Efficacy and safety of mitoxantrone hydrochloride liposome injection in treatment of peripheral T-cell lymphomas: a multicenter, non-interventional, ambispective cohort, real-world study (MOMENT) / 白血病·淋巴瘤

Objective:To evaluate the efficacy and safety of mitoxantrone hydrochloride liposome injection in the treatment of peripheral T-cell lymphoma (PTCL) in a real-world setting.Methods:This was a real-world ambispective cohort study (MOMENT study) (Chinese clinical trial registry number: ChiCTR2200062067). Clinical data were collected from 198 patients who received mitoxantrone hydrochloride liposome injection as monotherapy or combination therapy at 37 hospitals from January 2022 to January 2023, including 166 patients in the retrospective cohort and 32 patients in the prospective cohort; 10 patients in the treatment-na?ve group and 188 patients in the relapsed/refractory group. Clinical characteristics, efficacy and adverse events were summarized, and the overall survival (OS) and progression-free survival (PFS) were analyzed.Results:All 198 patients were treated with mitoxantrone hydrochloride liposome injection for a median of 3 cycles (range 1-7 cycles); 28 cases were treated with mitoxantrone hydrochloride liposome injection as monotherapy, and 170 cases were treated with the combination regimen. Among 188 relapsed/refractory patients, 45 cases (23.9%) were in complete remission (CR), 82 cases (43.6%) were in partial remission (PR), and 28 cases (14.9%) were in disease stabilization (SD), and 33 cases (17.6%) were in disease progression (PD), with an objective remission rate (ORR) of 67.6% (127/188). Among 10 treatment-na?ve patients, 4 cases (40.0%) were in CR, 5 cases (50.0%) were in PR, and 1 case (10.0%) was in PD, with an ORR of 90.0% (9/10). The median follow-up time was 2.9 months (95% CI 2.4-3.7 months), and the median PFS and OS of patients in relapsed/refractory and treatment-na?ve groups were not reached. In relapsed/refractory patients, the difference in ORR between patients with different number of treatment lines of mitoxantrone hydrochloride liposome injection [ORR of the second-line, the third-line and ≥the forth-line treatment was 74.4% (67/90), 73.9% (34/46) and 50.0% (26/52)] was statistically significant ( P = 0.008). Of the 198 PTCL patients, 182 cases (91.9%) experienced at least 1 time of treatment-related adverse events, and the incidence rate of ≥grade 3 adverse events was 66.7% (132/198), which was mainly characterized by hematologic adverse events. The ≥ grade 3 hematologic adverse events mainly included decreased lymphocyte count, decreased neutrophil count, decreased white blood cell count, and anemia; non-hematologic adverse events were mostly grade 1-2, mainly including pigmentation disorders and upper respiratory tract infection. Conclusions:The use of mitoxantrone hydrochloride liposome injection-containing regimen in the treatment of PTCL has definite efficacy and is well tolerated, and it is a new therapeutic option for PTCL patients.

Clinical treatment analysis of newly treated peripheral T-cell lymphoma / 白血病·淋巴瘤

