Late Relapses Characterize Autologous Transplantation (ASCT) in First Complete Remission (CR) for Peripheral T-Cell Lymphoma (PTCL).

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 5110-5110 ◽  
Author(s):  
Tarun Kewalramani ◽  
Steve Horwitz ◽  
Andrew D. Zelenetz ◽  
Stephen D. Nimer ◽  
Craig H. Moskowitz
Keyword(s):  
T Cell ◽  
Median Time ◽  
Progressive Disease ◽  
Cell Lymphoma ◽  
Autologous Transplantation ◽  
Conditioning Regimen ◽  
Allogeneic Transplantation ◽  
T Cell Lymphoma ◽  
Time To Progression

With the exception of ALK-positive anaplastic large cell lymphoma (ALCL), standard-dose chemotherapy is curative in a minority of patients (pts) with PTCL, and most pts have progressive disease less than 2 years from completing treatment. Several studies suggest that ASCT in 1st CR significantly improves the short-term outcome of pts with PTCL, but its long-term efficacy is not known. To address this, we assessed the outcome of sequential patients who underwent ASCT in 1st CR (n=15). Histologic subtypes were PTCL, unspecified, in 6 pts, angioimmunoblastic T-cell lymphoma in 5 pts, ALK-negative ALCL in 3 pts and hepatosplenic gamma delta T-cell lymphoma in 1 pt. Induction chemotherapy was CHOP (n=2) or CHOP-ICE hybrid (n=12) in 93% of pts. The age-adjusted IPI (AAIPI) was 2–3 in 9 of 14 assessable patients (64%), and 11 pts (73%) had stage III–IV disease. The conditioning regimen consisted of BEAM or CBV in 10 pts and TBI/Cy/VP-16 in 5 pts. All patients received peripheral blood progenitor cells for hematopoietic support. The median follow-up of all patients is 24 months (range 4.5–70). Five pts (33%) have progressed, with a median time to progression of 50 months (range 10–70). Four of the 5 pts who progressed did so more than 2 years from ASCT; they comprise 57% of patients with more than 2-years of follow-up. Four of 5 patients with progressive disease have died, with a median time from progression to death of 1 month (0.6–14.6). In this small series the AAIPI was not predictive of PFS or OS. While our results confirm the that ASCT in 1st CR significantly delays the time to progression, they suggest that it may not be curative in the majority of patients. If confirmed in ongoing larger prospective studies, this observation warrants trials of post-ASCT maintenance treatment and, for younger patients, trials of allogeneic transplantation in 1st CR or sequential ASCT followed by allogeneic transplantation. Figure Figure

Autologous Stem Cell Transplantation with Benda-BEAM (Bendamustine, Etoposide, Cytarabine, Melphalan) in Aggressive Non Hodgkin and Hodgkin´s Lymphoma

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 3982-3982 ◽  
Author(s):  
Thomas Noesslinger ◽  
Michaela Moestl ◽  
Christoph Tinchon ◽  
Elisabeth Koller ◽  
Werner Linkesch ◽  
...  
Keyword(s):  
Stem Cell ◽  
T Cell ◽  
Stem Cell Transplantation ◽  
Mantle Cell Lymphoma ◽  
Median Time ◽  
Autologous Stem Cell Transplantation ◽  
Cell Lymphoma ◽  
Conditioning Regimen ◽  
T Cell Lymphoma ◽  
Mantle Cell

