title: Successful treatment of a Caucasian case of multifocal Castleman's disease with TAFRO syndrome with a pathophysiology targeted therapy - a case report.
abstract: BACKGROUND
  Castleman-Kojima disease (TAFRO Syndrome) is characterized by Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly, multiple lymphadenopathy and histopathology pattern of atypical Castleman's disease (CD). Only few cases of this recently identified unique variant of Multicentric CD (MCD) are described in literature, all Japanese. It therefore poses serious diagnostic and therapeutic challenges.
  
  
  METHODS
  We describe a 21 year old woman with fever, asthenia, bilateral pleural effusion, ascites, hypoalbuminemia, severe thrombocytopenia, anemia, renal failure and proteinuria, whereas microbiological tests, immune serology (except ANA) and bone marrow biopsy were all negative. A CT-scan showed multiple lymphadenopathy and tissue samplings of mediastinal lymph nodes was compatible with a mixed-type CD. The diagnosis of MCD with TAFRO syndrome was made, but after an initial improvement with high dose corticosteroid therapy, clinical and laboratory features worsened. Based upon the high serum IL-6 levels and the high number of CD20-lymphocytes in lymph nodes tissue, we started tocilizumab (partial benefit), followed by rituximab combined with CVP (cyclophosphamide, vincristine and prednisone) chemotherapy, achieving a complete response. A total of six cycles of R-CVP were administered monthly, followed by maintenance with monthly rituximab. A complete remission persists at the 12th month of follow-up.
  
  
  CONCLUSIONS
  In patients with massive immune system activation and lymphadenopathy it is mandatory to rule out Castleman-Kojima disease. In our patient a therapy aimed at the prominent pathophysiological abnormalities has been successful so far. However, since the rarity of TAFRO Syndrome, a multicenter registry is strongly desirable for a better understanding of the disease mechanisms, hopefully leading to evidence-based therapeutic choices.
authors: Clinica Medica - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Via Conca 71, 60126 Ancona, Italy.
authors: Silvia
authors: S
authors: Tedesco
authors: Clinica Medica - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Via Conca 71, 60126 Ancona, Italy.
authors: Laura
authors: L
authors: Postacchini
authors: Clinica Medica - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Via Conca 71, 60126 Ancona, Italy.
authors: Lucia
authors: L
authors: Manfredi
authors: Anatomia Patologica - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Via Conca 71, 60126 Ancona, Italy.
authors: Gaia
authors: G
authors: Goteri
authors: Clinica Medica - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Via Conca 71, 60126 Ancona, Italy ; Clinica Medica - Ospedali Riuniti di Ancona, Via Conca 71, 60126 Ancona, Italy.
authors: Michele M
authors: MM
authors: Luchetti
authors: Clinica Medica - Ospedali Riuniti di Ancona, Via Conca 71, 60126 Ancona, Italy.
authors: Antonella
authors: A
authors: Festa
authors: Clinica Medica - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Via Conca 71, 60126 Ancona, Italy ; Clinica Medica - Ospedali Riuniti di Ancona, Via Conca 71, 60126 Ancona, Italy.
authors: Armando
authors: A
authors: Gabrielli
authors: Clinica Medica - Ospedali Riuniti di Ancona, Via Conca 71, 60126 Ancona, Italy.
authors: Giovanni
authors: G
authors: Pomponio
keywords: Castleman’s disease; Chemotherapy; Multicentric; PRES; Rituximab; TAFRO syndrome; Tocilizumab