Pre-treatment [18F]FDG PET/CT for assessing bone marrow involvement and prognosis in patients with newly diagnosed peripheral T-cell lymphoma.

PURPOSE: To explore the value of [18F]fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT) in assessing bone marrow involvement (BMI) and prognosis in newly diagnosed peripheral T-cell lymphomas (PTCLs) before treatment. METHODS: This retrospective study included 201 eligible PTCLs who received pre-bone marrow biopsy (BMB) and PET/CT. The status of bone marrow (BM) by PET was assessed using a visual examination and a quantitative index (the maximal standardized uptake value [SUVmax] of BM divided by the SUVmax of the liver [M/L]). RESULTS: Totally 148 patients had no evidence of BMI by PET or BMB; BMI was detected by both methods in 16 patients. The sensitivity and specificity of PET/CT for patients with confirmed BMI by BMB were 43.2% and 90.2%, respectively (κ = 0.353). In addition, 25 patients assessed by PET/CT staging (having stage I to II disease) had no evidence of BMI detected by both PET/CT and BMB. Image-guided biopsy was also recommended when PET/CT showed a focal FDG uptake outside the iliac crest. Survival analysis revealed that BMB was significant for overall survival (OS) (P = 0.020) while M/L for both progression free survival (P = 0.002) and OS (P < 0.001). In multivariate analysis, M/L (HR 1.825, 95% CI 1.071-3.110, P = 0.027) was an independent prognostic factor for OS. There were no statistical differences at the genetic level about BMI confirmed by PET or BMB. CONCLUSION: PET/CT has a complementary role in assessing BMI and an ability to predict prognosis in PTCL patients.

Prognostic Significance Of Sequential 18f-fdg Pet/Ct During Frontline Treatment Of Peripheral T Cell Lymphomas.

BACKGROUND/AIMS: The prognostic significance of 18F-fluorodeoxyglucose (FDG)-positron emission tomography-computed tomography (PET/CT) in peripheral T-cell lymphomas (PTCLs) are controversial. We explored the prognostic impact of sequential 18F-FDG PET/CT during frontline chemotherapy of patients with PTCLs. METHODS: In total, 143 patients with newly diagnosed PTCLs were included. Sequential 18F-FDG PET/CTs were performed at the time of diagnosis, during chemotherapy, and at the end of chemotherapy. The baseline total metabolic tumor volume (TMTV) was calculated using the the standard uptake value with a threshold method of 2.5. RESULTS: A baseline TMTV of 457.0 cm3 was used to categorize patients into high and low TMTV groups. Patients with a requirehigh TMTV had shorter progression-free survival (PFS) and overall survival (OS) than those with a low TMTV (PFS, 9.8 vs. 26.5 mo, p = 0.043; OS, 18.9 vs. 71.2 mo, p = 0.004). The interim 18F-FDG PET/CT response score was recorded as 1, 2-3, and 4-5 according to the Deauville criteria. The PFS and OS showed significant differences according to the interim 18F-FDG PET/CT response score (PFS, 120.7 vs. 34.1 vs. 5.1 mo, p < 0.001; OS, not reached vs. 61.1 mo vs. 12.1 mo, p < 0.001). CONCLUSION: The interim PET/CT response based on visual assessment predicts disease progression and survival outcome in PTCLs. A high baseline TMTV is associated with a poor response to anthracycline-based chemotherapy in PTCLs. However, TMTV was not an independent predictor for PFS in the multivariate analysis.

Prognostic value of 18F-FDG lesion dissemination features in patients with peripheral T-cell lymphoma (PTCL).

