Thrombotic microangiopathy on kidney biopsy in a patient with TAFRO syndrome

Ozeki, Toshikazu; Tsuji, Masashi; Yamamoto, Junichiro; Shigematsu, Chisato; Maruyama, Shoichi

doi:10.1007/s13730-018-0338-x

Cited by 19 publications

(12 citation statements)

References 11 publications

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“…To date, five biopsy cases describing the renal histology of TAFRO syndrome have been reported (Table 3). Two cases (case 1 and case 4) showed thrombotic microangiopathy-like lesions, another Mesangial expansion and widening of the subendothelial space with a large amount of electron-dense deposits two cases (case 3 and case 5) showed a membranoproliferative glomerulonephritis-like appearance, and one case (case 2) showed diffuse endocapillary proliferation with mesangiolysis [20][21][22][23][24]. Immunofluorescence studies were negative for immunoglobulins or complement factors in three cases (cases 2, 3, and 4).…”

Section: Discussionmentioning

confidence: 96%

An autopsy case of TAFRO syndrome with membranoproliferative glomerulonephritis-like lesions

et al. 2018

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TAFRO syndrome (thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly) is an atypical manifestation of multicentric Castleman's disease. Although overproduction of interleukin-6, vascular endothelial growth factor, and other cytokines may partially explain the pathophysiology of this rare syndrome, the precise mechanisms underlying the renal dysfunction associated with the condition remain unclear. Here, we describe a case of a 69-year-old male with TAFRO syndrome. He was treated with immunosuppressive agents and his renal function improved. Tapering of immunosuppressive agents resulted in a deterioration of renal function and an elevation of C-reactive protein. After 20 months of treatment, the patient died from tuberculous peritonitis and gastrointestinal bleeding. An autopsy revealed miliary tuberculosis, mediastinal lymphadenopathy, and gastric ulcers. Renal histopathology showed a membranoproliferative glomerulonephritis-like appearance. Almost all glomeruli showed lobular formations with mesangial proliferation and duplication of glomerular capillary walls on light microscopy. Immunofluorescence showed deposition of C1q and IgM along the glomerular capillary walls. Electron microscopy showed mesangial expansion and widening of the subendothelial space with a large number of electron-dense deposits. The glomerular lesions might be characteristic of TAFRO syndrome, and were regarded as the main cause of the patient's renal dysfunction.

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Section: Discussionmentioning

confidence: 96%

An autopsy case of TAFRO syndrome with membranoproliferative glomerulonephritis-like lesions

et al. 2018

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“…However, limited information is available about glomerular VEGF expression in TAFRO syndrome. Glomerular VEGF staining in TAFRO syndrome has been reported only in two cases [10, 11]. One was VEGF negative and the other was VEGF positive.…”

Section: Resultsmentioning

confidence: 99%

TAFRO syndrome as a cause of glomerular microangiopathy: a case report and literature review

et al. 2019

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BackgroundTAFRO syndrome is a systemic inflammatory disorder that manifests as thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Renal dysfunction is frequently complicated with TAFRO syndrome, however, it is challenging to perform kidney biopsy in patients with TAFRO syndrome in the presence of thrombocytopenia. Renal histology in TAFRO syndrome mainly shows membranoproliferative glomerulonephritis (MPGN)-like lesions or thrombotic microangiopathy (TMA)-like glomerulopathy. We review our case and previous reports of TAFRO syndrome with kidney biopsy findings and discuss the renal pathophysiology of TAFRO syndrome.Case presentationWe describe a previously healthy 48- year-old woman with TAFRO syndrome. Kidney biopsy performed before the treatment showed diffuse global endocapillary proliferative changes with endothelial cell swelling, double contours of partial capillary walls, and mesangiolysis, consistent with TMA-like glomerulopathy. Glucocorticoid therapy including steroid pulse was ineffective and she developed anasarca, renal dysfunction and oliguria. Hemodialysis was required. However, the anti-Interleukin (IL)-6 receptor antibody (tocilizumab) therapy was very effective. An increase in urinary volume was achieved about 2 weeks after the tocilizumab therapy and hemodialysis was discontinued. To investigate the renal pathophysiology of TAFRO syndrome, we performed immunohistological staining of vascular endothelial growth factor (VEGF)-A, CD34, and D2–40, in our case and a normal control kidney. Glomerular VEGF-A was especially positive in podocytes both, in the control and in the case, with no significant difference and there was a significant increase of VEGF-A staining area in the cortical peritubular capillaries in the case. Both glomerular and renal cortical CD34 expression were significantly decreased in our case. D2–40 expression in cortex was not significantly different.ConclusionsWe reviewed our case and other 10 previous reports about renal biopsy findings in TAFRO syndrome and found that glomerular microangiopathy was a common finding. IL-6-VEGF-axis-induced glomerular microangiopathy may play a crucial role in developing acute kidney injury in TAFRO syndrome and the anti-IL-6 receptor antibody therapy may be useful for TAFRO syndrome refractory to glucocorticoids. About the pathophysiology of VEGF in TAFRO syndrome, VEGF balance in the glomerulus and perhaps in the peritubular capillary system as well may be critical. Further investigation is needed.

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Section: Discussionmentioning

confidence: 99%

“…To our knowledge, biopsy findings of TAFRO syndrome have been reported in 14 cases, all of which, including our cases, consistently demonstrated findings suggesting marked glomerular endothelial cell injury (Table 1). [2][3][4][5][6][7][8][9][10][11][12][13][14][15] Castleman disease and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) syndrome are considered to be similar pathologic entities to TAFRO syndrome, and abnormal secretion and biological actions of IL-6 and VEGF may be involved in the pathogenesis of these diseases. In animal experiments, overproduction of IL-6 induces endothelial cell injury, whereas VEGF is known to increase vascular permeability.…”

Section: Discussionmentioning

confidence: 99%

TAFRO Syndrome With Kidney Involvement: A Case Series of Patients With Kidney Biopsies

et al. 2021

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TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal insufficiency, and organomegaly) syndrome is a systemic inflammatory disease sharing some features with Castleman disease and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome in relation to abnormal secretions of interleukin 6 and vascular endothelial growth factor. The kidney is a main target organ of TAFRO syndrome but the kidney histopathology associated with TAFRO syndrome is yet to be completely defined. We report 3 TAFRO syndrome cases with different clinical courses in which kidney biopsies were performed. In all 3 cases, kidney biopsies showed similar glomerular lesions of diffuse global swelling of the endothelium and expansion of subendothelial spaces, consistent with severe glomerular endothelial injury. Case 3 showed an additional finding of focal tubulointerstitial injury characterized by marked plasma cell infiltration, which was absent in the other 2 cases. Clinical symptoms in cases 1 and 2, which had lower disease severity scores of TAFRO syndrome, were effectively treated with the administration of corticosteroids or a combination of corticosteroids and cyclosporine A. Case 3, with a higher disease severity score, had an aggressive clinical course that was refractory to corticosteroids and tocilizumab; the patient ultimately died of multiple organ failure. In all 3 cases, kidney biopsy provided indications for the diagnosis process and clinical management of TAFRO syndrome.

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Thrombotic microangiopathy on kidney biopsy in a patient with TAFRO syndrome

Cited by 19 publications

References 11 publications

An autopsy case of TAFRO syndrome with membranoproliferative glomerulonephritis-like lesions

An autopsy case of TAFRO syndrome with membranoproliferative glomerulonephritis-like lesions

TAFRO syndrome as a cause of glomerular microangiopathy: a case report and literature review

TAFRO Syndrome With Kidney Involvement: A Case Series of Patients With Kidney Biopsies

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