Abstract Autologous Stem Cell Transplantation (ASCT) is standard of care in relapsed diffuse large B-cell lymphoma (DLBCL) and other lymphoproliferative disorders (relapsed Hodgkin´s disease, mantle or T-cell lymphoma). BCNU, Etoposide, Ara-C, Melphalan (BEAM) is a standard conditioning regimen, but BCNU is associated with interstitial pneumonia (range 2 to 20%) and a increased risk of death compared with busulfan or TBI based regimens. Therefore a less toxic regimen might improve the results in (relapsed) lymphoma patients. Bendamustine showed promising results in B- and T-cell lymphoma and dose escalation is safe and feasible. Here we report promising results with bendamustine replacing BCNU in the BEAM regimen described as Benda-BEAM, recently published in a phase two dose finding study (Visani, Blood 2011). Thirty-eight patients with Hodgkin´s (HL)(n=9) or Non-Hodgkin (n=29) lymphoma were consecutively treated with Benda-BEAM (bendamustine on two consecutive days at a dose of 200 mg/m2per day). Ten patients were diagnosed with DLBCL, also ten patients with mantle cell lymphoma, four patients with an anaplastic T-cell lymphoma, four patients with follicular lymphoma and one patient with an greyzone lymphoma. Twenty-four patients were male and fourteen female with a median age of 52 years (range 22-71) and 25% were above the age of sixty. The median lines of previous therapies were 2 (range: 1-4). 36 patients were treated with Benda-BEAM and 2 patients with mantle cell lymphoma received additionally Zevalin. All patients had chemosensitive disease and before transplantation 31 patients (82%) were in complete (CR) and 7 (18%) in partial remission (PR). A median number of 4,10*106 CD34+ cells/kg (range: 1,60-11,10) were infused. All patients showed engraftment with a median time to achieve an absolute neutrophil count > 1*109/L of 10 days (range 7-13) and to platelets >20*109/L of 11 days (range 5-26). The median time of fever was 6 days (range: 0 -22). The most common grade 3 and 4 toxicity during the whole treatment period were diarrhoea (n=10), mucositis (n=7) and febrile neutropenia (n=6), followed by nausea (n=4) and cardiologic toxicities (n=3). There were no pulmonary toxicities observed and no transplant related mortality occurred. After a median follow-up of 22 months, thirty-three patients were evaluable for response, with 21 patients (64%) still in CR, while 12 patients (36%) showed progression after a median time of 6 months after transplantation (range 2-22 months), Until today seven patients (21%) have died (5 DLBCL, 1 HL, 1 mantle cell lymphoma), all due to lymphoma progression. The 1-year PFS is 72% and the 1-year OVS 85%. Thus Benda-BEAM seems to be feasible with a promising response rate und a randomized phase II trial comparing Benda-BEAM with BEAM is planned. Disclosures Off Label Use: bendamustine as part of conditioning regimen before autologous stem cell transplanatation.

scholarly journals Oral Clofarabine for Relapsed/Refractory Non-Hodgkin Lymphomas: Final Results from a Phase 1-2 Trial

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4423-4423
Author(s):  
Jeremy S. Abramson ◽  
Eric D. Jacobsen ◽  
Robert Allyn Redd ◽  
Tak Takvorian ◽  
David C. Fisher ◽  
...  
Keyword(s):  
T Cell ◽  
Progressive Disease ◽  
Response Rate ◽  
Cell Lymphoma ◽  
Phase 1 ◽  
Median Number ◽  
T Cell Lymphoma ◽  
Phase 2 ◽  
Grade 3