PURPOSE: To explore the prognostic value of the distance between the two lesions that were farthest apart (Dmax) on baseline 18F-FDG PET/CT in peripheral T lymphoma (PTCL) and establish a new prognostic model for predicting the survival outcomes of patients with PTCL. METHODS: In this study, a retrospective analysis of 95 patients with PTCL who underwent baseline 18F-FDG PET/CT was performed to assess the predictive value of Dmax. The total metabolic tumour volume (TMTV), total lesion glycolysis (TLG), standardized uptake value (SUV), and Dmax were calculated with LIFEx software. Progression-free survival (PFS) and overall survival (OS) were used as endpoints. The prognostic model was developed based on the results of the multivariate analysis. The time-dependent area under the ROC curve (tdAUC), calibration curves, Harrell C-index, and decision curve analysis (DCA) were used to assess the model. RESULTS: Patients were followed up for a median of 17.0 months. Multivariate analysis showed that bone marrow biopsy (BMB) and Dmax were independent predictors of PFS (HR: 1.889, P = 0.039; HR: 1.965, P = 0.047) and OS (HR: 1.923, P = 0.031; HR: 1.982, P = 0.034). The model consisting of Dmax, TMTV, and BMB had substantial prognostic value for survival outcomes of PTCL and could successfully identify four groups of patients with significantly different prognoses (χ2 = 13.731, P = 0.003 for PFS; χ2 = 11.841, P = 0.008 for OS). The tdAUC, C-index, calibration curves, and DCA supported that the model was superior to the prognostic index for T-cell lymphoma (PIT) and International Prognostic Index (IPI) scores. CONCLUSION: BMB and Dmax were independent predictors of PTCL in our study. Moreover, a prognostic model based on the Dmax, TMTV, and BMB could be useful for predicting the survival outcomes of patients with PTCL.

The role of pre-treatment and mid-treatment 18F-FDG PET/CT imaging in evaluating prognosis of peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS).

BACKGROUND: It has been reported the prognostic value of MTV in predicting the disease prognosis of peripheral T-cell lymphoma (PTCL) through pre-treatment PET/CT imaging. However, these are limited data on pretreatment evaluation and prognosis assessments of peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS). This study aimed to determine the prognostic values of pre-treatment and mid-treatment total metabolic tumor volume (MTV), total lesion glycolysis (TLG), and Deauville 5-Point Scale (D-5PS) in accessing the prognosis of PTCL-NOS. METHODS: A retrospective analysis was conducted in 31 patients with pathologically diagnosed PTCL-NOS. These patients have undergone positron emission PET/CT scanning before and during chemotherapy. Follow-ups were also done to investigate the 2-year progression-free survival (PFS) and Overall Survival (OS) of these patients. During [Formula: see text]F-fluorodeoxyglucose ([Formula: see text]F-FDG) PET/CT scans, the MTV and TLG were recorded. Meanwhile, [Formula: see text]MTV and [Formula: see text]TLG were calculated. Furthermore, the receiver operating characteristic (ROC) curve was employed to classify and to define the threshold values. On the other hand, the mid-chemotherapy assessment and staging of these 31 patients were done by utilizing D-5PS. Subsequently, based on the D-5PS scores obtained, these patients were grouped into two categories: a group of patients with a score of <4 and another group with [Formula: see text]4 points. For these two groups of patients, the survival analysis was done by Kaplan-Meier analysis and a multivariate COX regression model. Moreover, Pearson's chi-square test ([Formula: see text] test) and Spearman rank correlation coefficient were used to comparing the collected data, respectively. RESULTS: During the 2-year follow-up period, 15 out of the 31 patients experienced disease progression. The optimal threshold values for both baseline MTV and TLG were 158.16 cm[Formula: see text] and 677.40.Additionally, the difference in 2-year PFS between the progressive and non-progressive groups was statistically significant ([Formula: see text], [Formula: see text]; [Formula: see text], [Formula: see text]), significant between-group difference was detected for MTV and for TLG ([Formula: see text], [Formula: see text]; [Formula: see text], [Formula: see text]). On the other hand, when these patients were classified into two groups according to the mid-chemotherapy Deauville score of <4 and [Formula: see text]4, the statistical difference of 2-year PFS between these two groups was significant, too ([Formula: see text], [Formula: see text]),but there is no significant between-group difference in OS ([Formula: see text], [Formula: see text]). COX analysis revealed that D-5PS are the independent factors influencing PFS, while MTV is the independent influencing factor of OS. CONCLUSION: The baseline total MTV obtained by PET/CT scanning, and D-5PS are crucial prognostic factors in evaluating the prognosis of PTCL-NOS.

