Clinical characteristics of T-cell lymphoma associated with hemophagocytic syndrome: Comparison of T-cell lymphoma with and without hemophagocytic syndrome

Abstract

T-cell lymphoma-associated hemophagocytic syndrome (T-LAHS) has been frequently reported in Asian countries and is considered with extremely poor prognosis. To summarize its clinical characteristics and explore its early diagnosis and treatment, we retrospectively analyzed the records of 113 patients with aggressive T cell lymphoma, of which 28 were associated with LAHS. According to WHO classification (2001), 22 cases were classified into peripheral T-cell lymphoma (unspecified), 2 into extranodal NK/T-cell lymphoma, and 4 into systemic anaplastic large cell lymphoma. The median survivals of the LAHS and no-LAHS groups were 40 days and 8 months, respectively. The elevating rates of serum lactate dehydrogenase (LDH) (100% vs. 55%), ferritin (100% vs. 64%), fasting triglycerides (79% vs. 43%), and hypofibrinogen (43% vs. 14%) levels were higher in the LAHS group than in the no-LAHS group (P < 0.05), so were bone marrow involvement (57% vs. 32%, P < 0.05) and liver dysfunction (40% vs. 13%, P < 0.05). Eleven of the 28 LAHS patients did not receive any chemotherapy, and 14 received CHOP regimen as initial chemotherapy. Three patients in critical conditions were given plasma exchange and gained the chance of initial chemotherapy. We suggest that in patients presenting with fever, hepatosplenomegaly, cytopenia, and constantly increasing levels of serum LDH, CA125, ferritin, transglutaminase, and beta2-microglobulin, T-LAHS should be taken into account. Repeating biopsies of multiple parts of bone marrow may help diagnosis. The therapeutic result of chemotherapy alone or combined for T-LAHS was discouraging and the survival time of most cases was no more than 1 year. Plasmapheresis as initial therapy is worth considering in critical cases.

L-Asparaginase-Induced Complete Response in a Relapsed Patient with Epstein-Barr Virus and Cytotoxic Peripheral T-Cell Lymphoma Not Otherwise Specified

“…There have been previous reports about prognostic factors for PTCL-NOS, including the presence of EBV (9), the expression of cytotoxic molecules such as granzyme B and TIA-1 (10), and hemophagocytic syndrome complications (11). The present patient demonstrated some adverse prognostic factors, including EBV positivity, cytotoxic molecule expression, and multiple drug resistance.…”

Hemophagocyte Lymphohistiocytosis Secondary to Bilateral Epididymal Diffuse Large B-Cell Lymphoma

“…In Japan, the annual incidence of HLH was estimated as 1 in 800,000 per year (primarily the acquired form) [3]. HLH was the most common in NK/ T cell lymphoma (i.e., up to 24.7 %) [10]. The mechanism of triggering HLH in lymphoma has not been fully elucidated, but a mutation of the perforin gene is partly responsible [11,12].…”

“…The prognosis of HLH secondary to lymphoma is unfavorable, and the median survival time is less than 40 days [2,10]. Importantly, survival has been shown to be dependent on the type of lymphoma.…”

Hemophagocytic Lymphohistiocytosis in B-Cell Lymphoproliferative Disorder: Report of a Rare Association

“…A study of T-cell lymphoma-associated HLH revealed elevated lactate dehydrogenase, ferritin, triglycerides and hypofibrinogenemia in the group with HLH as compared to those without HLH. This study showed a significantly worse outcome in T-cell lymphoma with HLH (median survival 40 days vs 8 months in group without HLH) [4].…”

“…The clinical features and diagnostic criteria of acquired HLH are similar to the familial form, except for the family history. Of the lymphomas, T-cell lymphoma and Hodgkin lymphomas have been reported frequently with HLH [3,4]. T-cell lymphoma-associated HLH usually involves extra-nodal sites like nasal, cutaneous or hepatosplenic locations.…”