Thrombotic thrombocytopenic purpura in a new onset lupus patient?

González, Nery Sablón; Lorenzo, Noel; Parodis, Yanet; Ortiz, María Belén Alonso; Kéchida, M.; Pérez, José Carlos Rodríguez

doi:10.1007/s12026-017-8898-y

Cited by 5 publications

(4 citation statements)

References 15 publications

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“…This treatment should be continued until the first platelet counts appear within normal limits for two consecutive days. 11 However, in this report the plasma exchange treatment was momentarily discontinued after four days even though the platelet level was not in the normal range. This approach was applied because, according to the consensus of experts in our hospital, the patient showed a favorable laboratory pattern in which the platelet level increased and the LDH concentration decreased.…”

Section: Discussionmentioning

confidence: 69%

Case Report: Systemic lupus erythematous associated with thrombotic thrombocytopenic purpura, a diagnostic challenge

et al. 2022

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Thrombotic thrombocytopenic purpura (TTP) is an uncommon microangiopathic disease and sometimes is associated with systemic lupus erythematous (SLE). However, this probable causal relationship has not been completely proven. The diagnostic differentiation of both diseases is difficult in the first instance because they share similar characteristics that may overlap. We present a case of a 32-year-old woman with antecedents of epileptic seizures since she was 12 years old. The patient was admitted to the emergency room with a clinical picture of headaches, fever, paleness in the skin and mucosa, confused state, paresthesia, and transient spasticity of the extremities. The laboratory results revealed direct Coombs negative hemolytic anemia, severe thrombocytopenia, significant elevation of lactate dehydrogenase, and presence of schistocytes ++ in the peripheral film. In addition, positive antinuclear antibodies and positive anti-native DNA in titers of 1/320 and 1/160, respectively, were found. Urinalysis showed that serum creatinine was in normal range. Because of limited hospital resources, ADAMTS13 was not evaluated. However, based on clinical, hematological, and biochemical findings, we concluded that it was a case of TTP associated with SLE and indicated treatment with plasmapheresis and methylprednisolone pulses, obtaining a satisfactory response (normalization of biomarker levels, health condition) after the second session of plasmapheresis. Diagnosis of both SLE and TTP is often difficult to achieve; however, adequate correlation of clinical manifestations and laboratory tests, along with the help of partial therapeutic interventions, may lead to good clinical response.

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Section: Discussionmentioning

confidence: 69%

Case Report: Systemic lupus erythematous associated with thrombotic thrombocytopenic purpura, a diagnostic challenge

et al. 2022

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“…Plasma exchange independent of ADAMTS13 activity is important and is recommended until the first platelet counts appear within normal limits for two consecutive days. 10 TTP should always be considered in patients with hemolytic anemia and thrombocytopenia; however, in young women, SLE and TTP can occur simultaneously.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Systemic lupus erythematous associated with thrombotic thrombocytopenic purpura, a diagnostic challenge

et al. 2021

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Thrombotic thrombocytopenic purpura (TTP) is an uncommon microangiopathic disease and often occurs as a complication of systemic lupus erythematous (SLE). However, this probable causal relationship has not been completely proven. The diagnostic differentiation of both diseases is difficult in the first instance because they share similar characteristics that may overlap. We present a case of a 32-year-old woman with antecedents of epilepsy since she was 12 years old. The patient was admitted to the emergency room with a clinical picture of headaches, fever, paleness in the skin and mucosa, confused state, paresthesia, and transient spasticity of the extremities. The laboratory results revealed Coombs negative direct autoimmune hemolytic anaemia, severe thrombocytopenia, significant elevation of the enzyme lactate dehydrogenase, and presence of schistocytes ++ in the peripheral film. In addition, positive antinuclear antibodies and positive anti-native DNA in titers of 1/320 and 1/160, respectively, were found. Renal function was conserved. We concluded that it was a case of TTP associated with SLE and indicated treatment with plasmapheresis and methylprednisolone pulses, obtaining a satisfactory response (normalization of biomarker levels, health condition) after the second session of plasmapheresis. Diagnosis of both SLE and TTP is often difficult to achieve; however, adequate correlation of clinical manifestations and laboratory tests, along with the help of partial therapeutic interventions, may lead to good clinical response.

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“…However, although there are case reports and single-arm reports reporting the effect of RTX on CTDassociated TTP/TMA [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30], with some reports of exacerbations [31], there are no studies comparing an RTX-intervention group to a control group. In our department, RTX has been administered for CTDassociated refractory TTP/TMA after obtaining ethics committee approval and written consent.…”

Section: Introductionmentioning

confidence: 99%

Efficacy and safety of rituximab in connective tissue disease-associated thrombotic thrombocytopenic purpura/thrombotic microangiopathy

Ohkubo

Nakayamada

Hanami

et al. 2023

Preprint

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Background This study examined the efficacy and safety of Rituximab (RTX) treatment in connective tissue disease (CTD)-associated thrombocytopenic purpura (TTP) and thrombotic microangiopathy (TMA), using historical controls as comparators. Methods Patients who were admitted to our department from March 1, 2013, to March 31, 2021, and diagnosed with CTD-associated TTP/TMA refractory to plasma exchange were included in the retrospective study. A patient with treatment-resistant disease was treated with RTX in addition to high-dose glucocorticoid (GC) therapy (GC + RTX). As historical controls, we selected patients with CTD-associated TTP/TMA who were admitted to our center and treated with GC and immunosuppressants (IS) such as cyclophosphamide. The primary endpoint was the survival rate at 52 weeks after initiation of treatment. Results Fifteen patients were enrolled in the study (GC + RTX). As a control group, 11 patients were enrolled in the same manner (GC + IS). There were no significant differences in age or sex or laboratory tests between the two groups. The primary endpoint of survival rate was higher in the GC + RTX group than in the GC + IS group. In the immunophenotyping analysis before treatment, among all subsets of immune cells, only plasmocytes were elevated in TTP patients compared to healthy controls. The proportion ofplasmocytes correlated with serum markers, suggesting increased B cell differentiation, which was markedly decreased after RTX treatment. Conclusion In CTD-associated TTP/TMA, the addition of RTX to plasma exchange and GC therapy may improve B cell-based pathology and survival.

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Thrombotic thrombocytopenic purpura in a new onset lupus patient?

Cited by 5 publications

References 15 publications

Case Report: Systemic lupus erythematous associated with thrombotic thrombocytopenic purpura, a diagnostic challenge

Case Report: Systemic lupus erythematous associated with thrombotic thrombocytopenic purpura, a diagnostic challenge

Case Report: Systemic lupus erythematous associated with thrombotic thrombocytopenic purpura, a diagnostic challenge

Efficacy and safety of rituximab in connective tissue disease-associated thrombotic thrombocytopenic purpura/thrombotic microangiopathy

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