Multiple osteolytic primary peripheral T-cell bone lymphoma: the first case report.

Peripheral T-cell lymphoma accounts for about 10% of all cases of non-Hodgkin's lymphoma. However, less than 5% of patients with non-Hodgkin's lymphoma present with hypercalcaemia as the initial symptom, and less than 1% present with primary bone lesions. We herein describe a 76-year-old Chinese man who was diagnosed with primary bone adult T-cell lymphoma with extensive osteolysis, including bone loss in the radius, as the initial manifestation. He had developed severe generalised bone pain and an inability to raise his arms. X-ray examination revealed osteolytic destruction of the forearm with loss of the radial diaphysis. The patient was diagnosed with peripheral T-cell lymphoma based on his immunohistochemical results. He began treatment with the CHOPE chemotherapy regimen, which resulted in significant improvement of his bone pain.

Primary soft tissue sarcoma: stage IV extranodal T-cell non-Hodgkin's lymphoma.

Non-Hodgkin's lymphoma (NHL) presenting as a soft tissue sarcoma is rare, occurring at a rate of 0.11%. Peripheral T-cell lymphomas comprise 4% of all NHLs. We report the case of a 49-year-old Caucasian man who presented with a mass in the medial aspect of the thigh. Ultrasound showed a complex subcutaneous mass. MRI demonstrated a superficial complex skeletal mass affecting the sartorius muscle with other lesions involving the femur and the gluteus maximus. Positron emission tomography-computed tomography (PET-CT) showed diffuse pulmonary metastases with no involvement of nodes, liver, spleen with high suspicion of advanced sarcoma. Core biopsy revealed a T-cell NHL, and staining was positive for anaplastic lymphoma kinase. The patient received six cycles of cyclophosphamide, doxorubicin, vincristine, etoposide and prednisone. Repeat PET-CT showed reduction in the mass, with no fluorodeoxyglucose-avid uptake. Latest MRI showed near-normal intensity. Further PET-CTs determine disease remission or progression.

The value of complete remission according to positron emission tomography prior to autologous stem cell transplantation in lymphoma: a population-based study showing improved outcome.

BACKGROUND: Chimeric antigen-receptor T-cell and bispecific antibody therapies will likely necessitate a reconsideration of the role of autologous stem-cell transplantation (ASCT) in lymphoma. Patients who are likely to profit from ASCT need to be better identified. METHODS: Here, we investigated the value of positron emission tomography/computerized tomography (PET/CT) before ASCT. All 521 patients transplanted for lymphoma 1994-2019 at Karolinska (497 conditioned with BEAM) were included. RESULTS: Outcome improved over three calendar periods 1994-2004, 2005-2014, 2015-2019 (2-year overall survival [OS]: 66, 73, 83%; P = 0.018). Non-relapse mortality (NRM) at 100 days over the three periods were 9.8, 3.9, 2.9%, respectively. The OS improvement between 1994 and 2004 and 2005-2014 was due to lower NRM (P = 0.027), but the large OS advance from 2015 was not accompanied by a significant reduction in NRM (P = 0.6). The fraction of PET/CT as pre-ASCT assessment also increased over time: 1994-2004, 2%; 2005-2014, 24%; 2015-2019, 60% (P < 0.00005). Complete responses (PET/CT-CR) were observed in 77% and metabolically active partial responses (PET/CT-PR) in 23%. PET/CT-CR was a predictor for survival in the entire population (P = 0.0003), also in the subpopulations of aggressive B-cell (P = 0.004) and peripheral T-cell (P = 0.024) lymphomas. Two-year OS and progression-free survival (OS/PFS) for patients in PET/CT-CR were in relapsed/refractory aggressive B-cell lymphoma 87%/75% and peripheral T-cell lymphoma 91%/78%. The corresponding figures in PET/CT-PR were 43%/44 and 33%/33%. Patients with solitary PET/CT-positive lesions showed acceptable outcome with ASCT followed by local irradiation (2-year OS/PFS 80%/60%). CT was less discriminative: 2-year OS/PFS: CT-CR, 76%/66%; CT-PR, 62%/51%. Outcome was inferior after BEAC compared with BEAM conditioning. CONCLUSIONS: We conclude that the improved outcome reflects better, PET/CT-informed, identification of patients who should proceed to ASCT. The excellent survival of patients in PET/CT-CR indicates that ASCT should remain part of standard therapy for lymphoma.

