scholarly journals Non-Hodgkin Lymphoma in Children with Primary Immunodeficiencies: Clinical Manifestations, Diagnosis, and Management, Belarusian Experience

Lymphoma ◽  
2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Alina Fedorova ◽  
Svetlana Sharapova ◽  
Taisia Mikhalevskaya ◽  
Svetlana Aleshkevich ◽  
Inna Proleskovskaya ◽  
...  
Keyword(s):  
Hodgkin Lymphoma ◽  
Clinical Manifestations ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
Cell Types ◽  
Nijmegen Breakage Syndrome ◽  
Primary Immune Deficiency ◽  
Chromosomal Breakage ◽  
Hematopoietic Stem ◽  
Non Hodgkin Lymphoma

Introduction. Non-Hodgkin lymphoma (NHL) is the most frequent malignancy associated with primary immune deficiency disease (PID). We aimed to present the clinical characteristics and outcomes of Belarusian children with PID who developed NHL. Procedure. We reviewed 16 patients with PID and NHL. Eight patients had combined PID: 5—Nijmegen breakage syndrome, 1—Bloom syndrome, 1—Wiskott-Aldrich syndrome, and 1—Х-linked lymphoproliferative syndrome. Results. In 75% cases PID was diagnosed simultaneously or after the NHL was confirmed. PID-associated NHL accounted for 5.7% of all NHL and was characterized by younger median age (6.3 versus 10.0 years, P<0.05) and by prevalence of large-cell types (68.8% versus 24.5%, P<0.001). Children with combined PID had median age of 1.3 years; 5 of them developed EBV-associated diffuse large B-cell lymphoma with lung involvement. Five of 6 patients with chromosomal breakage syndrome developed T-NHL. Six patients died of infections; two died after tumor progression; one child had early relapse; two died of second NHL and one of secondary hemophagocytic syndrome. Overall, 4 children are alive and disease-free after a follow-up from 1.4 to 5.7 years. Conclusions. PID needs to be diagnosed early. Individualized chemotherapy, comprehensive supportive treatment, and hematopoietic stem cell transplantation may improve survival of children with PID and NHL.

scholarly journals Improved Outcome of Relapsed or Refractory Pediatric B-Cell Non-Hodgkin Lymphoma in Japan

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5078-5078
Author(s):  
Tomoo Osumi ◽  
Tetsuya Mori ◽  
Naoto Fujita ◽  
Akiko M. Saito ◽  
Atsuko Nakazawa ◽  
...  
Keyword(s):  
Overall Survival ◽  
Survival Rate ◽  
B Cell ◽  
Hodgkin Lymphoma ◽  
Salvage Therapy ◽  
Combined Treatment ◽  
B Cell Lymphoma ◽  
Overall Survival Rate ◽  
Hematopoietic Stem ◽  
Non Hodgkin Lymphoma