Abstract Background: Clofarabine is a second-generation purine analogue FDA-approved as an intravenous formulation for relapsed/refractory pediatric ALL. Purine analogues demonstrate significant clinical activity in non-Hodgkin lymphomas (NHL). Clofarabine may offer pharmacologic advantages over other nucleoside analogues including being a more efficient substrate for deoxycytidine kinase, more completely inhibiting ribonucleotide reductase and DNA polymerase α, and demonstrating improved activity in cells that are non-dividing or have a low proliferation rate. This phase 1-2 trial studied an oral formulation of clofarabine in relapsed or refractory NHL. Methods: Patients were eligible if they had relapsed or refractory NHL of any histologic subtype. All pts were required to have adequate organ function and performance status ≤2 as well as absence of CNS involvement. Patients were treated at 4 dose levels (1mg, 2mg, 4mg and ultimately 3mg) with oral clofarabine administered once daily on days 1-21 of a 28 day cycle for up to 6 cycles. Three to 6 pts were treated at each dose level in a traditional 3+3 design followed by a 10 patient dose expansion at the recommended phase 2 dose (RP2D). The phase 1 portion of this study has been published (Leuk Lymph 2013; 45:1915-1920). Phase 2 was designed to enroll 24 additional subjects. The primary endpoint in phase 2 was overall response rate (ORR). Secondary endpoints were progression-free survival (PFS), overall survival (OS), and safety. A total of 50 patients were accrued on the phase 1-2 trial; 31 subjects were treated in phase 1 and 19 in phase 2. Phase 2 accrual was stopped prematurely due to discontinuation of the drug formulation used in the study. All patients treated at the RP2D (n=36) are included in the phase 2 efficacy analysis since there were no differences in treatment or follow-up for these patients. Results: The median age for all patients was 69 years (range 45-92). Eighty-two percent had advanced stage at study entry. The median number of prior regimens was 2 (range 1-7) and 4 patients had prior auto stem cell transplant. Histologies included follicular lymphoma (FL, 13 pts), small lymphocytic lymphoma (SLL, 8 pts), diffuse large B-cell lymphoma (DLBCL, 6 pts), marginal zone lymphoma (MZL, 11 pts), mantle cell lymphoma (MCL, 9 pts), T-cell lymphoma (TCL, 2 pts) and lymphoplasmacytic lymphoma (LPL 1 pt). The 3mg dose was declared the RP2D, as previously reported. The most common toxicities were anemia (78%), leukopenia (66%), neutropenia (64%), thrombocytopenia (62%) and fatigue (60%). Twenty-nine patients (58%) experienced at least one grade 3-4 toxicity. The most common grade 3-4 toxicities were leukopenia and neutropenia (48%), thrombocytopenia (30%), anemia (14%) and fatigue (6%). There were 2 deaths on study, both considered unrelated to study drug (cardiac arrest, progressive disease). The median number of cycles administered was 4, and 18 patients (36%) completed all 6 cycles of therapy. The most common reasons for discontinuing therapy were progressive disease (34%) and toxicity (16%). Of 50 patients on study, the ORR was 28% (95% CI: 16 - 42%) with complete response rate (CRR) of 10% (95% CI: 3 - 22%). An additional 36% had stable disease (SD). By histology, responses were seen in 5/11 MZL, 4/9 MCL, 3/8 SLL, 3/13 FL, and 1/1 LPL. No responses were observed in DLBCL or TCL, although an angioimmunoblastic T-cell lymphoma patient had SD with a 42% reduction in tumor volume, and a mycosis fungoides patient had significant reduction in cutaneous disease burden. Among 36 patients treated at the RP2D and included in the phase 2 analysis, the ORR was 28% (95% CI: 14 - 45%) with CRR of 8% (95% CI: 2 - 22%), and 44% of patients with SD. A higher proportion of patients treated at a non-RP2D experienced progressive disease on study (43% vs. 28% in the RP2D cohort). The median PFS was 5.5 months, and the one- and two- year PFS were 32% (95% CI: 20%, 45%) and 16% (95% CI: 7.5%, 27%), respectively. The median duration of follow-up was 3.8 years, with 26 patients alive and 22 deceased at last follow-up; two patients were lost to follow-up. The median OS was not reached, and the 3 year OS was 58% (95% CI: 43%, 71%). Conclusion: Oral clofarabine is generally well tolerated and produces disease control in a substantial proportion of patients with relapsed/refractory NHL, particularly in indolent histologies and MCL. Disclosures Abramson: Sanofi: Consultancy. Off Label Use: Clofarabine is not FDA-approved for non-Hodgkin lymphoma. Brown:Sanofi: Consultancy.

scholarly journals Long-Term Efficacy of Psoralen Plus Ultraviolet a Irradiation and Low-Dose Interferon-Alpha Therapy According to Conventional and Emerging Clinical Endopoints of Early Stage Cutaneous T Cell Lymphoma

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2462-2462
Author(s):  
Serena Rupoli ◽  
Gaia Goteri ◽  
Erika Morsia ◽  
Elena Torre ◽  
Kimberly Blaine Garvey ◽  
...  
Keyword(s):  
T Cell ◽  
Median Time ◽  
Partial Remission ◽  
Low Dose ◽  
Cell Lymphoma ◽  
Early Stage ◽  
T Cell Lymphoma ◽  
Cutaneous T Cell Lymphoma ◽  
Ultraviolet A