Esophageal peripheral T-cell lymphoma treated with radiotherapy: A case report.

RATIONALE: The clinical prognosis of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) patients is poor. Therefore, effective treatment is still a challenge at present. Moreover, little is known about the value of radiotherapy in the treatment of PTCL-NOS. PATIENT CONCERNS: A 55-year-old male patient with eating difficulties and progressive exacerbation for 3 months was diagnosed as non-Hodgkin's lymphoma. Airway compression occurred after 2 cycles of first line treatment with cyclophosphamide-Adriamycin-vincristine-prednisone regimen, radiotherapy (48Gy/24f) was given as the second line therapy. DIAGNOSIS: After radiotherapy, the patient complained that mild intermittent dysphagia still existed. Endoscopic biopsy of the upper digestive tract confirmed necrotic material and superficial squamous epithelial mucosa, suggesting esophageal stricture after radiotherapy, which was indistinguishable from tumor residue. INTERVENTIONS: The patient received anti-inflammatory treatment outside the hospital and did not receive any other special treatment. OUTCOMES: The symptoms of dysphagia disappeared and the focus showed complete response (CR). As of October 1, 2020, the patient has been diagnosed with PTCL-NOS for more than 57 months and the overall survival (OS) have not been achieved. LESSONS: Radiotherapy has obvious and rapid anti-tumor effect on cyclophosphamide-Adriamycin-vincristine-prednisone refractory PTCL-NOS. At the same time, hollow organs after radiotherapy can lead to lumen stenosis and the symptoms of suspected recurrence which is difficult to distinguish only from the imaging findings.

The role of PET in the first-line treatment of the most common subtypes of non-Hodgkin lymphoma.

This Review focuses on the use of 18F-fluorodeoxyglucose (18F-FDG) PET in the assessment of diffuse large B-cell lymphoma, follicular lymphoma, and peripheral T-cell lymphoma. PET is important for staging and prognostication with stage migration compared with CT. Better outcomes for patients with early stage diffuse large B-cell lymphoma and follicular lymphoma suggests better delineation of disease has translated to improved outcomes in such patients beyond simple stage migration. The aim of treatment of diffuse large B-cell lymphoma and peripheral T-cell lymphoma is potential cure, during which PET is mainly used to assess remission. Interim PET can assess chemosensitivity in these lymphomas, but it does not predict treatment success sufficiently well to enable treatment modification, particularly in the absence of more effective therapies for patients who remain PET-positive on interim scanning. In follicular lymphoma, traditionally viewed as an incurable lymphoma, the aim of treatment is to control disease for several years, while maintaining quality of life. PET can predict prognosis for patients with follicular lymphoma with high tumour burden at the end of induction chemotherapy, and it is being evaluated as a platform for response-adapted treatment of follicular lymphoma.

Diagnostic challenges of indolent peripheral T cell lymphoma: A case report and literature review.

INTRODUCTION: Peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) is a heterogeneous group of mature T cell lymphomas which do not correspond to any specific subtype of mature T-cell lymphoma in current classifications. Some researchers have suggested that PTCL with low Ki-67 labeling index should be classified as indolent PTCL PATIENT CONCERNS:: A 58-year old man diagnosed with alcoholic fatty liver 3 months prior complained of tenesmus and abdominal distension. Colonoscopy of the small and large intestines revealed multiple polyps, which were histologically diagnosed as lymphoid hyperplasia. One month later, he re-visited with a weight loss of 3 to 4 kg over 2 months. Radiologic examination revealed numerous small, homogenous, hypovascular lymph node enlargement in the para-aortic, mesenteric, and both inguinal areas, suggesting malignant lymphoma. DIAGNOSIS: Laparoscopic biopsy of an omental lymph node was performed, which was histologically confirmed as PTCL-NOS. INTERVENTIONS: The patient was administered 3 cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone, but his general condition did not improve. Therefore, treatment was changed to ifosfamide, carboplatin, and etoposide -dexamethasone (4 cycles) followed by allogeneic stem cell transplantation. OUTCOME: Even after allogeneic stem cell transplantation, fluorodeoxyglucose uptake in his abdominal lymph nodes and small bowel in positron emission tomography- computed tomography persisted at a Deauville score of 4. The patient has been followed-up for 2 years without progression. CONCLUSION: These indolent PTCLs histologically show diffuse infiltrated small lymphoid cells with low KI-67 labeling index and have a relatively good prognosis, although the epidemiology and pathogenesis are not fully elucidated. We report a case of indolent PTCL with cytogenetic abnormalities and poor response to chemotherapy, along with a brief review of the literature.