Abstract Introduction Relapsed or refractory pediatric B-cell non-Hodgkin lymphoma (B-NHL) has been reported to be extremely difficult to cure. We previously conducted a retrospective study of pediatric relapsed or refractory B-NHL in Japan between 1996 and 2004 and found that the four-year overall survival rate was only 20.5% (Fujita N. e al. PBC. 2008). Rituximab, a chimeric anti-CD20 monoclonal antibody, is expected to be effective in improving the prognosis of pediatric relapsed or refractory B-NHL and was approved for use in 2003 in Japan. Here, we retrospectively assessed the treatment and prognosis of pediatric relapsed or refractory pediatric B-NHL in the rituximab era in Japan. Methods We collected relapsed pediatric B-NHL cases from patients enrolled in our current study, JPLSG B-NHL03 study, which was the first nationwide multicenter trial for newly diagnosed pediatric B-NHL. We collected information on treatment and outcome after induction failure or relapse. Results In 33 patients enrolled this study, the median age at diagnosis was 9.7 years and male predominance was observed. Nine cases were pathologically subclassified initially as diffuse large B-cell lymphoma, 11 as Burkitt lymphoma, 11 as Burkitt Leukemia and two as others. According to initial Murphy staging, most cases were in the advanced stage. The most common site of relapse was abdomen (8 cases), followed by bone marrow (8 cases) and central nervous system (7 cases). Twenty-three cases relapsed in one site while 10 did so in multiple sites. Among them, 20 relapsed within six months after initial diagnosis. Among them, 28 patients received some rituximab combined treatment as salvage therapy. R-ICE (rituximab, ifosfamide, carboplatin and etoposide) regimen was the most popular and used in 22 patients as first-line salvage therapy. As a result, 22 patients (66.7%) achieved complete remission (CR) by some salvage therapy. 23 received hematopoietic stem cell transplantation (HSCT). Their 5-year overall survival rate was 48.5%, which was far more superior to both our previous study (Figure 1) and another on relapsed or refractory B-NHL in childhood. In risk factor analysis for survival, rituximab combined treatment and HSCT did not influence the outcome, but achievement of CR after salvage treatment resulted in significantly better survival (68.2% vs 9.1%, p=0.001). These results suggest that the advent of rituximab induced an improvement of CR rate for relapsed paediatric B-NHL, which resulted in an improvement of the survival rate. Conclusion In conclusion, the prognosis of the pediatric relapsed or refractory B-NHL in a Japanese cohort remained poor but is showing improvement in the rituximab era. Disclosures No relevant conflicts of interest to declare.

Gray Zone Lymphomas: Clinical and Histological Characteristics and Treatment with Dose-Adjusted-EPOCH-R

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3590-3590 ◽  
Author(s):  
Kieron Dunleavy ◽  
Stefania Pittaluga ◽  
Nicole Grant ◽  
Seth Steinberg ◽  
Margaret Shovlin ◽  
...  
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Cell Lymphoma ◽  
Treatment Strategies ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
Gray Zone ◽  
Non Hodgkin Lymphoma ◽  
Histological Characteristics

Abstract Gray zone lymphomas (GZL) are diseases with transitional morphology and immunophenotypic features between Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Their pathological and clinical characteristics are not well studied and the best treatment strategy (using HL or DLBCL regimens) has not been defined. Small previous series of HL-like ALCL, which would include GZL, suggest they have a poor outcome with HL treatments. We present GZL’s treated on studies of DA-EPOCH-R at the National Cancer Institute and describe their clinical and histological features and outcome. Overall, 14 patients with GZL were identified. Characteristics included median (range) age 30 (12–51) years; male sex 10 (71%); stage III/IV 2 (14%) and; elevated LDH 7 (50%). These cases could be divided into three Gray zone groups: classical HL (cHL) and primary mediastinal B-cell lymphoma (PMBL) in 9 (64%) patients; cHL and DLBCL in 2 (14%) and; lymphocyte predominant HL (LPHL) and T-cell histiocyte-rich large cell lymphoma (TCRBCL) in 2 (14%). Pathological characteristics are shown below. All but one case was CD 10 negative. Markers of cHL included CD15 in 33–50% and CD30 in 66–100% of cases. Morphologically, Reed-Sternberg like cells were typically seen in GZL with cHL features. Thirteen newly diagnosed patients received DA-EPOCH-R. Of 11 patients evaluable for response (2TE), 10 (91%) achieved CR and 1 PR. At a median follow-up time of 4 years, OS and PFS are is 86% and 57%, respectively. Of 9 patients with GZL between cHL and PMBL, 4 (44%) also required radiation therapy compared to only 3/31 (10%) patients with PMBL to achieve durable remissions. Gray zone lymphomas represent a biological and clinical continuum between HL and B-cell lymphomas. Clinically, they appear to be more resistant to treatment than either HL or DLBCL and may require aggressive treatment strategies including radiation. Accrual continues. Gray Zone Total 14 CD 20 CD 15 CD 30 cHL- PMBL 9 8 (89%) 7 (50%) 9 (100%) cHL-DLBCL 2 2 (100%) 1 (50%) 2(100%) LPHL-TCRBCL 3 3 (100%) 1 (33%) 2 (66%)