Objective:To investigate the treatment methods of peripheral T-cell lymphoma (PTCL).Methods:The clinical data of 251 newly treated PTCL patients in the First Hospital of Jilin University from August 2011 to October 2021 were retrospectively analyzed, from which 168 patients were intercepted from February 2015 (the first targeted drug of PTCL, chidamide, was launched in China) to October 2021, among which 20 patients received chemotherapy combined with brentuximab vedotin (BV, BV group), 37 patients received chemotherapy combined with chidamide (chidamide group), and 111 patients received non-targeted therapy (non-targeted therapy group); all patients received ≥2 courses of treatment. Ten patients received autologous peripheral blood hematopoietic stem cell transplantation, with non-transplanted patients in the same period as controls. The clinical efficacy and prognosis of patients with different treatment methods were analyzed. Kaplan-Meier method was used for survival analysis and log-rank test was performed.Results:Of all 251 patients with PTCL, 26.7% (67/251) received targeted therapy in combination with chemotherapy. In the chidamide group, the efficacy could be evaluated in 36 cases, with an overall response rate (ORR) of 91.7% (33/36); in the non-targeted therapy group, the efficacy could be evaluated in 88 cases, with an ORR of 71.6% (63/88); in the BV group, 20 cases were evaluable, with an ORR of 75.0% (15/20). The difference in ORR between the non-targeted therapy group and the chidamide group was statistically significant ( χ2 = 5.89, P = 0.015), and the difference in ORR between the non-targeted therapy group and the BV group was not statistically significant ( χ2 = 0.09, P = 0.759). The 1-year progression-free survival (PFS) rates were 79.9%, 88.2% and 64.2%, and the 1-year overall survival (OS) rates were 85.7%, 89.7% and 70.1% in the chidamide, BV and non-targeted therapy groups, respectively; the PFS and OS in the chidamide and BV groups were better than those in the non-targeted therapy group (all P < 0.05), and the adverse effects were mostly tolerable. Among patients treated with chemotherapy combined with BV, the ORR of patients with CD30 expression rate <60% and ≥60% were 54.5% (6/11) and 100.0% (9/9), and the difference was statistically significant ( P = 0.038). In the 10 hematopoietic stem cell transplanted patients and 50 non-transplanted patients, 1-year PFS rates were 87.5% and 59.5%, 1-year OS rates were 90.0% and 67.1%, and the differences were not statistically significant (both P > 0.05). Conclusions:Chemotherapy-based combination therapy is the main treatment methods for PTCL, and chemotherapy combined with chidamide or BV targeted therapy and hematopoietic stem cell transplantation can improve the long-term survival of PTCL patients.

Treatment progress of relapsed/refractory peripheral T-cell lymphoma / 白血病·淋巴瘤

Peripheral T-cell lymphoma (PTCL) is a group of heterogeneous diseases originating from retrothymic T lymphocytes or mature natural killer (NK) cells. With the innovation of medical technology, the prognosis of patients with PTCL has been greatly improved. However, there are still some patients who are refractory or relapsed after treatment and have poor prognosis. In recent years, the applications of second-line chemotherapy regimen, hematopoietic stem cell transplantation and several new drugs (histone deacetylase inhibitors, dihydrofolate reductase inhibitors, aurora A kinase inhibitors, phosphatidylinositol 3-kinase inhibitors, targeted therapy, etc.) have played an important role in the treatment of relapsed/refractory PTCL patients. Meanwhile, the choice of transplantation programs and the combination of new drug-based schemes have also become research hotspots.

Primary peripheral T-cell lymphoma of the cervix with mononeuritis multiplex: an unusual case presentation

Peripheral neuropathy (PN) is characterized by the injury to the peripheral nervous system of varied etiology. Lymphoma is one of the etiologies of PN, presenting various neurological manifestations. Neuropathy associated with peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is unusual and fewer cases are documented in the literature. In addition, PTCL, NOS is extremely rare as primary in the female genital tract, especially uterine cervix, and exhibits aggressive clinical course with poor therapy response. We hereby describe a 47-year-old female who presented with fever and chills for 15 days. Clinical examination revealed left-sided lower motor neuron type of facial nerve palsy with Bell's phenomenon. Nerve conduction study of all four limbs illustrated asymmetrical axonal neuropathy (motor > sensory), suggesting mononeuritis multiplex. She developed vaginal bleeding during her hospital stay. Pelvic examination and imaging revealed a 4x3cm polypoidal mass on the posterior lip of the cervix, which was excised and diagnosed as extranodal primary PTCL, NOS based on morphology, immunohistochemistry, and in-situ hybridization findings. Besides, the cerebrospinal fluid (CSF) was infiltrated by the lymphoma cells, detected on cell block preparation. The patient succumbed to her illness within one week despite best efforts and the commencement of chemotherapy. No consent was obtainable for nerve biopsy and autopsy. Thus, we report an extremely rare case of primary extranodal PTCL, NOS of the uterine cervix with unusual presentation of mononeuritis multiplex. Further, we discussed the differentials of PTCL, NOS at this extranodal site.