Abstract Introduction: Patients with early stage Cutaneous T cell Lymphoma (CTCL) usually have a benign and chronic disease course. Refractoriness under skin directed therapies and/or more extensive disease pose some therapeutic changes. Using the combination of psoralen plus ultraviolet A irradiation (PUVA) and low-dose Interferon-α (INF), the principal treatment goal is to keep confined the disease to the skin, preventing disease progression. Methods: We carry out a prospective data on 87 patients with early stage IA to IIA MF treated with low-dose IFN-α2b and PUVA, enrolled from 1997 to 2010. We collected data regarding clinical characteristics of MF, efficacy and outcome. Subcutaneous IFN-α2b was administered 1.5 MU/day during the first week; in the second week the dose was increased to 3MU/day. PUVA irradiation was started on the 3th week with IFN-α2b 3 MU 3 times weekly until CR, of for a maximum of 2 months. During maintenance therapy, IFN-α2b was scheduled for 3 MU 3 times weekly for 2 months and subsequently 3 MU 2 times weekly for 10 months and PUVA was gradually reduced every 2 months over a period of 12 months. Diagnostic, risk and response assignments were according to EORTC criteria. Results: Patient characteristics at time diagnosis, staging, response rates and overall outcome are shown in Table 1. Among the 87 patients, overall response rate (ORR) was 97.8% (n=85) and included complete remission (CR) in 70 patients (80.5%), very good partial remission in 5 patients (5.8%) and partial remission (PR) in another 10 (11.5%). The best response to therapy was seen after a median of 5 months (range, 1-30) and the 74.3% of patients who achieved a CR after induction therapy kept the complete response at the last follow up. Among the responders, 40 (47.1%) relapsed with minor event with in median time of 21 months (range, 0-71) and 7(8.2%) relapsed with major event in a median time of 6 months (range, 1-81). After a median follow up of 207 months (range, 6-295), 25 (28.7) patients died, only 1 for progressive disease. Median overall survival (OS) for our cohort was not reached (95% CI; 235-NR months) and median time to next treatment (TTNT) was 38.5 months (95% CI, 33-46 months). Moreover, disease free survival (DFS) in CR patients was 210 months (95% CI; 200-226 months). Conclusions: The long follow up of this study verifies our preliminary results and confirms the efficacy of INF-PUVA combination therapy in a real world setting, according conventional (OS and DFS) and emerging (TTNT) clinical endpoints of treatment efficacy. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Efficacy of Orally VP16 Plus Prednisone with or without Cyclophosphamide for Refractory and Relapsed Peripheral T-Cell Lymphoma: A Retrospective Single Center Experience

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 6020-6020
Author(s):  
Anne-Blandine Boutin ◽  
Marjan Ertault de la Bretonnière ◽  
Helene Monjanel ◽  
Marlene Ochmann ◽  
Hélène Doyen ◽  
...  
Keyword(s):  
Adverse Event ◽  
T Cell ◽  
Progressive Disease ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
End Of Treatment ◽  
Time To Initiation ◽  
Haematological Adverse Event

Abstract Background: Peripheral T-cell lymphoma represents a heterogeneous group of mature T/NK lymphoma with an aggressive presentation course and a poor outcome. Disease history is characterized by a high degree of relapsed or refractory disease and an inferior outcome. Etoposide phosphate (VP16) is a non-specific antineoplastic agent frequently used in combination in oncology since 50 years. The major mechanism of action of etoposide is the inhibition of DNA topoisomerase II involved in the unwinding of the DNA molecule during replication. A recent preclinical study suggests an immunomodulatory effect of etoposide by selectively eliminating activated T cells. Specific efficacy of VP16 in T-cell lymphoma and especially in AITL had not been described. Methods: We retrospectively analyzed data on adult patients with refractory or relapsed angioimmunoblastic T-cell lymphoma (AITL), anaplastic large cell lymphoma and peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) treated in the university hospital center of Tours (France) by VP16 with or without cyclophosphamide between January 1992 and June 2014. VP16 was administrated orally at 75mg daily continuously or at 100 mg/m² daily for 3 days in 15-day cycles if it was associated with oral cyclophosphamide (150mg/m² daily for 3 days). Treatment was associated with prednisone 60 mg/m²/day. All responses were described according to Cheson criteria (2007). Adverse event were reported according to CTCAE (Common Toxicity Criteria of Adverse Events). Treatment was prescribed until progressive disease, intensive therapy or death, and could be stopped according to clinician appreciation after prolonged remission. Overall survival (OS) and event-free survival (EFS) were estimated since time to initiation of VP16 using the method of Kaplan and Meier. Event was defined as disease progression, death or change of treatment for any cause (toxicity, allograft etc.). Results: A total of 23 patients were enrolled : 13 AITCL, 2 anaplastic lymphoma (all of them were ALK-negative) and 8 PTCL-NOS. Median age at diagnostic was 57.3 years (range, 31-80) with a male preponderance (14/9). Most patients had B-symptoms (n=14) and advanced stage (n=19) with more than one extranodal site for 10 patients. Lactate deshydrogenase was upper normal value for 14 patients. The median number of prior systemic therapies was two (range, 2 to 4). Five patients received VP16 with cyclophosphamide. The overall response rate (ORR) was 65.2% (15 of 23), including 39.1% complete response (CR) (9 of 23). With a median follow up of 64.8 months (range, 33.7 to 96), the median OS was 39 months (95% IC: 4 months-not reached), and EFS 5 months (95% IC: 3 to 39) (Figure 1 and 2). Median duration of treatment was 15 months (range, 1 to 91). For AITL, the ORR was 69.2% (9 of 13), including 46.2% CR (6 of 13). The median OS was 77 months (95% IC : 5 months-not reached), and EFS 39 months (95% IC : 3-73). One haematological adverse event ≥grade 3 induced end of treatment, none extra-haematological adverse event was reported. Three patients received intensive regimen follow by allogenic stem cells transplantation and two of them (both AITL) after the response obtained with VP16. Progressive disease was the main cause of end of treatment (n=15) and of death (n=9). Patients treated with VP16 and cyclophosphamide had the same outcome than those treated with VP16 alone. For the 9 patients who reached a CR, 7 are still under CR at the end of follow up with median time of response of 67.4 months (range, 3 to 244). Conclusion: With or without cyclophosphamide, VP16 seems to be an effective and safe treatment for relapsed and refractory PTCL. AITL could have a higher sensibility to VP16 than other subtypes with durable responses. A prospective study should confirm these results, even for patient eligible for transplant program. Finally, orally VP16 can be an effective alternative treatment for unfit patients who cannot be included in clinical studies evaluating novel agents for PTCL. Figure 1: Survival since time to initiation of VP16 with or without cyclophosphamide (n=23) Figure 1:. Survival since time to initiation of VP16 with or without cyclophosphamide (n=23) Figure 2 Figure 2. Disclosures No relevant conflicts of interest to declare.