Clinical Applications of the Genomic Landscape of Aggressive Non-Hodgkin Lymphoma

2017 ◽  
Vol 35 (9) ◽  
pp. 955-962 ◽  
Author(s):  
Andrea B. Moffitt ◽  
Sandeep S. Dave
Keyword(s):  
T Cell ◽  
B Cell ◽  
Hodgkin Lymphoma ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Adult T Cell Leukemia ◽  
Non Hodgkin Lymphoma ◽  
T Cell Lymphomas

In this review, we examine the genomic landscapes of lymphomas that arise from B, T, and natural killer cells. Lymphomas represent a striking spectrum of clinical behaviors. Although some lymphomas are curable with standard therapy, the majority of the affected patients succumb to their disease. Here, the genetic underpinnings of these heterogeneous entities are reviewed. We consider B-cell lymphomas, including Burkitt lymphoma, diffuse large B-cell lymphoma, Hodgkin lymphoma, and primary mediastinal B-cell lymphoma. We also examine T-cell lymphomas, including anaplastic large-cell lymphoma, angioimmunoblastic T-cell lymphoma, cutaneous T-cell lymphoma, adult T-cell leukemia/lymphoma, and other peripheral T-cell lymphomas. Together, these malignancies make up most lymphomas diagnosed around the world. Genomic technologies, including microarrays and next-generation sequencing, have enabled a better understanding of the molecular underpinnings of these cancers. We describe the broad genomics findings that characterize these lymphoma types and discuss new therapeutic opportunities that arise from these findings.

scholarly journals Outcome and survival in children with Non-Hodgkin’s lymphoma

2019 ◽  
Vol 7 (1) ◽  
Author(s):  
Tahani Awad Elkarim Elfadl ◽  
Ibrahim Abosoudah ◽  
Mohammed Bayoumy ◽  
Ali Al Harbi ◽  
Muhammad Matloob Alam ◽  
...  
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
Lymphoblastic Lymphoma ◽  
Non Hodgkin Lymphoma ◽  
Large B Cell Lymphoma ◽  
King Faisal Specialist Hospital ◽  
Large Cell Lymphoma

Background Non-Hodgkin lymphoma is the third most common malignant tumor in children. It includes four major subtypes: Burkitt Lymphoma (BL), Lymphoblastic Lymphoma (LL), Diffuse Large B-cell Lymphoma (DLBL) and Anaplastic Large Cell Lymphoma (ALCL). The use of multidrug chemotherapy, radiation therapy, biologic agents, and improved diagnostic and supportive care resulted in better cure rates. Objective This study is to report prognosis and outcome of Non-Hodgkin lymphoma (NHL) patients at tertiary health care facility in King Faisal Specialist Hospital and Research Center, Jeddah (KFSHRC-J). Materials and Method A retrospective cross-sectional study of all eligible patients with Non-Hodgkin lymphoma (NHL), admitted, diagnosed and managed at King Faisal Specialist Hospital and Research Center, Jeddah from Jan 2005 to December 2016, previously untreated, with biopsy proven NHL and Age ≤ 15 years at diagnosis. Clinical data Research Form used to collect patient’s data from medical records. Demographic, Clinical and Survival data analysed using Statistical Package for Social Sciences. Results Thirty-one pediatric patients with biopsy proven Non-Hodgkin lymphoma (NHL) fulfilled the inclusion criteria. Twenty-six (80.6%) were males. Nineteen (61.3%) patients were ≤ 10 years of age at diagnosis, while 12 (38.7%) were>10 years of age. The mean age at diagnosis was 8.1years. The commonest primary site is abdomen (n=19, 61.3%), followed by Head & Neck (n=9, 28.1%), mediastinum (n=1, 3.1%), primary CNS (n=1, 3.1%), bone (n=1, 3.1%) and skin (n=1, 3.1%). Regarding histology 19 (61.3%) had Burkitt Lymphoma (BL), 6 (19.4%) had Diffuse Large B-cell Lymphoma (DLBL), 2(6.4%) had T-cell Lymphoblastic Lymphoma, 2 (6.4%) had T-cell rich B Cell Lymphoma, 1 (3.1%) had B-cell Lymphoma not otherwise specified and 1 (3.1%) had Cutaneous Anaplastic Large Cell Lymphoma (ALCL). Predominantly, patients presented in advanced stages III (n=18, 60%) and IV (n=10, 33%).Twenty-five (77.8%) patients completed treatment and are well to date while six of the patients (18.6%) died during the study period. Conclusion Children admitted to the (KFSHRC-J) appeared affected by non-Hodgkin Lymphoma at a younger age, with a higher incidence of Burkitt's Lymphoma. The predominant presenting site is abdomen followed by head/neck. They present mostly with advance disease. Survival rates are similar to those described in the literature of developed countries.

