Successful Treatment of a Patient with Multicentric Castleman's Disease who Presented with Thrombocytopenia, Ascites, Renal Failure and Myelofibrosis Using Tocilizumab, an Anti-Interleukin-6 Receptor Antibody

Kawabata, Hiroshi; Kotani, Shunsuke; Matsumura, Yasuhiro; Kondo, Tadakazu; Katsurada, Tatsuya; Haga, Hironori; Kadowaki, Norimitsu; Takaori‐Kondo, Akifumi

doi:10.2169/internalmedicine.52.9482

Cited by 66 publications

(43 citation statements)

References 15 publications

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“…It is thought that the thrombocytopenia seen in TAFRO syndrome might be caused by an immune-mediated mechanism and can be overcome by anti-inflammatory therapy [2-4]. The mechanism of renal failure in patients with TAFRO syndrome is not clear because histological examinations of kidneys have not been reported in this disease.…”

Section: Discussionmentioning

confidence: 99%

The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman’s disease

et al. 2014

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BackgroundTAFRO syndrome is a unique clinicopathologic variant of multicentric Castleman’s disease that has recently been identified in Japan. It is characterized by a constellation of symptoms: Thrombocytopenia, Anasarca, reticulin Fibrosis of the bone marrow, Renal dysfunction and Organomegaly (TAFRO). Previous reports have shown that affected patients usually respond to immunosuppressive therapy, but the disease sometimes has a fatal course. TAFRO syndrome occurs in the middle-aged and elderly and there are no prior reports of the disease in adolescents. Here we report the first adolescent case, successfully treated with anti-IL-6 receptor antibody (tocilizumab, TCZ) and monitored with serial cytokine profiles.Case presentationA 15-year-old Japanese boy was referred to us with fever of unknown origin. Whole body computed tomography demonstrated systemic lymphadenopathy, organomegaly and anasarca. Laboratory tests showed elevated C-reactive protein and hypoproteinemia. Bone marrow biopsy revealed a hyperplastic marrow with megakaryocytic hyperplasia and mild reticulin fibrosis. Despite methylprednisolone pulse therapy, the disease progressed markedly to respiratory distress, acute renal failure, anemia and thrombocytopenia. Serum and plasma levels of cytokines, including IL-6, vascular endothelial growth factor, neopterin and soluble tumor necrosis factor receptors I and II, were markedly elevated. Repeated weekly TCZ administration dramatically improved the patient’s symptoms and laboratory tests showed decreasing cytokine levels.ConclusionTo our knowledge, this is the first report of TAFRO syndrome in a young patient, suggesting that this disease can occur even in adolescence. The patient was successfully treated with TCZ. During our patient’s clinical course, monitoring cytokine profiles was useful to assess the disease activity of TAFRO syndrome.

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Section: Discussionmentioning

confidence: 99%

The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman’s disease

et al. 2014

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“…The patient was treated by hemodialysis and was unable to take medicine orally, so we chose TCZ as a second-line treatment about 40 days after stopping steroid. In previous cases of successful TCZ treatment, steroid were used with TCZ [2,7]. This is thus the first report of successful treatment of TAFRO syndrome using only TCZ.…”

Section: Discussionmentioning

confidence: 80%

“…Our case thus represented a very rare case of successful treatment for grade 5 TAFRO syndrome. Some patients with TAFRO syndrome have been successfully treated with steroid, immunosuppressants, including cyclosporine A (CyA), TCZ and rituximab, and/or immunomodulatory drugs like thalidomide [2][3][4][5][6]. According to the 2015 treatment strategy for TAFRO syndrome, steroid is the first-line treatment, with CyA as the second-line therapy.…”

Section: Discussionmentioning

confidence: 99%

Successful treatment by tocilizumab without steroid in a very severe case of TAFRO syndrome

et al. 2017

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A 59-year-old Japanese man was admitted to our hospital with leg edema, fatigue and fever. He had a past medical history of duodenal ulcer, fatty liver, hyperuricemia and positive rheumatoid factor, but was not receiving any medication. His son had died 2 years prior to this admission after following a clinical course that suggested TAFRO syndrome.The patient showed mild fever (about 37-38 °C), polypnea and severe leg edema on admission. Chest examination revealed decreased breath sounds in the right lung. No superficial lymph nodes were palpable. Laboratory tests on admission revealed mild thrombocytopenia, hypoproteinemia, elevation of C-reactive protein (CRP), alkaline phosphatase (ALP), creatinine (Cr), and urine N-acetyl-β-d-glucosaminidase (NAG) ( Table 1). Urine testing showed granular casts without proteinuria or hematuria. Concentrations of aspartate aminotransferase (AST) and alanine transaminase (ALT) were elevated, but unchanged compared with the results of a medical check 6 months before admission, and so were attributed to fatty liver. Chest X-ray and computed tomography (CT) showed mild lymphadenopathy in the mediastinum, bilateral pleural effusion and ascites (Fig. 1).Blood, sputum and urine culture tests were all negative and no other infectious diseases were present. Negative results were obtained for all autoantibodies. Among the tumor markers, only soluble interleukin-2 receptor (sIL-2R) was elevated. Bone marrow biopsy was performed to rule out malignant lymphoma, revealing normocellular Abstract Successful use of tocilizumab (TCZ) to treat TAFRO syndrome has recently been reported. In those cases, TCZ was used with steroid. We present herein the case of a 59-year-old man with very severe TAFRO syndrome who was successfully treated using TCZ without steroid. He showed rapidly progressive anasarca, acute renal failure and very severe thrombocytopenia. We initially used steroid, but its efficacy was limited. Moreover, steroid use had to be stopped as soon as possible, because hemorrhagic shock developed due to severe duodenal ulcer. After overcoming infections (about 40 days after stopping steroid), administration of TCZ was started and the patient was discharged in clinical remission.

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“…It is interesting that the levels of IL-6 and VEGF in the ascitic fluid of patients were markedly higher than the levels in the serum and plasma [7].…”

mentioning

confidence: 95%

“…In several reported MCD cases tocilizumab was very effective: patients achieved a complete remission and the treatment was discontinued often without disease recurrence [7,14].…”

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confidence: 99%

Castleman–Kojima disease (TAFRO syndrome) in a Caucasian patient: A rare case report and review of the literature

Allegra

Rotondo

Russo

et al. 2015

Blood Cells, Molecules, and Diseases

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Successful Treatment of a Patient with Multicentric Castleman's Disease who Presented with Thrombocytopenia, Ascites, Renal Failure and Myelofibrosis Using Tocilizumab, an Anti-Interleukin-6 Receptor Antibody

Cited by 66 publications

References 15 publications

The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman’s disease

The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman’s disease

Successful treatment by tocilizumab without steroid in a very severe case of TAFRO syndrome

Castleman–Kojima disease (TAFRO syndrome) in a Caucasian patient: A rare case report and review of the literature

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