Prognostic Values of Baseline 18F-FDG PET/CT in Patients with Peripheral T-Cell Lymphoma.

PURPOSE: In the present study, we aimed to investigate whether the metabolic parameters on baseline 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) could be used to predict prognosis in peripheral T-cell lymphomas (PTCL). METHODS: A total of 51 nodal PTCL patients who underwent baseline 18F-FDG PET/CT were retrospectively evaluated in the present study. Total metabolic tumor volume (TMTV), total lesion glycolysis (TLG), and maximum standardized uptake value (SUVmax) were also assessed. Besides, the National Comprehensive Cancer Network International Prognostic Index (NCCN-IPI) was also included. Log-rank test and Cox regression analysis were used to evaluate progression-free survival (PFS) and overall survival (OS). RESULTS: The median follow-up was 18 months. Patients with low TLG, TMTV, and SUVmax levels had a significantly better clinical outcome than those with high TLG, TMTV, and SUVmax levels. The 2-year PFS rates of the high- and low-TMTV groups were 34.62% and 80%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (p < 0.001), whereas the corresponding 2-year OS rates were 46.15% and 84.00%, respectively (n = 10), intermediate-risk group with TMTV > 62.405 or NCCN-IPI score of 4-8 (2-year PFS and OS were 52.4% and 66.7%, respectively, n = 10), intermediate-risk group with TMTV > 62.405 or NCCN-IPI score of 4-8 (2-year PFS and OS were 52.4% and 66.7%, respectively, n = 10), intermediate-risk group with TMTV > 62.405 or NCCN-IPI score of 4-8 (2-year PFS and OS were 52.4% and 66.7%, respectively. CONCLUSIONS: Baseline TMTV and TLG were independent predictors of PFS and OS in PTCL patients, and SUVmax and NCCN-IPI scores were also independent predictors of OS. Moreover, the combination of TMTV and NCCN-IPI scores improved patient risk-stratification at the initial stage and might contribute to the adjustment of the therapeutic regime. This trial is registered with ChiCTR1900025526.

Baseline and interim PET-based outcome prediction in peripheral T-cell lymphoma: A subgroup analysis of the PETAL trial.

The prospective randomized Positron Emission Tomography (PET)-Guided Therapy of Aggressive Non-Hodgkin Lymphomas (PETAL) trial was designed to test the ability of interim PET (iPET) to direct therapy. As reported previously, outcome remained unaffected by iPET-based treatment changes. In this subgroup analysis, we studied the prognostic value of baseline total metabolic tumor volume (TMTV) and iPET response in 76 patients with T-cell lymphoma. TMTV was measured using the 41% maximum standardized uptake value (SUV41max ) and SUV4 thresholding methods. Interim PET was performed after two treatment cycles and evaluated using the ΔSUVmax approach and the Deauville scale. Because of significant differences in outcome, patients with anaplastic lymphoma kinase (ALK)-positive lymphoma were analyzed separately from patients with ALK-negative lymphoma. In the latter, TMTV was statistically significantly correlated with progression-free survival, with thresholds best dichotomizing the population, of 232 cm3 using SUV41max and 460 cm3 using SUV4 . For iPET response, the respective thresholds were 46.9% SUVmax reduction and Deauville score 1-4 vs 5. The proportion of poor prognosis patients was 46% and 29% for TMTV by SUV41max and SUV4 , and 29% and 25% for iPET response by ΔSUVmax and Deauville, respectively. At diagnosis, the hazard ratio (95% confidence interval) for poor prognosis vs good prognosis patients according to TMTV was 2.291 (1.135-4.624) for SUV41max and 3.206 (1.524-6.743) for SUV4 . At iPET, it was 3.910 (1.891-8.087) for ΔSUVmax and 4.371 (2.079-9.187) for Deauville. On multivariable analysis, only TMTV and iPET response independently predicted survival. Patients with high baseline TMTV and poor iPET response (22% of the population) invariably progressed or died within the first year (hazard ratio, 9.031 [3.651-22.336]). Due to small numbers and events, PET did not predict survival in ALK-positive lymphoma. Baseline TMTV and iPET response are promising tools to select patients with ALK-negative T-cell lymphoma for early allogeneic transplantation or innovative therapies.