Non-Hodgkin Lymphomas in Children and Adolescents

2020 ◽  
pp. 142-156
Author(s):  
Véronique Minard-Colin ◽  
Catherine Patte
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Burkitt Lymphoma ◽  
Cell Lymphoma ◽  
Survival Rates ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
Lymphoblastic Lymphoma ◽  
Non Hodgkin Lymphoma ◽  
Innovative Therapies

Non-Hodgkin lymphoma (NHL) is the fourth most common malignancy in children, with an even higher incidence in adolescents, and is primarily represented by only a few histological subtypes. Dramatic progress has been achieved, with survival rates exceeding 80%. Most patients with Burkitt lymphoma and diffuse large B-cell lymphoma are cured with short, intensive, pulse chemotherapy. The benefit of the addition of rituximab has been demonstrated for high-risk B-NHL and primary mediastinal B-cell lymphoma. Lymphoblastic lymphoma is treated with intensive, semi-continuous, longer ‘leukaemia-derived’ protocols. Relapses in B-cell and lymphoblastic lymphomas are rare and infrequently curable, even with intensive approaches. Event-free survival rates of about 75% have been achieved in anaplastic large-cell lymphomas with various regimens, including generally a short, intensive ‘B-like’ regimen. The role of immunity appears important in prognosis and needs further exploration in therapy. Anaplastic lymphoma kinase (ALK) inhibitor therapeutic approaches are currently being investigated. For all these paediatric lymphomas, the intensity of induction/consolidation treatments correlates with a high rate of immediate toxicities, but due to low cumulative doses of anthracyclines and alkylating agents, minimal or no long-term toxicity is expected. Challenges that remain include defining the value of prognostic factors, such as early response on positron emission tomography (PET)/computed tomography (CT) and monitoring of minimal disseminated and residual disease, utilizing new biological technologies to improve risk stratification and the development of innovative therapies, both at frontline and relapse. non-Hodgkin lymphoma, NHL, European Intergroup for Childhood NHL, EICNHL, Burkitt lymphoma, anaplastic large-cell lymphoma, ALK, lymphoblastic lymphoma

Single Institution Experience with Y-90 Ibritumomab Tiuxetan in the Treatment of Non-Hodgkin Lymphoma.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4699-4699
Author(s):  
Peter Martin ◽  
Rene Michel ◽  
Ahmed Galal
Keyword(s):  
Hodgkin Lymphoma ◽  
Median Time ◽  
B Cell Lymphoma ◽  
Similar Response ◽  
Cell Transplant ◽  
Single Institution ◽  
Ibritumomab Tiuxetan ◽  
Hematopoietic Stem ◽  
Platelet Recovery ◽  
Non Hodgkin Lymphoma

