Diagnosis and management of rare paediatric Non-Hodgkin lymphoma.

Mature B-cell lymphomas, (B- or T-cell) lymphoblastic lymphomas (LBL), and anaplastic large cell lymphoma (ALCL) correspond to about 90% of all non-Hodgkin lymphoma (NHL) cases occurring in children and adolescents. The remaining 10% encompass a complex group of entities characterized by low/very low incidences, paucity of knowledge in terms of underlying biology in comparison to their adult counterparts, and consequent lack of standardization of care, information on clinical therapeutic efficacy and long-term survival. At the Seventh International Symposium on Childhood, Adolescent and Young Adult NHL, organized on October 20-23, 2022, in New York City, New York, US, we had the opportunity to discuss clinical, pathogenetic, diagnostic, and treatment aspects of certain subtypes of rare B- or T-cell NHL and they will be the topic of this review.

Mature T-cell and NK-cell lymphomas: updates on molecular genetic features.

Mature T-cell and NK-cell lymphomas are a heterogeneous group of rare and typically aggressive neoplasms. Diagnosis and subclassification have historically relied primarily on the integration of clinical, histologic, and immunophenotypic features, which often overlap. The widespread application of a variety of genomic techniques in recent years has provided extensive insight into the pathobiology of these diseases, allowing for more precise diagnostic classification, improved prognostication, and development of novel therapies. In this review, we summarize the genomic features of the most common types of mature T-cell and NK-cell lymphomas with a particular focus on the contribution of genomics to biologic insight, classification, risk stratification, and select therapies in the context of the recently published International Consensus and updated World Health Organization classification systems.

Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich syndrome): Frequency, clinical implication, and prognosis.

BACKGROUND: Episodic angioedema with eosinophilia (EAE) (Gleich syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia, and frequent elevated serum IgM level. METHODS: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France. RESULTS: A total of 30 patients with a median age at diagnosis of 41 years (range, 5-84) were included. The median duration of each crisis was 5.5 days (range, 1-90), with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%), of whom 5 (17%) showed evidence of clonal T-cell receptor gamma locus gene (TRG) rearrangement. The median duration of follow-up was 53 months (range, 31-99). The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio, 4.15; 95% confidence interval, 1.18-14.66; P = .02). At last follow-up, 3 patients (10%) were able to have all treatments withdrawn and 11 (37%) were in clinical and biologic remission with less than 10 mg of prednisone daily. CONCLUSION: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare.

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.

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.

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.

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.

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.

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.

[Two Lethal Cases of Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Deteriorated Rapidly After Emergency Surgery for Intestinal Perforation].

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive form of primary gastrointestinal T-cell lymphoma. Symptoms can vary but often include fever, abdominal pain, weight loss, diarrhea, obstruction, and perforation. Disease-specific symptoms rarely present before patients reach an advanced stage, which contributes to delayed diagnosis and poor survival outcomes. Approximately half of the patients with MEITL undergo emergency surgery for acute intestinal obstruction or perforation, leading to peritonitis, septic shock, and multiple organ failure. These factors contribute to treatment delays, which are associated with a worse prognosis, particularly in the case of chemotherapy. This paper reports two fatal cases of patients with MEITL who deteriorated rapidly after emergency surgery for intestinal perforation. Patient 1 complained of persistent diarrhea, but a delayed diagnosis led to bowel perforation, and the subsequent chemotherapy treatment was canceled. Patient 2 was diagnosed relatively early, but treatment was delayed due to intestinal perforation. Despite its rarity, MEITL should be considered through a "high index of suspicion" approach when a patient complains of unexplained abdominal pain and diarrhea. This is expected to improve early diagnosis and ultimately patient prognosis.

MR Imaging Characteristics of Primary T-Cell Lymphoma of the Cauda Equina: A Case Report and Literature Review.

Primary central nervous system lymphoma is a rare form of extranodal non-Hodgkin lymphoma, and primary T-cell lymphoma of the cauda equina is extremely rare. We describe a case involving a 56-year-old female who presented with low back pain and radiating leg pain for 4 months. MRI of the lumbar spine revealed an elongated, multinodular intradural lesion of approximately 10 cm from the L4 body to the S2 body level with iso-signal intensity on T1-weighted imaging, heterogeneous iso- and high-signal intensity on T2-weighted imaging, and a heterogeneous intense enhancement on gadolinium contrast-enhanced T1-weighted imaging. A peripheral T-cell lymphoma of the cauda equina was diagnosed on the basis of immunohistochemical and T-cell receptor gamma gene rearrangement analysis after intradural biopsy of the mass.

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.

[Peripheral T-cell lymphoma with follicular helper of T cell phenotype of Waldeyer's ring: a clinicopathological and genetic study of eight cases].

Objective: To study the clinicopathologic and genetic features of Waldeyer's ring peripheral T-cell lymphoma with follicular helper T cell immunophenotypes (wPTCL-TFH), with comparison to the nodal peripheral T-cell lymphoma with TFH immunophenotypes (nPTCL-TFH) and angioimmunoblastic T-cell lymphoma (AITL), as to know this rare tumor better. Methods: The clinical data, histopathology features, EBV positivity, T cell clonality and IDH2(R172) gene mutation in 8 cases of wPTCL-TFH were collected at the First Affiliated Hospital of Zhengzhou University from December 2015 to April 2019, and analyzed by immunohistochemistry, in situ hybridization, TCR gene rearrangement (BIOMED-2) and Sanger sequencing.Follow-up data were obtained by telephone. Results: There were 6 males and 2 females with a median age of 62.5 years (age ranging from 30 to 75 years). All patients had neither fever nor skin manifestations, but were all found mucosa thickened or mass of waldeyer's ring with multiple lymph nodes enlarged by PET-CT/CT scans. Five of the 7 patients were at advanced stages (Ⅲ/Ⅳ stage). Microscopically, the mucosa was infiltrated diffusely and characteristically by numerous small-medium sized lymphocytes, lacking polymorphous inflammatory background and extra-follicular expansion of follicular dendritic cell networks (FDC networks). The clear T cells presented in 5 cases. Ulcers on mucosal surfaces (6 cases) and local-extensive loss of intramucosal glands (7 cases) were commonly noted. Granulomas composed of epithelioid histiocytes were observed in 2 cases. Immunohistochemically, all the tumor cells expressed CD4 and at least 2 types of follicular helper of T cell (TFH) markers: PD-1 (8/8), bcl-6 (8/8), CXCL13 (7/8) and CD10 (1/8). Most of the cases (6 cases) expressed CD30. EBV positive appeared in 4 cases. All 8 cases were T cell monoclonal. IDH2(R172) were wild-type in 6 cases. One patient died at the follow-up time on 18 months; the other 7 survived (the follow-up time varied from 3 to 10 months). Conclusions: wPTCL-TFH is rare, and its clinicopathological features are similar to nPTCL-TFH which may be the manifestation of the same disease at different stage, and partly overlapped with AITL. The differential diagnosis from PTCL-NOS is necessary and comprehensive analyses of clinical, morphological, immunohistochemical and genetic features can help make a correct diagnosis.

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.

[Clinical outcomes of peripheral blood stem cell transplantation for aggressive peripheral T-cell lymphoma].

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.

[Prognostic value of lymphocyte-to-monocyte ratio in angioimmunoblastic T cell lymphoma].

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.

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.

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.