Prognostic role of baseline 18F-FDG PET/CT metabolic parameters and relevant clinical factors in peripheral T-cell lymphoma, not otherwise specified / 中华核医学与分子影像杂志

Objective:To explore the prognostic role of baseline 18F-FDG PET/CT metabolic parameters for patients with peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). Methods:From February 2010 to January 2019, 47 PTCL-NOS patients (29 males, 18 females, age: (59.7±13.6) years) from Nanjing Drum Tower Hospital were retrospectively enrolled. Each patient underwent baseline 18F-FDG PET/CT imaging before treatment. The total metabolic tumor volume (TMTV) and total lesion glycolysis (TLG) were computed by using the margin threshold of 41% SUV max. Kaplan-Meier survival analysis, univariate and multivariate Cox proportional hazards regression models were used to evaluate progression-free survival (PFS) and overall survival (OS). Results:Over the follow-up of 5-119 months, 25 patients had disease progression, including 24 deaths. SUV max (hazard ratio ( HR)=8.581, 95% CI: 1.950-37.764, P=0.004), TMTV( HR=9.677, 95% CI: 3.521-26.593, P<0.001), TLG( HR=3.647, 95% CI: 1.245-10.682, P<0.001) and prognostic index for T-cell lymphoma (PIT; HR=4.593, 95% CI: 1.792-11.773, P=0.002) were significant predictors of PFS and OS( HR=8.720, 95% CI: 1.982-83.354, P=0.004; HR=9.325, 95% CI: 3.423-25.408, P<0.001; HR=3.439, 95% CI: 1.170-10.110, P<0.001; HR=4.437, 95% CI: 1.728-11.393, P=0.002). After multivariate analysis, TMTV was the independent predictor of PFS ( HR=4.371, 95% CI: 1.066-16.541, P<0.001) and OS ( HR=4.978, 95% CI: 1.123-21.329, P<0.001). The substratification analysis showed that patients with high TMTV(≥168.3 cm 3) had worse prognosis than those with low TMTV (<168.3 cm 3) for PFS ( χ2=14.60, P<0.001) and OS ( χ2=16.81, P<0.001) in low PIT (0-1) group, while patients with high TMTV had worse prognosis than those with low TMTV for PFS ( χ2=4.09, P=0.043) in high PIT (≥2) group. Conclusions:Baseline PET/CT metabolic parameters including SUV max, TMTV, TLG and PIT are able to predict survival in PTCL-NOS patients. TMTV is the independent predictor of PFS and OS, which can substratify PTCL-NOS patients in PIT group.

Progress of hematopoietic stem cell transplantation in treatment of peripheral T-cell lymphoma / 白血病·淋巴瘤

Peripheral T-cell lymphoma (PTCL) is a group of highly heterogeneous rare malignant lymphoproliferative diseases, and PTCL patients have low therapeutic efficacy rate and poor prognosis after conventional comprehensive treatments. Hematopoietic stem cell transplantation (HSCT) can improve the survival of PTCL patients, and previous studies showed that patients with a definite diagnosis should receive high-dose chemotherapy combined with autologous-HSCT (auto-HSCT) in the first remission. In recent years, a consensus on the role of auto-HSCT as the first-line consolidation therapy for PTCL patients has not been reached so far. Allogeneic-HSCT is an effective option for relapsed and refractory patients with PTCL, while auto-HSCT has unfavorable efficacies. This paper reviews the research progress of HSCT in treatment of PTCL.

Progress of angioimmunoblastic T-cell lymphoma / 白血病·淋巴瘤

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive peripheral T-cell lymphoma (PTCL) that originates from follicular helper T cells (TFH). It has the highest incidence in PTCL which is second only to PTCL-not otherwise specified (PTCL-NOS). Its clinicopathological diagnosis is difficult, it is easy to be misdiagnosed and missed, and the prognosis is poor. This article reviews the pathogenesis, clinical and pathological characteristics as well as treatment progress of AITL, in order to provide a reasonable basis for clinical diagnosis and treatment of the disease.