Abstract Background: Radioimmunotherapy (RIT) is an effective treatment of Non-Hodgkin Lymphoma (NHL). Nonetheless, the use of RIT outside of clinical trials has been limited. We report here the experience in 25 patients treated with Y-90 ibritumomab tiuxetan off-protocol at a single institution. Patients and Methods: The charts of all patients treated with RIT between October, 2004 and May, 2006 were retrospectively reviewed in accordance with Good Clinical Practice guidelines. 19 patients were treated with RIT alone while 6 were treated with RIT in combination with high-dose chemotherapy and autologous hematopoietic stem cell transplant (HSCT). Dosimetry was not performed. Results: The median age of patients was 56 years (range 43–71 years). 13 of 25 had high-grade lymphoma (either transformed lymphoma or diffuse large B-cell lymphoma). The median IPI was 3. The median number of prior regimens was 3 (range 1–6). 6 patients had received fludarabine. 3 patients had undergone prior autologous HSCT and one had undergone allogeneic HSCT. The median time to platelet recovery &gt;20 × 109/L was 35 days. One patient continues to have platelets &lt;20 × 109/L 380 days post-RIT. The median time to recover platelets to &gt;20 × 109/L was 72 among patients who had previously received fludarabine. Prior HSCT also appeared to be associated with prolonged thrombocytopenia. 7 patients remained neutropenic (ANC&lt;0.5 × 109/L) for &gt;30 days. One patient who had been treated with 4 lines of prior chemotherapy, including prolonged chlorambucil, developed acute myeloid leukemia at 6 months post-RIT. There have been 3 deaths to date, all due to disease progression at 1, 1, and 3 months post-RIT. The overall response rate in 21 of 25 patients was 84% with 9 patients achieving CR. With a median follow-up of 7 months, 5 patients have progressed. Conclusion: The use of off-protocol RIT in our institution was associated with similar response rates to those reported in patients treated on-protocol. Off concern, however, is the significant hematologic toxicity. There was an association between prior fludarabine and prolonged thrombocytopenia that has not been reported previously.

scholarly journals Geographical Distribution, Incidence, Malignancies, and Outcome of 136 Eastern Slavic Patients With Nijmegen Breakage Syndrome and NBN Founder Variant c.657_661del5

2021 ◽  
Vol 11 ◽  
Author(s):  
Svetlana O. Sharapova ◽  
Olga E. Pashchenko ◽  
Anastasiia V. Bondarenko ◽  
Svetlana S. Vakhlyarskaya ◽  
Tatjana Prokofjeva ◽  
...  
Keyword(s):  
Overall Survival ◽  
Hot Spot ◽  
Survival Rates ◽  
B Cell Lymphoma ◽  
Adult Survivors ◽  
Nijmegen Breakage Syndrome ◽  
Hematopoietic Stem ◽  
Delay In Diagnosis ◽  
Non Hodgkin Lymphoma ◽  
Base Pair Deletion

Nijmegen breakage syndrome (NBS) is a DNA repair disorder characterized by combined immunodeficiency and a high predisposition to lymphoid malignancies. The majority of NBS patients are identified with a homozygous five base pair deletion in the Nibrin (NBN) gene (c.657_661del5, p.K219fsX19) with a founder effect observed in Caucasian European populations, especially of Slavic origin. We present here an analysis of a cohort of 136 NBS patients of Eastern Slav origin across Belarus, Ukraine, Russia, and Latvia with a focus on understanding the geographic distribution, incidence of malignancy, and treatment outcomes of this cohort. Our analysis shows that Belarus had the highest prevalence of NBS (2.3 per 1,000,000), followed by Ukraine (1.3 per 1,000,000), and Russia (0.7 per 1,000,000). Of note, the highest concentration of NBS cases was observed in the western regions of Belarus and Ukraine, where NBS prevalence exceeds 20 cases per 1,000,000 people, suggesting the presence of an “Eastern Slavic NBS hot spot.” The median age at diagnosis of this cohort ranged from 4 to 5 years, and delay in diagnosis was more pervasive in smaller cities and rural regions. A total of 62 (45%) patients developed malignancies, more commonly in males than females (55.2 vs. 34.2%; p=0.017). In 27 patients, NBS was diagnosed following the onset of malignancies (mean age: 8 years). Malignancies were mostly of lymphoid origin and predominantly non-Hodgkin lymphoma (NHL) (n=42, 68%); 38% of patients had diffuse large B-cell lymphoma. The 20-year overall survival rate of patients with malignancy was 24%. However, females with cancer experienced poorer event-free survival rates than males (16.6% vs. 46.8%, p=0.036). Of 136 NBS patients, 13 underwent hematopoietic stem cell transplantation (HSCT) with an overall survival of 3.5 years following treatment (range: 1 to 14 years). Indications for HSCT included malignancy (n=7) and immunodeficiency (n=6). Overall, 9% of patients in this cohort reached adulthood. Adult survivors reported diminished quality of life with significant physical and cognitive impairments. Our study highlights the need to improve timely diagnosis and clinical management of NBS among Eastern Slavs. Genetic counseling and screening should be offered to individuals with a family history of NBS, especially in hot spot regions.