scholarly journals A randomized phase 3 trial of auto vs. allo transplantation as part of first-line therapy in poor-risk peripheral T-NHL

Blood ◽  
2020 ◽  
Author(s):  
Norbert Schmitz ◽  
Lorenz H Truemper ◽  
Krimo Bouabdallah ◽  
Marita Ziepert ◽  
Mathieu Leclerc ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Allogeneic Transplantation ◽  
T Cell Lymphoma ◽  
High Dose ◽  
First Line ◽  
High Dose Therapy ◽  
Peripheral T Cell Lymphoma ◽  
First Line Therapy ◽  
Line Therapy

Standard first-line therapy for younger patients with peripheral T-cell lymphoma consists of six courses of CHOP or CHOEP consolidated by high-dose therapy and autologous stem cell transplantation (AutoSCT). We hypothesized that consolidative allogeneic transplantation (AlloSCT) could improve outcome. 104 patients with nodal peripheral T-cell lymphoma except ALK+ ALCL, 18 to 60 years of age, all stages and IPI scores except stage 1 and aaIPI 0, were randomized to receive 4 x CHOEP and 1 x DHAP followed by high-dose therapy and AutoSCT or myeloablative conditioning and AlloSCT. The primary endpoint was event-free survival (EFS) at three years. After a median follow-up of 42 months, 3-year EFS of patients undergoing AlloSCT was 43% (95% confidence interval [CI]: 29%; 57%) as compared to 38% (95% CI: 25%; 52%) after AutoSCT. Overall survival at 3 years was 57% (95% CI: 43%; 71%) versus 70% (95% CI: 57%; 82%) after AlloSCT or AutoSCT, without significant differences between treatment arms. None of 21 responding patients proceeding to AlloSCT as opposed to 13 of 36 patients (36%) proceeding to AutoSCT relapsed. Eight of 26 patients (31%) and none of 41 patients died due to transplant-related toxicity after allogeneic and autologous transplantation, respectively. In younger patients with T-cell lymphoma standard chemotherapy consolidated by autologous or allogeneic transplantation results in comparable survival. The strong graft-versus-lymphoma effect after AlloSCT was counterbalanced by transplant-related mortality. CHO(E)P followed by AutoSCT remains the preferred treatment option for transplant-eligible patients. AlloSCT is the treatment of choice for relapsing patients also after AutoSCT.

scholarly journals Case of Primary Breast and Ipsilateral Axillary T-Cell Lymphoma: a Rare Occurrence

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Faryal Afridi ◽  
Garry D. Ruben ◽  
Eric Oristian
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Non Hodgkin Lymphoma ◽  
T Cell Lymphomas ◽  
Lost To Follow Up ◽  
Ipsilateral Axilla ◽  
Rare Diagnosis ◽  
Breast Malignancies

