PMID- 30680669
OWN - NLM
STAT- MEDLINE
DCOM- 20191114
LR  - 20200225
IS  - 1865-3774 (Electronic)
IS  - 0925-5710 (Linking)
VI  - 109
IP  - 6
DP  - 2019 Jun
TI  - Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia 
      A and development of cardiopulmonary arrest that were successfully treated with 
      VA-ECMO and tocilizumab.
PG  - 737-743
LID - 10.1007/s12185-019-02604-2 [doi]
AB  - TAFRO syndrome and acquired hemophilia A (AHA) are rare, life-threatening 
      diseases; however, the relationship between these two diseases is unknown. A 
      25-year-old man was transferred to our hospital because of bleeding tendency 
      accompanied by multiple organ failure with generalized edema, massive pleural 
      effusion, and ascites. He was diagnosed with AHA. Bypass therapy for hemostasis 
      and cyclophosphamide with prednisolone to eradicate possible inhibitors were 
      provided. However, he suffered from cardiopulmonary arrest. Veno-arterial 
      extracorporeal membrane oxygenation (VA-ECMO) was initiated as rescue therapy. 
      His hemodynamic status stabilized and he was weaned from VA-ECMO in 1 week. We 
      confirmed normal FVIII activity and disappearance of the inhibitor, and bypass 
      therapy was discontinued. However, generalized edema with massive ascites, 
      pleural effusion, and renal insufficiency persisted. Bone marrow biopsy showed 
      reticulin fibrosis. These symptoms fulfilled the diagnostic criteria of TAFRO 
      syndrome. He received tocilizumab (TCZ) and steroid was tapered off. After four 
      cycles of TCZ, symptoms of TAFRO syndrome gradually improved. To the best of our 
      knowledge, this is the first report of TAFRO syndrome accompanied by AHA with 
      rescue by VA-ECMO. Additionally, AHA and TAFRO syndrome were well controlled by 
      TCZ.
FAU - Suzuki, Kei
AU  - Suzuki K
AUID- ORCID: 0000-0002-2227-4737
AD  - Department of Hematology and Oncology, Mie University Graduate School of 
      Medicine, 2-174, Edobashi, Tsu, Mie, 514-8507, Japan. 
      keis@clin.medic.mie-u.ac.jp.
AD  - The Emergency and Critical Care Center, Mie University Hospital, 2-174, Edobashi, 
      Tsu, Mie, 514-8507, Japan. keis@clin.medic.mie-u.ac.jp.
FAU - Matsumoto, Takeshi
AU  - Matsumoto T
AD  - Department of Hematology and Oncology, Mie University Graduate School of 
      Medicine, 2-174, Edobashi, Tsu, Mie, 514-8507, Japan.
FAU - Iwashita, Yoshiaki
AU  - Iwashita Y
AD  - The Emergency and Critical Care Center, Mie University Hospital, 2-174, Edobashi, 
      Tsu, Mie, 514-8507, Japan.
FAU - Ishikura, Ken
AU  - Ishikura K
AD  - The Emergency and Critical Care Center, Mie University Hospital, 2-174, Edobashi, 
      Tsu, Mie, 514-8507, Japan.
FAU - Fujioka, Masaki
AU  - Fujioka M
AD  - The Emergency and Critical Care Center, Mie University Hospital, 2-174, Edobashi, 
      Tsu, Mie, 514-8507, Japan.
FAU - Wada, Hideo
AU  - Wada H
AD  - Department of Molecular and Laboratory Medicine, Mie University Graduate School 
      of Medicine, 2-174, Edobashi, Tsu, Mie, 514-8507, Japan.
FAU - Katayama, Naoyuki
AU  - Katayama N
AD  - Department of Hematology and Oncology, Mie University Graduate School of 
      Medicine, 2-174, Edobashi, Tsu, Mie, 514-8507, Japan.
FAU - Imai, Hiroshi
AU  - Imai H
AD  - The Emergency and Critical Care Center, Mie University Hospital, 2-174, Edobashi, 
      Tsu, Mie, 514-8507, Japan.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20190124
PL  - Japan
TA  - Int J Hematol
JT  - International journal of hematology
JID - 9111627
RN  - 0 (Antibodies, Monoclonal, Humanized)
RN  - I031V2H011 (tocilizumab)
RN  - Factor 8 deficiency, acquired
RN  - Multi-centric Castleman's Disease
SB  - IM
MH  - Adult
MH  - Antibodies, Monoclonal, Humanized/*therapeutic use
MH  - Castleman Disease/*complications/drug therapy/*therapy
MH  - Extracorporeal Membrane Oxygenation/*methods
MH  - Heart Arrest/*etiology/*therapy
MH  - Hemophilia A/*complications/therapy
MH  - Humans
MH  - Male
MH  - Treatment Outcome
OTO - NOTNLM
OT  - Acquired hemophilia A
OT  - Extracorporeal life support
OT  - TAFRO syndrome
OT  - Tocilizumab
EDAT- 2019/01/27 06:00
MHDA- 2019/11/15 06:00
CRDT- 2019/01/26 06:00
PHST- 2018/10/10 00:00 [received]
PHST- 2019/01/17 00:00 [accepted]
PHST- 2019/01/16 00:00 [revised]
PHST- 2019/01/27 06:00 [pubmed]
PHST- 2019/11/15 06:00 [medline]
PHST- 2019/01/26 06:00 [entrez]
AID - 10.1007/s12185-019-02604-2 [pii]
AID - 10.1007/s12185-019-02604-2 [doi]
PST - ppublish
SO  - Int J Hematol. 2019 Jun;109(6):737-743. doi: 10.1007/s12185-019-02604-2. Epub 
      2019 Jan 24.