Prognostic value of peripheral blood absolute lymphocyte count for patients with peripheral T-cell lymphoma, not otherwise specified / 白血病·淋巴瘤

Objective To explore the prognostic value of peripheral blood absolute lymphocyte count (ALC) for patients with peripheral T-cell lymphoma,not otherwise specified (PTCL-NOS).Methods The clinical data of 69 patients with PTCL-NOS treated in Tianjin Union Medical Center from January 2008 to January 2016 were analyzed retrospectively.The relationship between different levels of ALC and clinical characteristics,therapeutic efficacy and prognosis was analyzed.Results Among 69 patients,23 cases (33.3％) had low ALC (＜1.0×109/L),and 46 cases (66.7％) had high ALC (≥ 1.0×109/L).Compared with the high ALC group,the low ALC group showed the higher International Prognostic Index (IPI) and PTCL-NOS Prognostic Index (PIT) scores,advanced clinical stage and higher lactate dehydrogenase level (all P ＜ 0.05).The total efficacy rate in the low ALC group was lower than that in the high ALC group [56.5％ (13/23) vs.67.4％ (31/46)],but the difference was not statistically significant (x2 =0.784,P =0.376).The 3-year survival rate in the low ALC group was significantly lower than that in the high ALC group,and the difference was statistically significant (40.5％ vs.68.6％,x2 =7.846,P =0.010).Univariate analysis showed that the US Eastern Cooperative Oncology Group performance status score ≥ 2,IPI score ≥2,Ann Arbor stage Ⅲ-Ⅳ and ALC＜1.0×109/L were the poor prognostic factors (all P ＜ 0.05),while Cox multivariate analysis showed that the Ann Arbor stage Ⅲ-Ⅳ and ALC＜1.0×109/L were the independent risk factors for prognosis of patients with PTCL-NOS (P =0.008,P =0.029).Conclusion The decrease of peripheral blood ALC in patients with PTCL-NOS at the initial diagnosis suggests a poor prognosis,and ALC can be used as a new indicator for prognosis evaluation of PTCL-NOS patients.

Prognostic value of lymphocyte-to-monocyte ratio in angioimmunoblastic T cell lymphoma / 中华血液学杂志

Objective@#To evaluate the prognostic value of lymphocyte-to-monocyte ratio (LMR) in angioimmunoblastic T cell lymphoma (AITL).@*Methods@#Data of 64 patients diagnosed as AITL at the First Affiliated Hospital of Nanjing Medical University between June 2009 and July 2017, were analyzed retrospectively. Receiver Operator Characteristic (ROC) curve was used to calculate the cutoff value of LMR to divide this cohort of patients into high and low LMR groups. Characteristics between groups were compared by Pearson Chi-square or Fisher exact tests. Kaplan-Meier method and Cox regression were performed to probe prognostic factors associated with progression-free survival (PFS) and overall survival (OS).@*Results@#A total of 64 cases [39 cases male and 25 ones female with the median age of 63 (29-89) years old] were enrolled. The cutoff value of LMR was 3.07. Patients with low LMR showed inferior PFS (9 months vs 13 months, P=0.044) and OS (16 months vs not reached, P=0.014), respectively than those without low LMR during a median follow-up of 33 months (5 to 103 months). Multivariate analysis showed that low LMR was an independent prognostic factor associated with poor outcomes (HR=0.48, 95% CI 0.26-0.92 for PFS, P=0.027; HR=0.38, 95% CI 0.18-0.82 for OS, P=0.013, respectively). Subgroup analysis showed that patients with low LMR and under the situation of high score of Prognostic Index for peripheral T-cell lymphoma, Unspecified (PIT) (2-4) had shorter PFS and OS (P=0.013 and P=0.031, respectively). But in low score of PIT (0-1) group, low LMR seemed to play almost no effects on PFS and OS (P=0.949 and P=0.238, respectively).@*Conclusions@#The disease risk status of patients could be initially assessed according to PIT score and LMR level. Low LMR was demonstrated to be able to predict poor outcome in AITL.

