Efficacy and safety of cyclosporine-based regimens for primary immune thrombocytopenia: a systematic review and meta-analysis

Li, Xiaojing; Zhu, Wenwei; Bao, Jizhang; Li, Jiekai; Zhou, Yongming

doi:10.1177/03000605221149870

Cited by 3 publications

(2 citation statements)

References 30 publications

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“…The IL-2-dependent pathway, in addition to IL-6, has recently been assumed to affect the etiology of TAFRO syndrome (20). Cyclosporine is also used to suppress the release of proinflammatory cytokines, including IL-2 (21). Its effect on the suppression of immune-medicated reactions was successful and yielded favorable results in this case.…”

Section: Discussionmentioning

confidence: 72%

Case Report: A case of TAFRO syndrome with severe and prolonged thrombocytopenia: diagnostic pitfalls

Sato,

Kanno,

Sato

et al. 2023

Front. Immunol.

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Thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a rare condition with diverse clinical and pathological characteristics related to multi-organ damage. We report a case of TAFRO syndrome complicated by immune thrombocytopenia with prolonged fever and thrombocytopenia for several weeks. A 61-year-old man was transferred with sepsis caused by Enterococcus faecalis, and developed disseminated intravascular coagulation. Antibiotics treatment was initiated: however, low-grade fever and thrombocytopenia persisted despite the adequate antimicrobial treatment. Systemic edema, pleural effusion, and ascites had developed before hospitalization, and renal and liver function had deteriorated, resulting in progressive multi-organ damage. Prednisolone 40 mg/day was initiated based on the assumption of a condition in which excessive production of inflammatory cytokines would lead to systemic deterioration and fatal organ damage. Subsequently, the fever resolved, and renal function began to normalize. However, thrombocytopenia did not show much recovery trend after Helicobacter pylori eradication therapy and initiation of thrombopoietin receptor agonists. Bone marrow biopsy results showed normal bone marrow with no malignant findings. Alternatively, significant clinical signs met the diagnostic criteria for TAFRO syndrome, and a renal biopsy revealed thrombotic microangiopathy, which is also reasonable for renal involvement in TAFRO syndrome. The use of cyclosporine remarkably corrected the thrombocytopenia. We considered this a case of TAFRO syndrome that developed after sepsis with disseminated intravascular coagulation and performed the differential diagnosis of prolonged thrombocytopenia and excluded it. Although TAFRO syndrome is a unique disease concept, diagnostic criteria may consist of nonspecific elements such as generalized edema, thrombocytopenia, persistent fever, and elevated inflammatory response, and there are many differential conditions to exclude, requiring caution in diagnosing TAFRO syndrome.

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Section: Discussionmentioning

confidence: 72%

Case Report: A case of TAFRO syndrome with severe and prolonged thrombocytopenia: diagnostic pitfalls

Sato,

Kanno,

Sato

et al. 2023

Front. Immunol.

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“…Successful use of cyclosporin post rituximab has been described in case report and case series ( 141 , 142 ). In a recent meta-analysis cyclosporine-based combinations improved ORR and CR rates while reducing the rate of relapse ( 145 ). Importantly, this improvement was achieved without increasing the rate of adverse events.…”

Section: Treatment To Overcome Rituximab Resistancementioning

confidence: 99%

Rituximab resistance in ITP and beyond

Xiao

Murakhovskaya

2023

Front. Immunol.

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The pathophysiology of immune thrombocytopenia (ITP) is complex and encompasses innate and adaptive immune responses, as well as megakaryocyte dysfunction. Rituximab is administered in relapsed cases and has the added benefit of inducing treatment-free remission in over 50% of patients. Nevertheless, the responses to this therapy are not long-lasting, and resistance development is frequent. B cells, T cells, and plasma cells play a role in developing resistance. To overcome this resistance, targeting these pathways through splenectomy and novel therapies that target FcγR pathway, FcRn, complement, B cells, plasma cells, and T cells can be useful. This review will summarize the pathogenetic mechanisms implicated in rituximab resistance and examine the potential therapeutic interventions to overcome it. This review will explore the efficacy of established therapies, as well as novel therapeutic approaches and agents currently in development.

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Characteristics and treatment responses of immune thrombocytopenia in patients with primary Sjögren syndrome

Sun¹,

Qi²,

Wei³

et al. 2023

International Immunopharmacology

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Efficacy and safety of cyclosporine-based regimens for primary immune thrombocytopenia: a systematic review and meta-analysis

Cited by 3 publications

References 30 publications

Case Report: A case of TAFRO syndrome with severe and prolonged thrombocytopenia: diagnostic pitfalls

Case Report: A case of TAFRO syndrome with severe and prolonged thrombocytopenia: diagnostic pitfalls

Rituximab resistance in ITP and beyond

Characteristics and treatment responses of immune thrombocytopenia in patients with primary Sjögren syndrome

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