Clinical characteristics of T-cell lymphoma associated with hemophagocytic syndrome: Comparison of T-cell lymphoma with and without hemophagocytic syndrome
- 1 January 2008
- journal article
- research article
- Published by Taylor & Francis Ltd in Leukemia & Lymphoma
- Vol. 49 (1), 81-87
- https://doi.org/10.1080/10428190701713630
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.Keywords
This publication has 18 references indexed in Scilit:
- A Case Report of the Effect of Plasma Exchange on Reactive Hemophagocytic Syndrome Associated with Toxic Shock SyndromeTherapeutic Apheresis and Dialysis, 2004
- Serum CA125: A Tumor Marker for Monitoring Response to Treatment and Follow-up in Patients with Non-Hodgkin's LymphomaThe Oncologist, 2004
- Hepatosplenic T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patientsBlood, 2003
- Modern management of children with haemophagocytic lymphohistiocytosisBritish Journal of Haematology, 2003
- Peripheral T-cell lymphoma associated consecutively with hemophagocytic lymphohistiocytosis and hypereosinophilic syndromeEuropean Journal of Haematology, 2003
- Plasmapheresis for the treatment of acute pancreatitis induced by hemophagocytic syndrome related to hypertriglyceridemiaJournal of Clinical Apheresis, 2003
- Peripheral T-cell lymphoma (excluding anaplastic large-cell lymphoma): results from the Non-Hodgkin’s Lymphoma Classification ProjectAnnals of Oncology, 2002
- Clinical Characteristics of B-cell Lymphoma-associated Hemophagocytic Syndrome (B-LAHS): Comparison of CD5+ with CD5- B-LAHS.Internal Medicine, 2001
- Haemophagocytosis in Bone Marrow Aspirate – A Review of the Clinical Course of 10 CasesActa Haematologica, 1995
- A Predictive Model for Aggressive Non-Hodgkin's LymphomaThe New England Journal of Medicine, 1993