A case of TAFRO syndrome after vaccination, successfully treated with cyclosporine

Abstract

Background: TAFRO syndrome is a rare disorder that causes thrombocytopenia, generalized oedema, fever, organ enlargement, and renal impairment. Few reports have suggested an association with vaccines, and few cases have undergone renal biopsy. TAFRO syndrome is often severe and fatal, and its cause is unknown. We report a case of TAFRO syndrome that occurred after vaccination with the coronavirus disease 2019 (COVID-19) vaccine.

Case presentation: An 82-year-old woman received two doses of the BNT162b2 mRNA vaccine 3 weeks apart. Two weeks later, she was admitted to the hospital with oedema, accompanied with renal failure and thrombocytopenia. After close examination, she was diagnosed with TAFRO syndrome. She was treated with steroids, cyclosporine, and thrombopoietin receptor agonists. The patient was discharged after several months in remission.

Conclusions: Although an incident of TAFRO syndrome after COVID-19 vaccination has been previously reported, this is a rare case in which the patient went into remission and was discharged. A renal biopsy was also performed in this case, which was consistent with previous reports. The favorable treatment course for TAFRO syndrome provides valuable insights.

Keywords: BNT162b2 mRNA; COVID-19; ITP (idiopathic thrombocytopenic purpura); MIS-A (multisystem inflammatory syndrome in adults); SCLS (systemic capillary leak syndrome); TAFRO syndrome.

Fig. 1

Computerized tomography imaging. A The liver is enlarged, and the spleen is mildly enlarged. B, C Scattered lymph node enlargement (< 1.5 cm) is noted at the neck, axillae and abdomen

Fig. 2

Clinical course of the patient. CRP, C-reactive protein; mPSL, methylpredonisolone; PSL, prednisolone; CyA: cyclosporine A; HD, hemodialysis

Fig. 3

Chest X-ray and computed tomography imaging taken on the 6th day of hospitalization. A, B Pleural effusion increased on the left side compared to results at admission

Fig. 4

Pathological imaging. A, B Bone marrow biopsy: Mild fibrosis and megakaryocytosis are observed, without malignant findings or increase in blasts and plasma cells. (Periodic acid–Schiff staining, × 400). C, D Axillary lymph node biopsy: Resinous vascular growth and an increased number of plasma cells are observed. No malignant findings, such as cellular degeneration. (hematoxylin and eosin staining, × 100)

Fig. 5

Renal biopsy imaging on light microscopy. A, B Periodic acid–Schiff staining and Masson trichrome staining, × 100: Total of 7 glomeruli, one obsolescence, semilunar formation ( −), Adhesions ( −). C Periodic acid–Schiff staining, × 400: Mesangium substratum is generally thickened. Thickening of the hoof wall and narrowing of the hoof cavity are observed. D Periotic acid methenamine silver staining, × 400: Doubling of the mooring hoof wall is observed. No nodule formation is observed.

Fig. 6

Immunofluorescence imaging of renal biopsy. Granular deposition of IgA and C3 on the capillary wall is observed. Other (IgG, IgM, and C1q) depositions were negative

Fig. 7

Electron microscopy imaging of renal biopsy. Electron microscopy: Edematous enlargement of the subendothelial space. Edematous changes in the mesangial area without deposits are noted

Fig. 8

International standard treatment guidelines and previously reported treatments. R-CHOP, rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone; R-VDT-PACE, rituximab, bortezomib, dexamethasone, thalidomide, cisplatin, doxorubicin, cyclophosphamide, etoposide; R-CVP, rituximab, cyclophosphamide, vincristine, prednisone