Clinical outcomes of peripheral blood stem cell transplantation for aggressive peripheral T-cell lymphoma / 中华血液学杂志

Objective@#To evaluate clinical outcomes of autologous and allogeneic peripheral blood stem cell transplantation (PBSCT) for aggressive peripheral T-cell lymphoma (PTCL).@*Methods@#From June 2007 to June 2017, clinical data of PTCL patients who underwent PBSCT were assessed retrospectively.@*Results@#Among 41 patients, 30 was male, 11 female, and median age was 38(13-57) years old. Seventeen patients with autologous PBSCT (auto-PBSCT) and 24 patients with allogeneic PBSCT (allo-PBSCT) were enrolled in this study. Eight patients (8/17, 47.1%) in auto-PBSCT group were ALK positive anaplastic large cell lymphoma (ALCL), 7 patients (7/24, 29.2%) with NK/T cell lymphoma and 9 patients (9/24, 37.5%) with PTCL-unspecified (PTCL-U) in allo-PBSCT group (P=0.035). There were 58.8% patients (10/17) in complete response (CR) status and 11.8% (2/17) in progression disease (PD) status before transplantation in auto-PBSCT group, and 8.3% (2/24) in CR status and 45.8% (11/24) in PD status before transplantation in allo-PBSCT group (P=0.026). The 2-years cumulative overall survival (OS) were (64.0±10.8)% and (53.5±9.7)% for auto-PBSCT and allo-PBSCT respectively (P=0.543). The 2-years cumulative disease-free survival (DFS) were (57.1±12.4)% and (53.5±10.6)% for auto-PBSCT and allo-PBSCT respectively (P=0.701). In patients with dead outcomes after PBSCT, 83.3% (5/6) of death cause was relapse in auto-PBSCT and 41.7% (5/12) of death cause was relapse in allo-PBSCT.@*Conclusion@#Both auto-PBSCT and allo-PBSCT were effective for PTCL. Allo-PBSCT maybe was better than auto-PBSCT for high-risk PTCL with poor prognosis.

Treatment progress of peripheral T-cell lymphoma / 白血病·淋巴瘤

Peripheral T-cell lymphoma (PTCL), also known as mature T-cell lymphoma, is a group of malignant proliferative diseases derived from mature T cells. Because natural killer (NK) cells are similar in immunophenotype and function to T cells, NK-cell lymphoma and mature T-cell lymphoma are often classified as one class. The 60th American Society of Hematology (ASH) Annual Meeting has covered various fields about PTCL, especially in terms of treatment. Brentuximab vedotin combined with CHP regimen, histone deacetylase inhibitor combined with HMA regimen, and classic CHOP regimen together with lenalidomide or alemtuzumab provide new options for the treatment of PTCL patients; novel drugs represented by JAK inhibitors, daratumumab, and TP53 inhibitors have also initially demonstrated clinical utility, but large-scale clinical trials are still needed for validation.

Prognostic significance of NCCN-International Prognostic Index (NCCN-IPI) for patients with peripheral T-cell lymphoma treated with CHOP-based chemotherapy / 中华血液学杂志

Objective@#To validate the prognostic value of NCCN-International Prognostic Index (NCCN-IPI) for patients with peripheral T-cell lymphoma (PTCL) treated with CHOP-based chemotherapy.@*Methods@#A retrospective analysis in 162 PTCL patients who were initially diagnosed and treated in Rui Jin Hospital from January 2003 to May 2013 was conducted. Baseline characteristics were collected, and survival analysis was performed according to the IPI and NCCN-IPI model.@*Results@#The estimated 5-year overall survival (OS) rate and progression free survival (PFS) rate were 33% and 20%, with median OS and PFS of 17.0 months and 9.2 months, respectively. Multivariate analysis indicated ECOG score (PFS: HR=2.418, 95%CI 1.535-3.809, P<0.001; OS: HR=2.347, 95%CI 1.435-3.839, P= 0.001) , specific extra-nodal sites (PFS: HR=1.800, 95%CI 1.216-2.665, P=0.003; OS: HR=1.608, 95% CI 1.054-2.454, P=0.027) and pathology type (PFS: HR=0.424, 95% CI 0.184-0.975, P=0.043; OS: HR=0.276, 95% CI 0.087-0.877, P=0.029) were independent prognostic factors of OS and PFS for the patients with PTCL. The survival rates of low risk patients based on NCCI-IPI were remarkably higher than the counterparts based on IPI (5-year OS 74% vs 54%, χ2=5.041, P=0.025, 5-year PFS 50% vs 38%, χ2= 5.295, P=0.021) . NCCN-IPI was outstanding to identify the subgroup of low risk patients with PTCL, who may benefit from conventional chemotherapy such as CHOP or CHOP-like regimen.@*Conclusion@#NCCN-IPI is more powerful for low risk PTCL patients and a strong supplement for IPI.

