A life-threatening case of TAFRO syndrome with dramatic response to tocilizumab, rituximab, and pulse steroids: The first case report in Latin America.
|Abstract||This is the report of the first case of TAFRO syndrome (Thrombocytopenia, Anasarca, myelofibrosis, Renal dysfunction, Organomegaly) in Latin America.|
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.
|Publication Year Start||2017-01-01|
PMID- 28353560 OWN - NLM STAT- MEDLINE DA - 20170329 DCOM- 20170406 LR - 20170413 IS - 1536-5964 (Electronic) IS - 0025-7974 (Linking) VI - 96 IP - 13 DP - 2017 Mar TI - A life-threatening case of TAFRO syndrome with dramatic response to tocilizumab, rituximab, and pulse steroids: The first case report in Latin America. PG - e6271 LID - 10.1097/MD.0000000000006271 [doi] AB - RATIONALE: This is the report of the first case of TAFRO syndrome (Thrombocytopenia, Anasarca, myelofibrosis, Renal dysfunction, Organomegaly) in Latin America. PATIENT CONCERNS: The patient was a 61-year-old white woman of Ashkenazi Jewish descent, who presented with a history of 8 days of nausea, vomiting, and fever; severe pitting edema in both legs, ascites, splenomegaly, and palpable axillary lymph nodes. DIAGNOSES: Abdominal computed tomography (CT) showed bilateral pleural effusion and retroperitoneal lymph node enlargement. INTERVENTIONS: Anasarca and worsening of renal function led to admission to the intensive care unit (ICU) with multiple organ failure, requiring mechanical ventilation, vasopressor medications, and continuous renal replacement therapy (CRRT). Diagnosis of TAFRO syndrome was made on day 18 after admission, based on clinical findings and results of bone marrow and lymph node biopsies. She was treated with methylprednisolone, tocilizumab, and rituximab. One week after the first tocilizumab dose, she had dramatic improvements in respiratory and hemodynamic status, and was weaned from ventilator support and vasopressor medications. OUTCOMES: After 2 weeks of therapy, CRRT was switched to intermittent hemodialysis. On day 46, the patient was discharged from the ICU to the general ward, and 3 months after admission, she went home. LESSONS: Provided the interleukin-6 measurement is available, this approach is suggested in cases of TAFRO syndrome, in order to customize the treatment. FAU - Jose, Fabio Freire AU - Jose FF AD - aDepartment of Rheumatology bDepartment of Hematology cDepartment of Pathology, Hospital Israelita Albert Einstein dDepartment of Pathology, Faculdade de Medicina da Universidade de Sao Paulo eDepartment of Nuclear Medicine fDepartment of Internal Medicine gDepartment of Hematology, Hospital Israelita Albert Einstein, Sao Paulo, SP, Brazil. FAU - Kerbauy, Lucila Nassif AU - Kerbauy LN FAU - Perini, Guilherme Fleury AU - Perini GF FAU - Blumenschein, Danielle Isadora AU - Blumenschein DI FAU - Pasqualin, Denise da Cunha AU - Pasqualin DD FAU - Malheiros, Denise Maria Avancini Costa AU - Malheiros DM FAU - Campos Neto, Guilherme de Carvalho AU - Campos Neto GC FAU - de Souza Santos, Fabio Pires AU - de Souza Santos FP FAU - Piovesan, Ronaldo AU - Piovesan R FAU - Hamerschlak, Nelson AU - Hamerschlak N LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Immunologic Factors) RN - 4F4X42SYQ6 (Rituximab) RN - I031V2H011 (tocilizumab) RN - X4W7ZR7023 (Methylprednisolone) RN - Multi-centric Castleman's Disease SB - AIM SB - IM MH - Anti-Inflammatory Agents/*administration & dosage MH - Antibodies, Monoclonal, Humanized/*administration & dosage MH - Drug Therapy, Combination MH - Female MH - Giant Lymph Node Hyperplasia/*drug therapy MH - Humans MH - Immunologic Factors/*administration & dosage MH - Methylprednisolone/*administration & dosage MH - Middle Aged MH - Rituximab/*administration & dosage PMC - PMC5380244 EDAT- 2017/03/30 06:00 MHDA- 2017/04/07 06:00 CRDT- 2017/03/30 06:00 AID - 10.1097/MD.0000000000006271 [doi] AID - 00005792-201703310-00006 [pii] PST - ppublish SO - Medicine (Baltimore). 2017 Mar;96(13):e6271. doi: 10.1097/MD.0000000000006271.
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