Prognostic significance of FDG-PET/CT in determining upfront autologous stem cell transplantation for the treatment of peripheral T cell lymphomas.

This observational study aimed to evaluate the prognostic significance of interim and final 18F-fluorodeoxyglucose positron emission tomography-computed tomography (PET/CT) responses to upfront autologous stem cell transplantation (ASCT) in patients with peripheral T cell lymphomas (PTCLs). A total of 118 patients, from two independent institutions, with newly diagnosed PTCLs were enrolled, and 96 of them were evaluated. PET/CT was assessed at diagnosis, and during and after the primary treatment. Clinical outcomes of interim and final PET/CT were compared between transplanted and non-transplanted patients. The responses of PET/CT were assessed based on visual analysis using the Deauville five-point scale (5-PS). Clinicopathological features of transplanted patients (n = 37) were similar to those of non-transplanted patients (n = 59). After a median follow-up of 60.8 months, only final PET/CT response based on 5-PS was the independent prognostic factor of survival outcome (P < 0.001; HR 8.215; 95% C.I. 2.97-22.72) in multivariate analysis. Interim PET/CT response did not have a differential potential for predicting progression-free survival (PFS). In 59 patients, with score 1 or 2 in final PET/CT, the PFS rate was not significantly different between transplanted and non-transplanted patients (P = 0.970). Moreover, among the 37 patients with final PET/CT response score of 3-4, the PFS rate was equally poor in both transplanted and non-transplanted patients (P = 0.178). Final PET/CT assessment, based on 5-PS, was an important prognostic parameter for primary treatment of PTCLs, regardless of upfront ASCT. Interim PET/CT response could not be an indicator to determine the requirement for upfront ASCT.

NK/T-cell lymphomas.

NK/T-cell lymphomas are extranodal EBV-related malignancies, mostly of NK-cell and occasionally of T-cell lineage. They are divided into nasal, non-nasal, and disseminated subtypes. Nasal NK/T-cell lymphomas involve the nose, nasopharynx and the upper aerodigestive tract. Non-nasal NK/T-cell lymphomas involve the skin, gastrointestinal tract, testis and other sites. Disseminated NK/T-cell lymphoma involves multiple organs, and may present with a leukemic phase. Initial evaluation requires positron emission tomography computed tomography (PET/CT) and quantification of circulating EBV DNA. Radiotherapy alone is inadequate with frequent relapses. Anthracycline-containing regimens are ineffective. Regimens incorporating asparaginase are currently the standard. For stage I/II disease, combined chemotherapy and radiotherapy is recommended. For stage III/IV disease, asparaginase-containing regimens are needed. Autologous hematopoietic stem cell transplantation (HSCT) is of limited efficacy, whereas allogeneic HSCT may be useful in patients with stage III/IV and relapsed diseases. Immunotherapy with antibodies against CD30, programmed cell death protein 1 and CD38 is promising.

Structural and diffusion weighted MRI demonstrates responses to ibrutinib in a mouse model of follicular helper (Tfh) T-cell lymphoma.

