scholarly journals Acute Kidney Injury Caused by TAFRO Syndrome in a Chinese Patient: Efficacy of Long-Term Corticosteroids Combined with Bortezomib and Cyclophosphamide

2020 ◽  
Vol 45 (4) ◽  
pp. 623-630 ◽  
Author(s):  
Peng Xia ◽  
Lu Zhang ◽  
Menglian Zou ◽  
Tengyue Zhang ◽  
Ran Li ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Poor Prognosis ◽  
Renal Involvement ◽  
Castleman Disease ◽  
High Dose ◽  
Axillary Lymph ◽  
Tafro Syndrome ◽  
Vascular Type

Introduction: Thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a newly recognized and rare clinical subtype of Castleman disease. Renal involvement in TAFRO syndrome usually presents with mild proteinuria, microscopic hematuria, and acute renal injury requiring temporary renal replacement. There is no standard therapy available and treatment failures are common, leading to a poor prognosis. We report a case of acute renal failure caused by TAFRO syndrome, successfully managed by long-term corticosteroids combined with bortezomib and cyclophosphamide. Case Presentation: The patient was a 52-year-old female who presented with fever, anasarca, oliguria, and abdominal distension at first. She progressed rapidly to anuric renal failure requiring hemodialysis. She also demonstrated thrombocytopenia, anemia, coagulopathy, and a hyperinflammatory status. Her CT scan showed severe polyserositis, splenomegaly, and lymphadenopathy. Her serum vascular epithelial growth factor level was significantly elevated. Axillary lymph node biopsy showed hyaline-vascular type Castleman disease, supporting the diagnosis of TAFRO syndrome. Her renal function recovered after high-dose steroids and supportive treatment. A weekly dosing regimen of bortezomib, cyclophosphamide, and dexamethasone combined with medium dose prednisone in between were deployed. Her blood cell count and renal function remained stable after 6 months. The inflammation was suppressed and the polyserositis resolved completely. Conclusion: TAFRO syndrome is rare and has a poor prognosis due to the lack of standard treatment. Our patient might be the first TAFRO case successfully treated by bortezomib, cyclophosphamide, and corticosteroids.

scholarly journals Renal involvement in sarcoidosis: Histologic findings and clinical course

2019 ◽  
Vol 5 ◽  
pp. 205930071983492 ◽  
Author(s):  
Shirin Naderi ◽  
Kerstin Amann ◽  
Ulf Janssen
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Interstitial Nephritis ◽  
Clinical Course ◽  
Renal Involvement ◽  
Response To Treatment ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Common Diagnosis

Background: Renal failure in sarcoidosis is rare and data on its long-term outcome are scarce. Aim: To investigate the pattern of renal involvement in sarcoidosis, its clinical course and response to treatment in the long-term. Methods: A single-center retrospective study with review of renal biopsies and medical charts was performed. Results: Between January 2005 and December 2016, seven patients with sarcoidosis underwent a kidney biopsy. This is equivalent to a frequency of 1.6% in a total of 434 biopsies from native kidney performed in our institution. All patients presented with renal failure. Five patients had granulomatous interstitial nephritis (GIN) and one patient each interstitial nephritis without granuloma and nephrocalcinosis. Three patients had concomitant glomerular disease: IgA nephropathy (n = 2), membranous and focal proliferative glomerulonephritis (n = 1). Most patients (n = 5) presented with hypercalcemia. All patients initially received oral prednisolone 1 mg/kg/day (n = 3) or 0.5 mg/kg/day (n = 4), respectively, with subsequent tapering or suspension. One patient was started on azathioprine after 18 months to spare steroids. After a mean follow-up of 59 months mean estimated glomerular filtration rate (eGFR) had improved from 19 ± 7 at presentation to 49 ± 16 mL/min. No patient required dialysis. All patients started on prednisolone 1 mg/kg/day developed transient diabetes mellitus while patients on 0.5 mg/kg/day did not. Renal function improvement did not differ between both treatment groups. Conclusion: GIN was the most common diagnosis in sarcoidosis patients with renal failure. Initial hypercalcemia was observed in the majority. Early steroid treatment lead to sustained renal function improvement.