Methotrexate and etanercept-induced primary cutaneous CD4 positive small/medium-sized pleomorphic T-cell lymphoma

Abstract: Immunosuppressive drugs and biological agents may represent a potential risk of lymphoma development in patients with rheumatoid arthritis. But most cases are diffuse, large B-cell lymphomas. Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma, a provisional entity in the 2005 WHO-EORTC classification of cutaneous lymphomas, is only described in a limited number of reports. To our knowledge, our case is a rare instance of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma, after associated treatment with methotrexate and etanercept, in a patient with moderate rheumatoid arthritis who had undergone an orchidectomy incorrectly.

Efficacy of CHOP chemotherapy on unspecified peripheral T cell lymphoma in patients aged 80 years and over and its prognostic analysis / 中华老年医学杂志

Objective To evaluate the efficacy of CHOP (cyclophosphamide,doxorubicin,vincristine,and prednisone)chemotherapy on peripheral T cell lymphomas-unspecified (PTCL-U)in patients aged 80 years and over,and investigate the chemotherapy adverse effects and its prognostic factors.Methods Clinical data of 57 patients with PTCL-U confirmed by pathology and immunohistochemistry in Guangzhou First People's Hospital from January 2008 to January 2015 were retrospectively analyzed.The efficacy and adverse effects of CHOP chemotherapy,and its prognostic factors were evaluated.Results Of 57 patients,11 (19.3％) achieved complete remission,23(40.4％)had partial remission,14(24.6％)had stable disease,and 9(15.8％)had progressive disease after CHOP chemotherapy,with a total effective rate of 59.6％ (34 cases).The expected 1-year,2-year and 3-year overall survival(OS)rate was 70.2％ (40 cases),36.8％ (21 cases) and 17.5％ (10 cases),respectively.The median survival time was 18.2 months.Among the 57 patients,40(70.2％) had hypoplasia of bone marrow at degree Ⅰ-Ⅱ,17 (29.8％) at degree Ⅲ-Ⅳ,49 (86.0％) had mild nausea and vomiting(degree Ⅰ-Ⅱ),8(14.0％)had severe nausea and vomiting(degree Ⅲ-Ⅳ),7 had liver dysfunction,2 had cardiac toxicity and 7 had disturbance of blood coagulation.Univariate analysis showed that the alkaline phosphatase,Ki-67 percentage,lymphoma international prognostic index(IPI)score,extranodal involved sites(＞1),as well as the efficacy and course of chemotherapy were the prognostic factors for survival time in very elderly patients.Multivariate analysis indicated that IPI score＞2(95％CI:1.12～6.35,x2 ＝5.45,P＜0.01),extranodal involved sites(＞1)(95％ CI:2.58-15.32,x2 ＝ 16.42,P＜0.01),disease progression(95％CI:1.82～12.15,x2 ＝ 10.23,P＜0.01),chemotherapy courses(＞4) (5％CI:0.18～0.79,x2 ＝7.28,P＜0.01)were the independent prognostic factors for the median survival time in very elderly patients.Conclusions PTCL U patients aged 80 years and over have poor prognosis.CHOP chemotherapy has a certain effect on PTCL-U in very elderly patients,and chemotherapy side effects can be tolerated.IPI score＞2 and extranodal involved sites(＞ 1)are the important prognostic factors in very elderly PTCL-U patients.The completion of full course of chemotherapy with remission occurrence is important to prolong survival time in very elderly PTCL-U patients.

