Atypical Hyaline Vascular-Type Castleman's Disease With Thrombocytopenia, Anasarca, Fever, and Systemic Lymphadenopathy

Abstract

Recently, atypical Castleman's disease (CD) was reported in Japan. This disease is considered as TAFRO syndrome or non-idiopathic plasmacytic lymphadenopathy (IPL), a constellation of clinical symptoms, namely, thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly without hyper-γ-globulinemia. Histopathologically, this disease is similar to hyaline vascular (HV)-type CD. Here, we present a 43-year-old Japanese woman meeting the clinical criteria of TAFRO syndrome who was successfully treated with combined corticosteroid therapy. She showed a rapidly progressive course of thrombocytopenia, systemic lymphadenopathy, fever, anasarca, and increase in acute inflammatory proteins without hyper-γ-globulinemia. Lymph node biopsy was performed and revealed HV-type CD without human herpes virus 8 infection, which was clinicopathologically compatible with non-IPL. The association of these atypical features with well-known multicentric Castleman's disease (MCD), namely, HV-type histology with systemic lymphadenopathy, marked thrombocytopenia even with a high level of interleukin-6, and increased acute inflammatory proteins without hyper-γ-globulinemia, suggests that TAFRO syndrome as presented in our case is a novel entity, which may have been diagnosed as MCD in the past. To define this novel entity more clearly and to demonstrate its etiology, further nationwide surveys of this syndrome and MCD are needed.

TAFRO Syndrome with an Anterior Mediastinal Mass and Lethal Autoantibody-Mediated Thrombocytopenia: An Autopsy Case Report

“…Typically, thrombocytopenia is attributed to either decreased platelet production or increased platelet destruction. Previous studies reported 11 cases of TAFRO syndrome and elevated antiplatelet antibodies (Table 1) [5, 6, 11-18]. Similar to our case, anti-GPIIb/IIIa antibody was positive in 1 case [5] and anti-HLA class I antibody was positive in 2 cases [12].…”

The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman’s disease

“…It is interesting that the levels of IL-6 and VEGF in the ascitic fluid of our patient were markedly higher than the levels in his serum and plasma. One previous case has been reported of a patient with severe anasarca and markedly elevated IL-6, suggesting systemic inflammation of the serosa [3]. …”

“…There are no adolescent cases reported to date, and this is the first case of a young patient with an apparent diagnosis of TAFRO syndrome. All patients were treated with steroids and some improved with the addition of cyclosporine A [2,6] or TCZ [3,4] with rituximab therapy [3]. Unfortunately, three of the eight patients died [5,6], indicating that this syndrome sometimes results in a fatal outcome in spite of treatment.…”

International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease

“…11,14,[16][17][18][19][20] iMCD patients without TAFRO syndrome typically have thrombocytosis, hypergammaglobulinemia, and less severe fluid accumulation. This non-TAFRO group has been called idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia or IPL-type.…”