scholarly journals AB0750 FUNCTIONAL CONDITION OF KIDNEYS IN THE LONG-TERM COURSE OF SLE IN ADOLESCENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1403.2-1403
Author(s):  
L. Bohmat ◽  
N. Shevchenko ◽  
I. Bessonova
Keyword(s):  
Renal Function ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Clinical Analysis ◽  
Average Dose ◽  
One Year

Background:Lupus nephritis is the most severe and adverse systemic lupus erythematosus (SLE) syndrome. According to modern recommendations, the clinical manifestations of active nephritis should be taken under medical control in 6 months after the start of the disease’s treatment1.Objectives:The aim of this study was to examine the functional status of the kidneys in children with SLE in the course of the disease for more than one year.Methods:The analysis included case histories of 43 patients with SLE, mostly females (41), aged 7 to 18 years (mean age 14.4 years) with disease duration of 4.75 ± 0.58 years of whom 22 were less than three years, 21 - more than three years. All children received corticosteroid therapy, at the time of the examination the average dose was 13.85 ± 1.86 mg per day in terms of prednisolone. The second component of therapy was azathioprine (average dose 97.61 ± 2.11 mg). All children received hydroxychloroquine (5 mg/kg per day).To determine the functional state of the kidneys a clinical analysis of urine, a study of the scope of specific gravity of urine during the day (Zymnytsky test), the content of creatinine and urea in serum to determine the glomerular filtration rate (GFR), the level of microalbuminuria per day were evaluated.Results:Renal involvement in the developed SLE occurred in 73.08% of patients. Among them, therapy during the first 6 months was considered quite effective in 58.06% of patients. It was found that in children with disease duration from one to three years proteinuria was registered in 68.18%, a decrease in GFR in 4.45% and hyperfiltration in 9.09%. In the group of children with duration of SLE more than three years revealed deeper changes in renal function; there was proteinuria in 90.47%, the frequency of GFR decreased was in 19.04%, a decrease of renal concentration function was in 14.28% of cases.Indicators of renal function in children with SLE depending on the duration of the disease (M ± m)IndicatorDuration of the diseasefrom 1 year to 3 years n = 22over 3 yearsn = 21Creatinine, mmol/l0,080 ± 0,0140,090 ± 0,018Мочевина, mmol/l5,66 ± 1,425,63 ± 1,61GFR, ml/min117,05 ± 19,68100,20 ± 18,98 *Microalbuminuria, mg/day24,41 ± 13,1334,73 ± 24,76Density min1,007 ± 0,0051,006 ± 0,005Density max1,024 ± 0,0051,019 ± 0,005 ***р<0,03;**р<0,01 the probability of differences when comparing between groupsConclusion:Long-term follow-up of children with SLE over one year reveals a prolongation of renal dysfunction, which worsens after three years, and is the basis for the development of irreversible renal impairment.References:[1]European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative /Noortje Groot, Nienke de Graeff, Stephen D Marks et all. //Ann Rheum Dis. 2017 Dec;76(12):1965-1973.Disclosure of Interests:None declared

scholarly journals Renal function following acute renal failure in childhood: A long term follow-up study

1989 ◽  
Vol 35 (1) ◽  
pp. 84-89 ◽  
Author(s):  
Helen N. Georgaki-Angelaki ◽  
David B. Steed ◽  
Cyril Chantler ◽  
George B. Haycock
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Acute Renal Failure ◽  
Follow Up Study ◽  
Long Term Follow Up

TAFRO syndrome: A case report with biopsy findings from three sites

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S151-S152
Author(s):  
N C Jadhav ◽  
J Freeman
Keyword(s):  
Case Report ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Ventilatory Support ◽  
Bone Marrow Examination ◽  
Castleman Disease ◽  
High Dose ◽  
Inflammatory Disorder ◽  
Cervical Lymph Nodes ◽  
Tafro Syndrome ◽  
Organ Systems

