Am J Hematol: Is TAFRO syndrome a subtype of iexclusivecoman multicenter Castleman disease?

!---- Castleman disease (CD) is a rare lymphatic hyperpluse disease that can be single-centered or multi-central.multi-central CD (MCD) can also be subdivided into human herpes virus type 8-related, POEMS-related and imcable (iMCD).TAFRO syndrome is a newly discovered disease with unknown etiology characterized by platelet reduction, anorexia, fever, mesh protein bone marrow fibrosis, renal dysfunction and organ swelling.TAFRO syndrome is sometimes considered to be a subtype of iMCD (TAFRO-iMCD), while iMCD without TAFRO syndrome is considered 'not specified' (iMCD-NOS).However, some patients with TAFRO syndrome were diagnosed with iMCD without a lymph node biopsy (TAFRO syndrome was not confirmed as iMCD; TAFRO-w/op-iMCD).To clarify the clinical characteristics of iMCD-NOS, TAFRO-iMCD, and TAFRO-w/op-iMCD, we reviewed 220 patients, including 87 cases of iMCD-NOS, 63 tAFRO-op-w/iMCD patients, extracted from a multicenter collaborative retrospective research database that established the tAFRO syndrome concept.all three groups showed elevated levels of anemia, hypoalbuminemia, serum C reactive protein and interleukin-6. there was no significant difference in clinical, laboratory, and prognosis characteristics between theTAFRO-iMCD, and the TAFRO-w/op-iMCD group.but the iMCD-NOS group is characterized by polyclonal high-globulinemia. the five-year survival rates of patients in the iMCD-NOS and TAFRO participant groups were 100% and 66.5%, respectively,the iMCD-NOS group (the latter significantly decreased in the previous months).iMCD-NOS and TAFRO-iMCD samples are typically characterized by plasma cells and hybrid histology, respectively., iMCD can therefore be divided into two different subtypes, iMCD-NOS and TAFRO-iMCD., TAFRO-iMCD and TAFRO-w/op-iMCD can be considered the same entity and require timely diagnosis and intensive care..