Benefit of prednisolone alone in nodal peripheral T-cell lymphoma with T follicular helper phenotype

Abstract

A 71-year-old Japanese man presented with severe thrombocytopenia. A whole-body CT at presentation showed small cervical, axillary, and para-aortic lymphadenopathy, leading to suspicion of immune thrombocytopenia due to lymphoma. Biopsy was difficult to perform because of severe thrombocytopenia. Thus, he received prednisolone (PSL) therapy and his platelet count gradually recovered. Two and a half years after PSL therapy initiation, his cervical lymphadenopathy slightly progressed without other clinical symptoms. Hence, a biopsy from the left cervical lymph node was performed, and he was diagnosed with nodal peripheral T-cell lymphoma (PTCL) with T follicular helper (TFH) phenotype. Due to various complications, we continued treatment with prednisolone alone after the diagnosis of lymphoma; however, there was no further increase in lymph node enlargement and no other lymphoma-related symptoms for one and a half years after diagnosis. Although immunosuppressive therapy has been reported to produce a response in some patients with angioimmunoblastic T-cell lymphoma, our experience suggests that a similar subset may exist in patients with nodal PTCL with TFH phenotype, which has the same cellular origin. Immunosuppressive therapies may constitute an alternative treatment option even in the era of novel molecular-targeted therapies, especially for elderly patients who are ineligible for chemotherapy.

Keywords: immune thrombocytopenia; nodal peripheral T-cell lymphoma with T follicular helper phenotype; prednisolone.

Fig. 1

A whole-body computed tomography (CT) shows a right retroperitoneal mass without cervical, axillary, and para-aortic lymphadenopathy (A–C; red arrow). A whole-body CT reveals small cervical, axillary, and para-aortic lymphadenopathy (D–F; red arrows). Positron emission tomography-CT taken during slightly progressive cervical lymphadenopathy shows the increased uptake of fluorodeoxyglucose at the same sites (G–I; red arrows). A whole-body CT shows no exacerbation of cervical, axillary, and para-aortic lymphadenopathy for one and a half years after diagnosis of nodal peripheral T-cell lymphoma with T follicular helper phenotype (J–L; red arrows)

Fig. 2

Hematoxylin and eosin (H & E) staining of the left cervical lymph nodes shows the dense proliferation of medium-sized lymphoid cells between enlarged follicles (A, ×400). The medium lymphoid cells are CD4+ (B, ×400), programmed cell death-1+ (C, ×400), and inducible T-cell co-stimulator+ (partially) (D, ×400). The formation of follicular dendritic cell meshwork is limited in follicles on CD21 staining (E, ×20). Epstein–Barr virus-encoded small RNA in situ hybridization reveals scattered positive cells (F, ×400). A pathological review of the CT-guided biopsy specimens from the right retroperitoneal mass reveals a dense infiltrate of small-to-medium-sized lymphocytes with germinal centers on H & E staining (G, ×400). A large number of CD4+ and PD-1+ T-cells were found outside the follicles (H, I, ×400)