Abstract Introduction/Objective TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) is a rare systemic inflammatory disorder. First reported in 2010, the majority of cases are from Japan, where it is currently regarded as a subtype of HHV8-negative Multicentric Castleman Disease. Methods/Case Report We report a case of TAFRO occurring in a 17-year old from Puerto Rico. She originally presented with abdominal pain, vomiting, and fever, and was also found to have splenomegaly, elevated sedimentation rate and C-reactive protein. Over the course of the next several days she developed respiratory distress, requiring ventilatory support, and anuric renal failure. Renal biopsy demonstrated evidence of thrombotic microangiopathy, which was interpreted as atypical hemolytic syndrome, requiring dialysis and treated with eculizumab. Because other diagnostic considerations at the time also included hemophagocytic lymphohistiocytosis and catastrophic antiphospholipid syndrome, bone marrow examination was performed which demonstrated increased megakaryocytes, without fibrosis or hemophagocytosis. Over the next two months, she developed anasarca with ascites, pleural effusion, pericardial effusion, multiple enlarged axillary and cervical lymph nodes, and persistence of splenomegaly. Biopsy of a left cervical lymph node demonstrated features suggestive of Castlemans disease, plasma cell variant. The constellation of symptoms, laboratory investigations and biopsy diagnoses support the diagnosis of TAFRO syndrome. The patient received rituximab, high dose steroids, and hemodialysis with clinical improvement. At 16 months follow up, the patient is in remission. Results (if a Case Study enter NA) NA Conclusion From a pathology standpoint, the case provides biopsy findings of three distinct organ systems all with relatively unusual findings that together are characteristic of TAFRO syndrome. It highlights the risk of accepting “biopsy proven” diagnoses such as atypical hemolytic uremic syndrome that also require significant laboratory and clinical correlation for true confirmation, and points out the importance and opportunity for pathologists to see the forest along with the trees in order to recognize rare diseases such as TAFRO.

scholarly journals Renal function at hospital admission as a prognostic factor in adult hemolytic uremic syndrome. The Italian Registry of Haemolytic Uremic Syndrome.

1992 ◽  
Vol 2 (11) ◽  
pp. 1640-1644 ◽  
Author(s):  
A Schieppati ◽  
P Ruggenenti ◽  
R P Cornejo ◽  
F Ferrario ◽  
G Gregorini ◽  
...  
Keyword(s):  
Renal Function ◽  
Hemolytic Uremic Syndrome ◽  
Acute Phase ◽  
Renal Involvement ◽  
Laboratory Data ◽  
Elevated Serum ◽  
Long Term Outcome ◽  
Uremic Syndrome ◽  
Clinical Records

The clinical records of adult patients with a diagnosis of hemolytic uremic syndrome were retrospectively reviewed with the aim of evaluating the long-term outcome of renal function. The setting is the Italian Registry of Haemolytic Uraemic Syndrome, with which 13 Nephrology Centers have participated. Clinical and laboratory data of 43 patients with hemolytic uremic syndrome were evaluated. The mean age at onset was 34.3 +/- 18.3 yr. Men and women were equally affected. No seasonal trend in presentation was observed. In 20 patients, hemolytic uremic syndrome was primitive, whereas in 23, it was associated with another disease (cancer, preeclampsia, malignant hypertension, vasculitides). Gastrointestinal symptoms were the most frequently observed prodromes. Thirty (70%) patients required dialysis during the acute phase of the disease. Six patients died during the acute phase of the disease, and one died later after discharge (overall mortality, 16%). After 1 yr of follow-up, 11 (26%) patients had recovered a normal renal function, 14 (33%) had hypertension and/or renal insufficiency, and 11 (26%) were on regular dialysis. When prognostic factors of survival and recovery of renal function were considered, it was found that older age was associated with higher mortality in the acute phase, whereas severe renal involvement at the onset of the disease (as expressed by elevated serum creatinine) was associated with a long-term unfavorable prognosis.

scholarly journals An Unusual Case of Bilateral Adrenal Hyperplasia

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A116-A116
Author(s):  
Alicia De Castro ◽  
Richard J Comi ◽  
Andrew Robert Crawford ◽  
Natasa Radovanovic ◽  
Hima Ammana ◽  
...  
Keyword(s):  
Renal Failure ◽  
Abdominal Pain ◽  
Muscle Weakness ◽  
High Dose ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Adrenal Hyperplasia ◽  
Tafro Syndrome ◽  
Hypokalemic Alkalosis ◽  
Bilateral Adrenal Hyperplasia