Primary pulmonary peripheral T-cell lymphoma, not otherwise specified：two cases report and literature review / 白血病·淋巴瘤

Objective To study the clinical characteristics, diagnosis, treatment and prognosis of primary pulmonary peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). Methods Two cases of primary pulmonary PTCL-NOS were studied and relevant literature were reviewed. Results Case 1 diagnosed as primary pulmonary PTCL-NOS was a 44 years old woman and disease progressed after GLD (Gem+L-OHP+DXM) chemotherapy regimen. At last, the patient died of respiratory failure after one month. Case 2 diagnosed as primary pulmonary PTCL-NOS was a 46 years old man and reach partial response after CHOP regimen, and still alive now.Conclusion Primary pulmonary PTCL-NOS is very rare. It is easy to be misdiagnosed due to non-specific clinical and imaging manifestations. Acquiring enough tissue specimens for pathologic examination is the key to a definitive diagnosis. At present, there is no standard chemotherapy regimen for these patients, the prognosis is relatively poor.

Efficacy observation of pegaspargase combined with intensity modulated conformal radiotherapy in the treatment of 20 patients with early-stage nasal NK/T cell lymphoma / 白血病·淋巴瘤

Objective To study the clinical characteristics, diagnosis, treatment and prognosis of primary pulmonary peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). Methods Two cases of primary pulmonary PTCL-NOS were studied and relevant literature were reviewed. Results Case 1 diagnosed as primary pulmonary PTCL-NOS was a 44 years old woman and disease progressed after GLD (Gem+L-OHP+ DXM) chemotherapy regimen. At last, the patient died of respiratory failure after one month. Case 2 diagnosed as primary pulmonary PTCL-NOS was a 46 years old man and reach partial response after CHOP regimen, and still alive now.Conclusion Primary pulmonary PTCL-NOS is very rare. It is easy to be misdiagnosed due to non-specific clinical and imaging manifestations. Acquiring enough tissue specimens for pathologic examination is the key to a definitive diagnosis. At present, there is no standard chemotherapy regimen for these patients, the prognosis is relatively poor.

Linfoma de células t angioimunoblástico em paciente idoso e com diagnóstico prévio de mononucleose infecciosa: um desafio para o tratamento oncológico ? relato de caso / Angioimmunoblastic T-cell lymphoma in elderly patient with a previous diagnosis of infectious mononucleosis: a challenge for cancer treatment ? case report

O linfoma de células T angioimunoblástico (AITL) é subtipo raro de linfoma de células T periférico que representa cerca de 2% dos linfomas não Hodgkin (LNH). Acomete predominantemente homens, na sexta e sétima décadas de vida e se apresenta como doença sistêmica. É agressivo no comportamento e responde mal à quimioterapia, com tratamento padrão ainda não definido. Este relato objetiva apresentar o prognósticodesfavorável nos pacientes com AITL e a falta de consolidação de seu tratamento. Os fatores que podem estar associados à má-resposta são: idoso, estágios avançados e sintomatologia constitucional.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of peripheral T-cell lymphoma that represents about 2% of non-Hodgkin?s lymphomas (LNH). It affects mainly men, in the sixth and seventh decades of life, and appears as a systemic disease. It has an aggressive behavior and responds poorly to chemotherapy, still with undefined standard treatment. This report aims to provide the poor prognosis in patients with AITLand the lack of treatment consolidation. The factors that may be associated with the poor response are being elderly, advanced stages, and constitutional symptoms.

