Peripheres T-Zell-Lymphom mit Imitation einer Granulomatose mit Polyangiitis

Ediboglu, E. Durak; Solmaz, Dilek; Özkal, Şermin; Erdoğan, Nuri; Akar, Servet

doi:10.1007/s00393-023-01370-1

Cited by 1 publication

(1 citation statement)

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“…In this case report, the patient was previously diagnosed with lung cancer with multiple metastases at an outside hospital. However, ANCA-associated vasculitis has a higher chance of being combined with a tumor compared to the normal population, and a case of peripheral T-cell lymphoma mimicking GPA has also been reported (32). Therefore, we still cannot slacken the tracking of tumors in such patients during follow-up.…”

Section: Discussionmentioning

confidence: 99%

Combination treatment with telitacicept, cyclophosphamide and glucocorticoids for severe Granulomatous polyangiitis: a case report and literature review

Huang,

Lin,

Liu

et al. 2023

Front. Immunol.

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Granulomatous polyangiitis (GPA) is a rare autoimmune disease that can involve multiple systems throughout the body, including the ear, nose, upper and lower respiratory tracts. It is classified as an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Telitacicept is a novel recombinant fusion protein targeting B-lymphocyte stimulator (BLyS). Telitacicept can inhibit the development and maturation of abnormal B cells by blocking BLyS, and inhibit the production of antibodies by abnormal plasma cells by blocking APRIL (A proliferation-inducing ligand), which is expected to become a new drug for the treatment of GPA. We report a 64-year-old man diagnosed at our hospital with GPA involving multiple systems including kidneys, lungs, nose and ears. Renal involvement was severe, with a clinical characteristic of rapidly progressive glomerulonephritis and a pathologic manifestation of crescentic nephritis with plasma cell infiltration. The patient was treated with hormones, immunoglobulins and cyclophosphamide (CYC) with the addition of telitacicept and a rapid reduction in hormone dosage. The patient’s renal function improved significantly within a short period of time, and his hearing and lung lesions improved significantly. At the same time, he did not develop serious infections and other related complications. Our report suggests that short-term control of the patient’s conditions is necessary in GPA patients with organ-threatening disease. Telitacicept combined with CYC and glucocorticoids may be an induction therapy with safety and feasibility. However, more clinical trials are needed to validate the efficacy and safety of the therapeutic regimen.

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Section: Discussionmentioning

confidence: 99%