Successful allogeneic bone
marrow transplantation for hepatosplenic
gd
T cell lymphoma
Jun Ooi, Tohru Iseki, Daiki Adachi, Takayuki Yamashita, Akira
Tomonari, Arinobu Tojo, Kenzaburo Tani and Shigetaka Asano
Department of Hematology and Oncology, Institute of Medical
Science, University of Tokyo, Tokyo, Japan
Correspondence: Jun Ooi, M.D. Department of Hematology and
Oncology, Institute of Medical Science, University of Tokyo, 4-6-1
Shirokanedai, Minato-ku, Tokyo 108-8639, Japan. Tel:
81-3-5449-5543 Fax: 81-3-5449-5429. E-mail: jun-ooi@ims.u-tokyo.ac.jp
[when
available]
Abstract
Hepatosplenic
gd
T cell lymphoma is generally very refractory to regular
chemotherapy, and to date, no curative treatment modality for
hepatosplenic gd
T cell lymphoma has been established. We report a case of
hepatosplenic gd
T cell lymphoma successfully treated with allogeneic stem cell
transplantation (SCT). The patient was a 23-year-old male who was
admitted to our hospital in June 1999 and subsequently diagnosed
with hepatosplenic gd
T cell lymphoma. After two courses of chemotherapy, a complete
response was achieved and he subsequently received with
fractionated TBI (12 Gy) and high-dose etoposide (60 mg/kg)
followed by allogeneic bone marrow transplantation from his
HLA-matched younger sister. During the follow up period, grade II
acute GVHD and extensive chronic GVHD occurred but both were
successfully treated with a dose modification of cyclosporine
(CyA). At a follow-up period of 23 months, the patient is alive
and free of disease. Hepatosplenic gd
T cell lymphoma is a malignant lymphoma characterized by a
predominance in young adult males, an extranodal presentation with
marked hepatosplenomegaly, the expression of the T cell receptor
(TCR) gd
chain, and an aggressive course of disease. Since Farcet et al.
reported two cases, approximately 60 cases have been
described.1-7 Although many treatment approaches have
been reported, the long-term prognosis of patients with
hepatosplenic gd
T cell lymphoma is poor, with a reported survival of approximately
8 months.3 We report here a case of hepatosplenic
gd
T cell lymphoma that was successfully treated using allogeneic
stem cell transplantation (SCT). The patient was a 23-year-old
male who was admitted to our hospital in June 1999 with abdominal
distension, malaise, and night sweats. On physical examination
hepatosplenomegaly was noted and some superficial lymph nodes with
normal physiological size and texture were palpable. The serum
lactate dehydrogenase (LDH) level was elevated (1801 IU/l), and a
complete blood count revealed pancytopenia with a white blood cell
count of 1.15x109/L (atypical lymphoid cell 28.5%),
hemoglobin 9.0 g/dl and platelets 27x109/l. Bone marrow
aspirate showed normocellularity composed of 64.5% of atypical
lymphoid cells. The phenotypic analysis showed that the peripheral
and bone marrow atypical lymphoid cells were positive for CD2,
CD3, CD7, CD8, CD16, CD56, HLA-DR and
TCRgd
and negative for CD4, CD5, CD10, CD13, CD20, CD33, CD34 and TCRab.
According to previously established criteria,1-3 the
patient was diagnosed with hepatosplenic
gd
T cell lymphoma with bone marrow involvement and two courses of
conventional chemotherapy were administered according to B-NHL86
protocol.9 After the two courses of chemotherapy, the
patient achieved a complete response and since previous reports
showed that relapses were common and occurred shortly after
chemotherapy in hepatosplenic gd
T cell lymphoma patients, allogeneic SCT from his HLA-matched
younger sister was performed. The conditioning regimen included
four fractionated 12 Gy TBI on days -9 and -8 and high-dose
etoposide (60 mg/kg), which was administered as 24 h continuous
intravenous (i.v.) infusion on day -4. After the conditioning, a
total of 1.3x108/kg unmanipulated bone marrow nucleated
cells were infused. GVHD prophylaxis consisted of cyclosporine
(CyA) (3 mg/kg once daily i.v. infusion over 10 h) and short-term
methotrexate (15 mg/m2 i.v. on day 1, 10
mg/m2 on days 3 and 6). G-CSF (5 mg/kg/day) was
administered by i.v. infusion starting on day 1 until durable
granulocyte recovery was achieved. Neutrophil and platelet
recovery was achieved on days 17 and 33, respectively. During the
follow up period, grade II acute GVHD and extensive chronic GVHD
occurred but both were successfully treated with a dose
modification of CyA. At a follow-up period of 23 months, the
patient is alive and free of disease.
The long-term disease-free survival (DFS) rate for patients with
hepatosplenic gd
T cell lymphoma is poor. The lack of durable remission after
conventional chemotherapy may result in a poor rate of DFS, and to
date, the optimal therapy for patients of hepatosplenic
gd
T cell lymphoma remains to be determined. To our knowledge, 7
cases including our case have been treated with allogeneic SCT
(Table
1),2-7 of
which 3 of 7 were alive and free of disease at the time writing in
each report. Although the effectiveness of allogeneic SCT for
hepatosplenic gd
T cell lymphoma patients has not been established, based on the
prognosis in this patient, we suggest that prompt allogeneic SCT
after diagnosis may be beneficial.
Acknowledgements : The authors would like to thank the physicians and nurses who cared for the patient and donor.
