Clinical analysis and prognostic significance of lymphoma-associated hemophagocytosis in peripheral T cell lymphoma

Abstract

This study aims to retrospectively analyze the clinical characteristics, treatments, and prognosis of aggressive peripheral T cell lymphoma (PTCL) patients with a lymphoma-associated hemophagocytosis syndrome (LAHS). We compared the clinical features and the overall survival (OS) rates of 159 PTCL patients with and without LAHS as well as the treatment outcomes of these patients with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or intensive chemotherapy regimens. We observed that in 23 % (36/159) patients PTCL was associated with LAHS. Different subtypes of PTCL in LAHS patients were diagnosed and peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) was the main subtype (78 %). The median survival rates of the LAHS and non-LAHS groups were 3 and 16 months, respectively. The elevated rates of serum β2-microglobulin, ferritin, fasting triglycerides, and hypofibrinogen levels were higher in the LAHS group, so were bone marrow involvement, liver dysfunction, hepatosplenomegaly, and B symptoms. Three patients who were treated with a plasma exchange had a longer survival time. There was no statistically significant difference in the OS rates between the intensive chemotherapy and CHOP regimen groups (P > 0.05). PTCL patients with LAHS had a poorer prognosis. Awareness of the clinical symptoms and laboratory findings are crucial in order to diagnose LAHS in an early stage and repeated biopsies of multiple bone marrows from different locations in those patients without enlargement of superficial lymph nodes are necessary to improve the diagnosis. Intensive chemotherapy due to its severe toxicity was not obviously advantageous for the OS rate compared to the CHOP regimen.

Serum ferritin level is a prognostic marker in patients with peripheral T‐cell lymphoma

“…In addition, our study was limited by its retrospective design and heterogeneous treatment methods that were used. Besides, patients with PTCL occasionally complicate lymphoma‐associated hemophagocytosis (LAHS) and have extremely hyperferritinemia with poor outcomes . Unfortunately, central pathological reviews were not performed and we have no data about the presence of hemophagocytic syndrome at diagnosis.…”

Hemophagocytic Lymphohistiocytosis Secondary to Diffuse B-Cell Lymphoma

“…LAHS is most commonly associated with T-cell or natural killer (NK) cell lymphoma (T/NK-LAHS). This association is well established in both eastern and western populations [4][5][6]. T/NK-LAHS is an aggressive disease with a poor prognosis and a mean survival of about 3 months despite treatment with intensive chemotherapy [5].…”

Lymphoma Presenting as Secondary HLH: A Review With a Tale of Two Cases

“…Hence, the greater mortality rate for LAHS patients cannot be attributed to the delay of lymphoma diagnosis and treatment alone. In a retrospective review of 159 cases of PTCL, 36 patients developed LAHS and had significantly worse outcomes compared with those who did not develop LAHS, 23 the most common lymphoproliferative malignancy associated with HLH and lymphoma or leukemia of peripheral T-cell or NK-cell lineages. 24–26 Cutaneous manifestations such as panniculitis in patients with HLH should raise the suspicion of an underlying T-cell lymphoma.…”

“…46 In a study by Xie et al, of 159 PTCL patients, patients with LAHS who received intensive chemotherapy appeared to have an improved overall survival, however, it was not statistically significant compared with CHOP therapy alone. 23 Allogeneic stem cell transplantation has shown promising results in children with a 3-year survival rate as high as 70% with primary HLH. 47,48 However, data for stem cell transplant in adults is extremely limited and should largely be based on underlying etiology.…”