Background. Malignant lymphomas of the breast are rare and can be primary or secondary. Non-Hodgkin Lymphoma involving the breast is even rarer comprising 0.04-0.5% of all breast malignancies (Takemura). The incidence is even lower for T-cell lymphomas compared with B-cell subtype. We report the rare incidence of primary T-cell lymphoma involving both breast and ipsilateral axilla. Case. This is the case of an 80-year-old female who initially presented with asymmetry of her right breast. Initial mammograms were inconclusive. MRI could not be performed due to the patient’s severe claustrophobia. The patient was then lost to follow-up but re-presented with a new palpable density in the same breast. Subsequent mammogram showed a suspicious lesion with suspicious right axillary lymphadenopathy. Core biopsy was consistent with T-cell lymphoproliferative disorder involving both the breast and the axilla. She was then referred to medical oncology for management. Conclusion. Although rare, lymphoproliferative disorders of the breast can be encountered during workup for suspicious breast lesions. It is imperative that the surgeon is aware of this rare diagnosis to facilitate appropriate therapeutic intervention.

Retrospective Study on T Cell Malignent Lymphoma: Classification, Manifestation, Laboratory Finding and Survival.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4652-4652
Author(s):  
Hongyan Tong ◽  
Feng Xiao ◽  
Tieying Dai ◽  
Jie Jin ◽  
Haitao Meng ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Cell Lymphoma ◽  
Bone Marrow Involvement ◽  
Autologous Bone Marrow Transplantation ◽  
T Cell Lymphoma ◽  
World Health ◽  
Clinical Staging ◽  
T Cell Lymphomas

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.

Allogeneic Stem Cell Transplantation in Angioimmunoblastic T-Cell Lymphoma (AITL): A Retrospective Survey from the Lymphoma Working Party (LWP) of the European Group for Blood and Marrow Transplantation (EBMT).

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3042-3042
Author(s):  
Charalampia Kyriakou ◽  
C. Canals ◽  
G. Taghipour ◽  
J. Finke ◽  
H. Kolb ◽  
...  
Keyword(s):  
Overall Survival ◽  
Stem Cell ◽  
T Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Relapse Rate ◽  
Allogeneic Stem Cell Transplantation ◽  
Cell Lymphoma ◽  
T Cell Lymphoma

Abstract AITL is a rare peripheral T-cell lymphoma characterised by an aggressive behaviour, which primarily affects the elderly. Chemotherapy regimens fail to alter the high relapse rate and overall survival hardly exceeds 25% at 5 years. To date, there is no information on the potential role of allogeneic stem cell transplantation (allo-SCT) in the management of AITL. We report the outcome of 39 patients with a median age of 47 years (24–68), who underwent an allo-SCT between 1995 and 2004 for AITL, and were reported to the EBMT registry. The median time from diagnosis to transplant was 10 months (4–72). Thirty-four patients (87%) had previously received two or more treatment lines, and 16 patients (41%) a previous autologous SCT. Fifteen patients (38%) had a primary refractory disease, 13 (33%) were transplanted in partial remission and the remaining patients were in complete remission (CR) (mostly in 2nd and 3rd CR). Twenty-four patients were transplanted from an HLA-identical sibling and 15 from a matched unrelated donor. A myeloablative conditioning regimen (MAC) was used in 21 patients (cyclophosphamide + total body irradiation in 14), while 18 patients received fludarabine-based reduced intensity conditionings (RIC). Peripheral blood was the source of stem cells in 35 patients (90%). Three patients failed to engraft (one patient in the RIC group). Twenty-one patients (54%) developed acute graft versus host disease (grade I-II, n=16; grade III-IV, n=5). Twenty-eight patients (72%) achieved a CR after the allogeneic procedure. Nine patients died from transplant related mortality (TRM) and 5 patients from disease progression. The cumulative incidence of TRM at 12 months was 19% for the MAC and 26% for the RIC group. After a median follow-up for the surviving patients of 20 months (6–74), 25 patients are alive. Relapse rates at 1 and 3 years were estimated at 10% and 18% for the MAC and 16 and 20% for the RIC patients. Progression free survival rates at 3 years were 67% and 50% and the overall survival at the same time 71% and 56% for the MAC and RIC group of patients, respectively. Although follow up is rather short, these data suggest that allo-SCT results in good overall response and is associated with a low relapse rate in this group of poor risk heavily pre-treated and rather elderly group of AITL patients. Allo-SCT could be considered a therapeutic option for eligible high-risk AITL patients. Nevertheless, the impact of this approach should be further explored in prospective collaborative studies.

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