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.     

Recurrent thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

Abstract

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.     

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.     

Fatal cardiac arrhythmia in a patient with thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP), a well-recognized syndrome with typical diagnostic features, has been implicated in rare causes of sudden death. Involvement of TTP on the myocardium and cardiac conduction system has been characterized, but the consequences related to these effects are infrequently reported. The outcome is almost always death, thus underscoring the need for early detection and therapy. A case is reported of a 36-year-old man who suffered a fatal cardiac arrhythmia related to intramyocardial hemorrhage with a diagnosis of TTP established by postmortem examination.     

Systemic lupus erythematosus presenting as thrombotic thrombocytopenic purpura

We describe a 10-year-old girl, who presented with thrombotic thrombocytopenic purpura (TTP) and shortly thereafter developed systemic lupus erythematosus (SLE). The association between TTP and SLE is known, but this is the first report of SLE presenting as TTP.     

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.     

Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood.

OBJECTIVE: To determine the association between childhood-onset thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE). METHODS: The charts of all 5 patients diagnosed with idiopathic TTP at the Hospital for Sick Children (HSC) in Toronto from 1975 to 1998, and all cases of childhood-onset TTP (ages 6-20 years) reported in the literature over the same period were reviewed. Fourteen of the 44 patients identified in the literature were excluded from the analysis for lack of clinical and laboratory information. The remaining 35 patients were grouped into either an SLE/TTP group or a TTP only group, according to the presence or absence of the American College of Rheumatology (ACR) classification criteria for SLE. The groups were compared for differences in clinical or laboratory features. RESULTS: The clinical presentation and initial disease course of pediatric patients with TTP were similar to those observed in adults. Of the 35 patients with childhood-onset TTP included in this review, 9 (26%) fulfilled > or = 4 ACR criteria for SLE and 8 (23%) were found to have incipient SLE. Of the 5 patients initially diagnosed with idiopathic TTP at the HSC, 3 were diagnosed with SLE within 3 years, and the other 2 patients fulfilled 3 ACR classification criteria for SLE within 4 years of disease onset. The clinical syndrome of pediatric TTP presenting with proteinuria, especially with high-grade proteinuria, was significantly associated with the development or coexistence of childhood-onset SLE. CONCLUSION: TTP in childhood is a rare, but life-threatening, disease. Unlike in adults, TTP in childhood is commonly associated with SLE. High-grade proteinuria at diagnosis of TTP is the best predictor for the presence or subsequent development of SLE.     

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.     

Response to belimumab in thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a case-based review

Clinical Rheumatology - Thrombotic thrombocytopenic purpura (TTP), a life-threatening syndrome characterized by acute microangiopathic hemolytic anemia, thrombocytopenia, and visceral ischemia, can...     

Severe thrombocytopenic purpura due to rubella infection in a patient with systemic lupus erythematosus

We report the case of a woman suffering from systemic lupus erythematosus who developed a severe thrombocytopenic purpura (platelet count < 1 x 10(9)/l) secondary to rubella infection. The search for antiplatelet antibodies revealed transient circulating anti-GPIIb-IIIa and anti-GPIb-IX platelet antibodies. After a few weeks, bound anti-GPIIb-IIIa antiplatelet antibodies were still detectable and they persisted several months after recovery, probably in relation to a mild autoimmune thrombocytopenia related to systemic lupus erythematosus. To our knowledge, this is the first case report of severe thrombocytopenic purpura due to rubella in an adult with systemic lupus erythematosus.     

The frequency of thrombotic thrombocytopenic purpura in patients with systemic lupus erythematosus undergoing kidney biopsy

OBJECTIVE: To characterize the frequency of thrombotic thrombocytopenic purpura (TTP) among patients with systemic lupus erythematosus (SLE) undergoing kidney biopsy. METHODS: A retrospective review of all renal biopsies of patients with SLE at Rush-Presbyterian-St. Luke's Medical Center was performed for the years 1989 to 2001. RESULTS: Four cases of clinical and histopathological TTP were identified among the 257 patients with SLE who underwent renal biopsy during the 12 year study period. CONCLUSION: TTP appears to occur at higher than expected frequency among SLE patients undergoing biopsy for unexplained renal failure.     

Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus

We report the case of a patient with systemic lupus erythematosus (SLE) who first revealed hemophagocytic syndrome (HPS), which was treated successfully with glucocorticoid and intravenous cyclophosphamide. The patient then demonstrated refractory thrombotic thrombocytopenic purpura (TTP) with normal a disintegrin and metalloprotease with thrombospondin motifs (ADAMTS)-13 activity that responded well to rituximab. After rituximab treatment, the patient showed a flare of HPS that was controlled by additional intravenous cyclophosphamide treatment. This case showed that TTP with normal ADAMTS-13 activity is B-cell dependent and indicated that B-cell depletion might exacerbate some autoimmune conditions in SLE.     

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 preceding systemic lupus erythematosus.

The case of a patient admitted with thrombotic thrombocytopenic purpura nine years after developing systemic lupus erythematosus (SLE) is reported. Thrombotic thrombocytopenic purpura associated with SLE has been described on other occasions, but in most patients the diagnosis of SLE precedes that of thrombotic thrombocytopenic purpura. The unusual sequence and the chronological separation of the two diseases is emphasised.     

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.