The expression and significance of MDSCs in peripheral blood in patients with peripheral T cell lymphoma / 天津医药

Objective To study the relationship between the number of myeloid-derived suppressor cells (MDSCs) in peripheral blood and prognosis in patients with peripheral T-cell lymphoma (PTCL). Methods Twenty-three patients with PTCL were selected in this study and 14 healthy persons were used as the normal control. The proportion of MDSCs/mononu?clear in peripheral blood was detected by flow cytometry. The correlation between the number of MDSCs and the clinical character and prognosis in patients with PTCL was analyzed. Results The proportion of MDSCs in peripheral blood was sig?nificantly higher in patients with PTCL than that of healthy subjects [(4.59±1.41)%vs (1.36±0.59)%, P<0.01]. The number of MDSCs in peripheral blood was significantly decreased after chemotherapy compared with that before treatment [( 1.29 ± 2.95)%, P<0.05)]. There were significant differences in MDSCs expressions in patients between different extranodal involve?ment, bone marrow involvement, clinical stage and IPI score (P<0.05). Conclusion The higher expression of MDSCs may be related to the progression in patients with PTCL.

Therapeutic efficacy of peripheral T cell lymphoma patients treated with first-line intensive chemotherapy followed by autolo-gous stem cell transplantation / 中国医师杂志

Objective To investigate the outcomes of unselected peripheral T cell lymphoma (PTCL) patients treated with in-tensive first-line chemotherapy with high-dose therapy followed by autologous stem cell transplantation (ASCT).Methods Here a nonrandom study was reported for 23 PTCL patients treated with first-line intensive chemotherapy followed by autologous stem cell trans-plantation and 23 PTCL patients treated with conventional chemotherapy during January in 2000 to 2011 .All patients had received E-CHOP for 6~8 cycles, and autologous stem cell transplantation group was administrated with intensive chemotherapy followed by ASCT after complete remission or partial remission .Results There was no statistically significant difference in short-term therapeutic effect between two groups( P >0.05), but the 5-year overall survival(OS) of autologous stem cell transplantation group( 58%) was higher than conventional chemotherapy group , as well as 5-year disease-free survival time (DFS) (45%in autologous stem cell transplanta-tion group, and 21%in conventional chemotherapy group ) with both statistical significance ( P <0.05).Only the incidence of Ⅳ° myelosuppression in autologous stem cell transplantation group ( 100%) was higher than that in conventional chemotherapy group ( 13%) ( P <0.01 ) .Conclusions First-line intensive chemotherapy followed by autologous stem cell transplantation for peripheral T cell lymphoma was quietly safe utility , it was better than conventional chemotherapy which would be considered as first -line method.

Recurrence of peripheral T-cell lymphoma as granulomas in the lower limbs / Granulomas epitelióides cutâneos, como manifestação de recidiva de linfoma de células T periférico

The presence of granulomas in the skin of T-cell lymphoma patients is a rare but well-known phenomenon. The authors present the case of a 44-year-old Caucasian male, with a previously treated peripheral T-cell lymphoma, with cutaneous infiltration and extensive dyschromia on his lower limbs. Skin biopsies revealed the presence of sarcoid granulomas associated with the recurrence of the lymphoma, confirmed by immunostaining and molecular analysis. Although a new scheme of chemotherapy was started, he died 18 months later. There are two different patterns of skin granulomas associated with lymphoma: granulomatous infiltrates admixed with neoplastic cells and cutaneous granulomas constituting a nonspecific manifestation of the underlying lymphoma, but presently there is no evidence supporting their relationship with lymphoma prognosis.

A presença de granulomas cutâneos associados a linfomas é um fenômeno raro, mas bem conhecido. Um homem, 44 anos de idade, com diagnóstico prévio de linfoma de células T periférico, foi enviado à nossa consulta por infiltração cutânea e extensa discromia nos membros inferiores. A biopsia cutânea revelou a presença de granulomas epitelioides associados à recidiva do linfoma, confirmada pela marcação imuno-histoquímica e estudo molecular. Apesar de iniciado novo esquema de quimioterapia, o doente faleceu 18 meses depois. Na literatura são descritos dois tipos de granulomas cutâneos na presença de linfomas: associados à infiltração cutânea pelo linfoma ou como uma manifestação não específica do linfoma. Contudo, atualmente não há evidência que suporte a sua relação com o prognóstico dos linfomas.