Castleman disease and TAFRO syndrome: To improve the diagnostic consciousness is the key.

Abstract

BACKGROUNDCastleman disease (CD) and TAFRO syndrome are very rare in clinical practice. Most clinicians, especially non-hematological clinicians, do not know enough about the two diseases, so it often leads to misdiagnosis or missed diagnosis.AIMTo explore the clinical features and diagnosis of CD and TAFRO syndrome.METHODSWe retrospectively collected the clinical and laboratory data of 39 patients who were diagnosed with CD from a single medical center.RESULTSClinical classification identified 18 patients (46.15%) with unicentric Castleman disease (UCD) and 21 patients (53.85%) with multicentric Castleman disease (MCD), the latter is further divided into 13 patients (33.33%) with idiopathic multicentric Castleman disease-not otherwise specified (iMCD-NOS) and 8 patients (20.51%) with TAFRO syndrome. UCD and iMCD are significantly different in clinical manifestations, treatment, and prognosis. However, a few patients with MCD were diagnosed as UCD in their early stage. There was a correlation between two of Thrombocytopenia, anasarca and elevated creatinine, which were important components of TAFRO syndrome. In UCD group, the pathologies of lymph modes were mostly hyaline vascular type (13/18, 72.22%), however plasma cell type or mixed type could also appear. In iMCD-NOS group and TAFRO syndrome group, the pathologies of lymph mode shown polarity of plasma cell type and hyaline vascular type respectively. Compared with patients with TAFRO syndrome, patients with iMCD-NOS were diagnosed more difficultly.CONCLUSIONThe clinical and pathological types of CD are not completely separate, there is an intermediate situation or mixed characteristics between two ends of clinical and pathological types. The clinical manifestations of patients with CD are determined by their pathological type. TAFRO syndrome is a special subtype of iMCD with unique clinical manifestations.