Recent analyses of the genetics of peripheral T-cell lymphoma (PTCL) have shown that a large proportion of cases are derived from normal follicular helper (Tfh) T-cells. The sanroque mouse strain bears a mutation that increases Tfh cell number and heterozygous animals (Roquinsan/+) develop lymphomas similar to human Tfh lymphoma. Here we demonstrate the usefulness of Roquinsan/+ animals as a pre-clinical model of Tfh lymphoma. Long latency of development and incomplete penetrance in this strain suggests the lymphomas are genetically diverse. We carried out preliminary genetic characterisation by whole exome sequencing and detected tumor specific mutations in Hsp90ab1, Ccnb3 and RhoA. Interleukin-2-inducible kinase (ITK) is expressed in Tfh lymphoma and is a potential therapeutic agent. A preclinical study of ibrutinib, a small molecule inhibitor of mouse and human ITK, in established lymphoma was carried out and showed lymphoma regression in 8/12 (67%) mice. Using T2-weighted MRI to assess lymph node volume and diffusion weighted MRI scanning as a measure of function, we showed that treatment increased mean apparent diffusion coefficient (ADC) suggesting cell death, and that change in ADC following treatment correlated with change in lymphoma volume. We suggest that heterozygous sanroque mice are a useful model of Tfh cell derived lymphomas in an immunocompetent animal.

Diagnostic and Prognostic Value of Using 18F-FDG PET/CT for the Evaluation of Bone Marrow Involvement in Peripheral T-Cell Lymphoma.

PURPOSE: No study has analyzed a sizeable cohort that comprised solely peripheral T-cell lymphoma (PTCL) patients for the diagnostic and prognostic performance of F-FDG PET/CT for bone marrow (BM) involvement. This study aimed to investigate the utility of PET/CT for the identification of BM involvement and to explore its prognostic relevance in patients with PTCL. METHODS: Eighty-three consecutive patients with newly diagnosed PTCL were enrolled in this retrospective study. The diagnosis of BM involvement was confirmed on the basis of positive BM histology or disappearance of marrow abnormalities on follow-up PET/CT concurrently with a successful treatment response. RESULTS: Of 28 patients with confirmed BM involvement, BM biopsy (BMB) and PET/CT detected an involvement in 17 and 25 patients. Among 66 patients with negative BM histology, 11 patients had BM involvement detected by PET/CT and furthermore showed significantly shorter progression-free and overall survival than patients without BM involvement. We recategorized the International Prognostic Index (IPI) risk groups based on the presence of BM involvement using the combined assessment of iliac crest marrow biopsy histology and PET/CT (PET/BMB-based IPI). PET/BMB-based IPI tended to perform better than conventional BMB-based IPI. CONCLUSIONS: This study included the largest population of PTCL patients for PET/CT evaluation of BM involvement so far. PET/CT exhibited a higher sensitivity for BM involvement than BMB. Furthermore, BM assessment using PET/CT identified patients at high risk of disease progression and mortality among those with negative BM histology, suggesting that PET/CT may have a potential to improve existing prognostic strategies in PTCL.

CT findings predict survival of patients with peripheral T cell lymphoma: a preliminary study.

Background Peripheral T-cell lymphoma (PTCL) is an uncommon disease with poor clinical outcomes. Radiological reports on the survival of patients with PTCL are scarce. The purpose of this study is to investigate the prognostic value of CT findings to predict clinical outcomes in fifty-one patients with histologically proven PTCL. Patients and methods The clinical data and CT images of all patients were retrospectively reviewed. CT features including number of involvement sites, lesion size, shape, margin, density, peritumoral invasion, intratumoral necrosis, lymph node involvement, and degree of contrast enhancement were evaluated. Univariate and multiple logistic regression analysis were used to determine the association between the clinical outcome and radiologic factors. Results Multiple site involvement, an ill-defined margin with peritumoral invasion, inhomogeneous density, and intratumoral necrosis were found to be associated with poor outcomes in univariate analysis (P < 0.05). An ill-defined margin with peritumoral invasion, was identified as an independent risk sign by further multivariate logistic regression analysis (P < 0.05). The area under the ROC curve of this CT feature was 0.745 (P < 0.05). Conclusions An ill-defined margin with peritumoral invasion was a valuable prognostic factor to predict the worse clinical outcomes in patients with PTCL.