microvasculature within all the organs. Until recently the mortality in TTP has exceeded 90%. The progress of medical research in the last two decades has significantly improved our understanding of the pathogenesis of TTP and allowed a reduction in mortality. This paper describes a case of a 36-year-old female patient with systemic lupus erythematosus (SLE) who was admitted to our hospital for jaundice and speech disturbances. Laboratory tests revealed hemolytic anemia with a negative Coombs test. Abundant schistocytes were present in peripheral blood smear. During the first day of hospitalization progression of neurological signs was observed -the patient received high-dose corticosteroids (1000 mg of methylprednisolone daily for 5 consecutive days) and underwent plasma exchange therapy. We observed significant improvement of the patient's condition, as well as a complete resolution of clinical and laboratory abnormalities. In the following 24 months there have been no signs of the relapse of TTP. The article contains also a brief update of this unusual disease.     

Thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura is an uncommon disorder, but it continues to be of considerable interest. The disease mechanisms are unclear and the aetiology is unknown. Perhaps most enigmatic of all, the mode of action of plasma therapy, which successfully induces remission in about two-thirds of cases, is wholly inexplicable. There are currently several areas of debate on the subject of thrombotic thrombocytopenic purpura. This paper addresses these points of contention: the definition of the disease, its distinction from haemolytic uraemic syndrome, the nature of the platelet aggregating factors in the plasma of patients with acute disease, the importance of the abnormalities of von Willebrand's factor observed in the acute and quiescent phases of the disease, the nature of the factor in normal plasma that induces remission, and the possible causes of the observed superiority of plasma exchange combined with plasma infusion, over plasma infusion alone.     

Fatal thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

An immunologic mechanism, possibly immune complex mediated, has been suggested as the basis for the pathogenesis of thrombotic thrombocytopenic purpura (TTP). The evidence supporting this concept has been the association of TTP with systemic lupus erythematosus and the successful therapy of TTP by plasmapheresis. However, most investigators have failed to demonstrate elevated circulating immune complexes during the course of TTP. This report describes a young woman with systemic lupus who developed TTP as a terminal event. Elevated levels of immune complexes were associated with periods of active lupus but were not detectable at the time she developed TTP.     

Rituximab in recalcitrant thrombotic thrombocytopenic purpura secondary to systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a typical autoimmune disease with manifestations due to unopposed production of autoantibodies against the patient's own cells. The clinical features are diverse, ranging from musculoskeletal involvement, lupus nephritis to cerebral and even haematological involvement. We report a case of a young woman with known SLE who developed thrombotic thrombocytopenic purpura (TTP) secondary to SLE resistant to conventional treatment with plasma exchange. She was then treated with rituximab (MabThera®), a CD20 monoclonal antibody, and showed remarkable improvement. To our best knowledge this is the first case reporting the use of rituximab in acute resistant TTP secondary to SLE.     

Recurrent thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

We encountered a 39-year-old female patient with systemic lupus erythematosus (SLE) in whom thrombotic thrombocytopenic purpura (TTP) recurred. The patient was successfully treated with corticosteroid in combination with immunosuppressive agents. Because TTP complicating SLE is more resistant to treatment than idiopathic TTP, prompt diagnosis and efficacious initial treatment are critical.