TY - JOUR
T1 - Exploring the Clinical Diversity of Castleman Disease and TAFRO Syndrome
T2 - A Japanese Multicenter Study on Lymph Node Distribution Patterns
AU - Otsuka, Mizuna
AU - Koga, Tomohiro
AU - Sumiyoshi, Remi
AU - Fukui, Shoichi
AU - Kaneko, Yuko
AU - Shimizu, Takayuki
AU - Katsube, Atsushi
AU - Yano, Shingo
AU - Masaki, Yasufumi
AU - Ide, Makoto
AU - Yoshifuji, Hajime
AU - Kitano, Masayasu
AU - Sato, Yasuharu
AU - Sawa, Naoki
AU - Niiro, Hiroaki
AU - Nakamura, Naoya
AU - Fajgenbaum, David C.
AU - van Rhee, Frits
AU - Kawakami, Atsushi
N1 - Publisher Copyright:
© 2025 The Author(s). American Journal of Hematology published by Wiley Periodicals LLC.
PY - 2025/4
Y1 - 2025/4
N2 - Individuals diagnosed with Castleman disease (CD) and TAFRO syndrome (characterized by thrombocytopenia, anasarca, fever, bone marrow fibrosis, and organomegaly) displays a wide range of clinical symptoms, including varying patterns of lymph node enlargement, systemic inflammation, and impaired organ function. Some patients may present with both CD and TAFRO syndrome concurrently. A retrospective study conducted across multiple centers in Japan examined 321 cases to determine if the quantity and position of swollen lymph nodes could forecast the clinical progression and intensity of these conditions. Interestingly, the study revealed that patients with TAFRO syndrome exhibited lymphadenopathy across all ranges of lymph node region counts. Moreover, no specific clinical patterns were associated with the number of affected lymph node regions in CD patients, regardless of whether they also had TAFRO syndrome. These results enhance our understanding of the clinical variability in CD and TAFRO syndrome, suggesting that a comprehensive clinical evaluation, rather than relying solely on lymph node count, is crucial for effectively managing these conditions. Additional studies are required to establish reliable diagnostic markers and to predict disease severity at the time of diagnosis, ultimately improving patient outcomes.
AB - Individuals diagnosed with Castleman disease (CD) and TAFRO syndrome (characterized by thrombocytopenia, anasarca, fever, bone marrow fibrosis, and organomegaly) displays a wide range of clinical symptoms, including varying patterns of lymph node enlargement, systemic inflammation, and impaired organ function. Some patients may present with both CD and TAFRO syndrome concurrently. A retrospective study conducted across multiple centers in Japan examined 321 cases to determine if the quantity and position of swollen lymph nodes could forecast the clinical progression and intensity of these conditions. Interestingly, the study revealed that patients with TAFRO syndrome exhibited lymphadenopathy across all ranges of lymph node region counts. Moreover, no specific clinical patterns were associated with the number of affected lymph node regions in CD patients, regardless of whether they also had TAFRO syndrome. These results enhance our understanding of the clinical variability in CD and TAFRO syndrome, suggesting that a comprehensive clinical evaluation, rather than relying solely on lymph node count, is crucial for effectively managing these conditions. Additional studies are required to establish reliable diagnostic markers and to predict disease severity at the time of diagnosis, ultimately improving patient outcomes.
KW - Castleman disease
KW - TAFRO syndrome
KW - clinical variability
KW - lymph node distribution
KW - multicenter cohort
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U2 - 10.1002/ajh.27612
DO - 10.1002/ajh.27612
M3 - Article
AN - SCOPUS:85216080754
SN - 0361-8609
VL - 100
SP - 592
EP - 605
JO - American Journal of Hematology
JF - American Journal of Hematology
IS - 4
ER -