A case of TAFRO syndrome with DIC and neurologic and cardiac involvement

Moy, Lindsay N.; Patel, Mauli; Eschbach, Joseph W.; Knouse, Phillip; Galvez, Angel G.

doi:10.1002/ccr3.7340

Cited by 3 publications

(1 citation statement)

References 13 publications

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“…As described above, we reported cardiomyopathy, a rare complication of TAFRO syndrome, which was reversed upon treatment with tacrolimus and glucocorticoids. To date, cardiomyopathy in TAFRO syndrome has been reported in only a few cases [57][58][59], one of which was refractory to anti-IL-6 therapy, suggesting that other proinflammatory cytokines may be involved [57]. Given the documented association between high-dose IL-2 immunotherapy and reversible cardiomyopathy [60], and our case's successful treatment outcome with tacrolimus, IL-2 might contribute to cardiomyopathy in TAFRO syndrome, although the mechanism remains poorly understood.…”

Section: Rituximabmentioning

confidence: 76%

Tacrolimus Treatment for TAFRO Syndrome

Shirai,

Ichikawa,

Saegusa

2024

Biomedicines

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TAFRO syndrome is an acute systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. While its lymph node pathology is similar to that of idiopathic multicentric Castleman disease (iMCD), the clinical features of TAFRO syndrome differ from those of typical iMCD, as they include a more aggressive clinical course and high mortality. However, an optimal treatment strategy for TAFRO syndrome has not yet been established, owing to a poor understanding of its pathogenesis. The limited cases we encountered suggest that tacrolimus treatment in combination with glucocorticoids may potentially be effective and well tolerated as an initial treatment, and hold promise as a glucocorticoid-sparing agent. Herein, we report an additional case and review the sparse literature available regarding TAFRO syndrome treated via tacrolimus.

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Section: Rituximabmentioning

confidence: 76%

Tacrolimus Treatment for TAFRO Syndrome

Shirai,

Ichikawa,

Saegusa

2024

Biomedicines

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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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