Abstract
T-cell lymphoma is the special malignant type of non-Hodgkin’s lymphoma. The diagnosis and the treatment were usually troublesome for physician in clinical practice. We retrospectively reviewed 63 cases of T-cell lymphomas from 360 cases of lymphomas in our hospital during the period from January 2000 to July 2006. This study is to determine the clinicopathological characteristics of T cell lymphomas. The patients were reclassified according to the World Health Organization classification system. Clinical data, including age, gender, clinical staging, and follow-up, were scrutinized.
The median follow-up duration was 5 months (range 21days to 36 months). There were slightly more males than females (36 versus 27), and the median age at the onset were 40 years (range 13 to 77 years). The major subtype was peripheral T-cell lymphoma, which accounted for 78% (49/63). Besides, there were 5 cases of anaplastic T large cell lymphoma, 3 lymphoblastic lymphoma, 2 T/NK-cell lymphoma, 2 angioimmunoblastic lymphoma, 1 mycosis fungoides and 1 pre-T cell lymphoma.
The most common manifestation was fever, which accounted for 60% (38/63). 27% (17/63) patients presented with obvious enlargement of lymphonodes. Other manifestation included skin rash or phymata, pruritus, jaundice, abdominal pain, rhinorrhagia, puffiness, diarrhea, hoarseness and ulcus. Interestingly, we found that only 32% obvious enlarged lymphonodes could be confirmed by physical examination, hepatomegaly 33% and Splenomegaly 44% respectively.
Besides, there were several significant laboratory findings: 40% cases had cytopenia of at least 2 cell lines, 68% had high level of LDH, 70% had elevated β2-microglobulin and 68% were detected T-cell receptor (TCR) and immunoglobulin heavy chain (IgH) gene rearrangement. Furthermore, 53% (33/63) patients had bone marrow involvement at the onset and 27% were diagnosed only by bone marrow biopsy.
We also observed 20 cases of lymphoma associated hemophagocytic syndromes (LAHS). The median age for this disease was 37 year. The median life span was 39 days (range 21days to 10 months). The initial manifestations included fever (19/20), splenohepatomegaly (18/20), and cytopenias in all patients. Only 15% patients had enlargement of lymphonodes, which was suggested to be infrequent in LAHS. Immatural T-cell infiltration in bone marrow was detected in 75% (15/20) cases.
Chromosome disorder of [der(21)(p11), −22] was detected in 3 cases. We also found that 2 cases which underwent plasmapheresis got much better after chemotherapy. 19 cases were under our follow-up. 17 patients could not survival longer than 6 months. The 6-month overall survival (OS) for LAHS was merely 2 of all 20. Furthermore, nobody survived more than 1 year, which indicated the poor prognosis of LAHS.
There were 11 out of 63 cases had received trial chemotherapy including liposomal Doxorubicin, L-asparaginase, velcade, autologous bone marrow transplantation, or plasmapheresis before chemotherapy. The median survival time prolonged obviously from 2 months up to 8 months, which suggested the encouraging efficiency of these methods.
Distinct Features of Angioimmunoblastic T-Cell Lymphoma With Bone Marrow Involvement