Kidney biopsy findings in two patients with TAFRO syndrome: case presentations and review of the literature

Abstract

Background TAFRO syndrome is a clinical subtype of idiopathic multicentric Castleman disease (iMCD) that is characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis (or renal dysfunction), and organomegaly. TAFRO syndrome has only recently been described, and many clinicians are unaware of this disease, leading to delays in diagnosis and treatment. We present two patients with TAFRO syndrome in whom renal biopsies were performed. Case presentation Both patients had subacute onset and exhibited renal insufficiency, edema, anemia, thrombocytopenia, polyserositis and lymphadenopathy over the disease course. However, there were many differences in their clinical manifestations. Case 1 was a 30-year-old woman admitted due to intermittent vaginal bleeding for 3 weeks. Laboratory tests on admission showed severe renal insufficiency (creatinine: 624 μmol/L), severe anemia (Hb: 41 g/L), and moderate thrombocytopenia (61 × 109/L). Case 2 was a 42-year-old man. Acute epigastric pain was his initial complaint, and computed tomography (CT) revealed retroperitoneal exudation around the pancreas. He was diagnosed with acute pancreatitis, and after treatment with a proton pump inhibitor (PPI) and somatostatin, his abdominal pain still recurred. During treatment, renal failure gradually increased, with oliguria, fever, anemia, thrombocytopenia, edema and massive ascites. Lymph node histologies were consistent with the hyaline-vascular (HV) type and mixed type, respectively, and renal histopathologies were consistent with thrombotic microangiopathy (TMA)-like renal lesions and membranoproliferative glomerulonephritis (MPGN), respectively. Their general conditions improved after glucocorticoid therapy, but their renal functions did not recover completely. On the basis of glucocorticoids, second-line treatments with tocilizumab and rituximab, respectively, were applied. Conclusions The diagnosis of TAFRO syndrome is based mainly on clinical manifestations and lymph node biopsies. A reliable early diagnosis and appropriate rapid treatment are essential to improve patient outcomes. Clinicians should deepen their understanding of this disease and similar conditions. Once the disease is suspected, lymph node biopsies should be performed as soon as possible. In addition, renal biopsies should be actively performed in patients with renal involvement.

Investigation of the Effect of Therapeutic Plasma Exchange for TAFRO Syndrome: A Pilot Study

“…Although the pathogenesis of the kidneys' involvement in TAFRO syndrome is not clearly understood, cytokine storms involving IL-6 and VEGF have been suggested to cause immunological disorders and vascular endothelial cell damage. In kidney histopathological examination, TMA-like lesions caused by glomerular endothelial cell damage have been reported in TAFRO syndrome patients [22][23][24]. Although TMA is caused by a variety of etiologies, TPE is commonly introduced when a patient is diagnosed with TMA, often before the underlying etiology is identified.…”

“…Although TMA is caused by a variety of etiologies, TPE is commonly introduced when a patient is diagnosed with TMA, often before the underlying etiology is identified. This requirement is appropriate for many clinical settings [24]. TPE may also be effective in TMA associated with TAFRO syndrome.…”

Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab

“…Given that our patient had a platelet count exceeding 7 × 10 9 /L, renal biopsy was performed without any complications. The main pathological findings of renal involvement in TAFRO syndrome are thrombotic microangiopathy-like glomerulopathy and membranoproliferative glomerulonephritis-like lesions [ 11 , 12 ]. In particular, thrombotic microangiopathy-like glomerulopathy is considered a typical pathological characteristic of acute-phase TAFRO syndrome [ 11 , 12 ].…”

“…The main pathological findings of renal involvement in TAFRO syndrome are thrombotic microangiopathy-like glomerulopathy and membranoproliferative glomerulonephritis-like lesions [ 11 , 12 ]. In particular, thrombotic microangiopathy-like glomerulopathy is considered a typical pathological characteristic of acute-phase TAFRO syndrome [ 11 , 12 ]. According to the pathologist, the renal histology in our patient was consistent with thrombotic microangiopathy-like findings, suggesting that our patient was in the acute phase of TAFRO syndrome.…”

Castleman disease and TAFRO syndrome: To improve the diagnostic consciousness is the key

“…Hence, it is easy to cause misdiagnosis or missed diagnosis. In 2020, we reported two consecutive cases who were diagnosed with TAFRO syndrome according to their findings of lymph node and renal biopsy[ 15 ]. To further understand the clinical features of various types of CD and the internal relationship between TAFRO syndrome and iMCD, we collected CD cases diagnosed and treated in Peking University People's Hospital in the last 5 years and etrospectively analyzed their clinical data.…”