Abstract Background: Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly (TAFRO) syndrome is a variant of Castleman Disease, which is a rare lymphoproliferative disease that can be life threatening. Diagnosis is often delayed because of its nonspecific presentation. Bilateral adrenal hyperplasia has been a reported complication, however the majority of cases reported have been in Asian patients. Prior accounts of elevated ACTH in TAFRO have been in the context of adrenal insufficiency. Clinical Case: A 28-year-old Caucasian male with a history of multiple sclerosis was seen in the ED with abdominal pain. On presentation, he was afebrile and normotensive. Physical exam was notable for cervical lymphadenopathy and abdominal tenderness. There was no facial rounding/plethora, bruising, abnormal striae, or proximal muscle weakness. He had normal blood counts, serum chemistry and liver function. An abdominal CT scan showed marked bilateral adrenal hyperplasia with pre-aortic, peri-aortic and retroperitoneal lymphadenopathy. An 8AM serum cortisol was 14.1 mcg/dl (4.8–19.6 mcg/dl) and adrenocorticotrophic hormone (ACTH) was elevated at 152 pg/ml (7.2–63 pg/ml). A repeat serum 8AM cortisol following low dose dexamethasone suppression test (LDDST) was 14.7 mcg/dl, however at that point the patient had developed new fevers and thrombocytopenia. Blood pressure, blood glucose and potassium remained normal. An MRI of the brain showed a normal appearing pituitary gland. An extensive infectious and rheumatologic evaluation was negative, and he underwent an inguinal lymph node biopsy which showed nodal expansion with histiocytes, consistent with TAFRO. High dose methylprednisolone and Siltuximab (an IL-6 inhibitor) were started, and his fever and abdominal pain resolved. He was discharged home on oral prednisone. Conclusion: We describe a case of bilateral adrenal hyperplasia with elevated ACTH and non-suppressed cortisol on LDDST suggestive of ACTH-driven cortisol excess. However, interpretation of his LDDST is made difficult in the context of persistent fevers. Although we cannot definitively exclude pathologic hypercortisolism at this time, given his lack of suggestive features such as proximal muscle weakness, abnormal striae or hypokalemic alkalosis, his over-all presentation was more consistent with hyperplasia secondary to TAFRO rather than an underlying pathologic hypercortisolism. Adrenal hyperplasia has been noted in TAFRO, however its pathogenesis remains poorly understood. TAFRO should be added among the differentials for bilateral adrenal hyperplasia to facilitate early diagnosis and treatment. References: Ducoux G, et al. Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with Bilateral Adrenal Hemorrhage in Two Caucasian Patients. Am J Case Rep. 2020;21:e919536.

scholarly journals TAFRO syndrome with skin manifestations treated with bortezomib and tocilizumab; a case report

2019 ◽  
Vol 6 (1) ◽  
pp. e01-e01
Author(s):  
Seyedeh Tahereh Mohaddes ◽  
Zahra Rezaei Borojerdi ◽  
Maryam Miri ◽  
Mohammad Moeini Nodeh ◽  
Alireza Rezaei ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Renal Failure ◽  
Case Reports ◽  
Abdominal Distension ◽  
Skin Lesions ◽  
Castleman Disease ◽  
Tafro Syndrome ◽  
Multicentric Castleman Disease ◽  
Bone Marrow Specimen ◽  
Mesangioproliferative Glomerulonephritis

TAFRO syndrome is a new presentation of idiopathic multicentric Castleman disease which is termed as thrombocytopenia, anasarca, myelofibrosis, renal failure and organomegaly (TAFRO). The exact pathophysiology of TAFRO syndrome is unclear and management is mostly based on case reports and expert opinion. In this report, a 37 years old male patient with TAFRO syndrome is discussed. The patient was referred with fever, sweating, anorexia, abdominal distension and generalized edema which has been hospitalized multiple times for such complaints. The patient also developed skin lesions dispersed in red nodules, which was reported as "granuloid hemangioma". Renal biopsy suggested mesangioproliferative glomerulonephritis and bone marrow specimen showed hypercellular active marrow with reticulin fibrosis. The lymph node biopsies were reported as Castleman disease. This report demonstrates that different manifestations of TAFRO syndrome may overlap with other syndromes and can be managed by Bortezomib and Tocilizumab.

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

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Qianyun Zhou ◽  
Yuanyuan Zhang ◽  
Guangping Zhou ◽  
Jihong Zhu
Keyword(s):  
Lymph Node ◽  
Renal Insufficiency ◽  
Epigastric Pain ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Castleman Disease ◽  
Tafro Syndrome ◽  
Multicentric Castleman Disease ◽  
Renal Biopsies ◽  
Case Presentations

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.

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