scholarly journals LIMFOMA NON-HODGKIN SEL B PADA MAMMAE

2019 ◽  
Vol 8 (1S) ◽  
pp. 69
Author(s):  
Ninda Septia Yuspar ◽  
Irza Wahid
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
Non Hodgkin Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Limfoma mammae merupakan kasus yang jarang karena jaringan limfoid tidak ada di regio mammae. Subtipe non-Hodgkin lymphoma (NHL) berkisar 0,5% dari karsinoma mammae, 1% dari semua NHL dan 2% dari limfoma ekstranodal. Limfoma mammae sering terjadi pada perempuan. Limfoma Mammae diklasifikasikan sebagai limfoma primer dan sekunder. Limfoma mamae primer biasanya non-Hodgkin jenis sel B, angka kejadian terbanyak adalah sub tipe Difusse large cell B Limfoma. Perempuan, 45 Tahun, keluhan benjolan pada leher, payudara dan ketiak`sejak 6 bulan yang lalu, Pasien telah didiagnosis Limfoma malignum non hodgkin 10 bulan sebelumnya, penurunan berat badan sekitar 15 kg sejak 4 bulan yang lalu dan didapatkan keluhan demam tidak tinggi yang hilang timbul. Pasien menjalani kemoterapi Rituximab, Cylophosphamide, Hydroxydaunorubicin, Oncovin, Prednison dan benjolan mengecil. Pemeriksaan fisik ditemukan benjolan di kedua mammae, colli sinister, axila sinistra dan inguinal, tidak eritem, konsistensi kenyal padat, terfiksir dan tidak nyeri tekan. Laboratorium, leukopenia dan LDH meningkat. USG mammae, Multipel nodul kedua mammae, axila bilateral, mammary interna kiri. USG colli, multipel limfadenopati regio colli, supraclavicula sinistra gambaran limfoma. Histopatologi, Limfoma malignan mammae bilateral. Pada pemeriksaan Imunohistokimia dengan hasil diffuse large B cell lymphoma, CD20 positif, Non GCB. Pasien didiagnosis Limfoma non hodgkin pada mammae relaps. Diberikan kemoterapi Rituximab, Etoposide, Actoplactin, Ifosfamide. Limfoma non-Hodgkin primer pada mammae termasuk kasus yang sangat jarang terjadi. Manifestasi klinis dan radiologis dari penyakit ini memiliki kesamaan dengan tumor mammae. Diagnosis penyakit ini ditegakkan melalui histopatologi serta pemeriksaan imunohistokimia. Penatalaksanaan PBL yaitu kemoterapi dengan atau tanpa rituximab dan radioterapi. Pada Pasien ini terjadi Limfoma Non-hodgkin pada Mammae Relaps selanjutnya diberikan kemoterapi lini kedua dengan regimen Rituximab, Etoposide, Actoplactin, Ifosfamide. memberikan hasil yang cukup baik.

scholarly journals Nivolumab Induces Remission in High- PD-L1 Expressing Aggressive B-Non Hodgkin Lymphoma: A Single Center Experience

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1865-1865 ◽  
Author(s):  
Stephan R Bohl ◽  
Stefan Schoensteiner ◽  
Henriette Huber ◽  
Florian Kuchenbauer ◽  
Peter Moeller ◽  
...  
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
B Cell Lymphoma ◽  
Response Assessment ◽  
Grey Zone ◽  
Phase Ii Trials ◽  
Hematopoietic Stem ◽  
Allogeneic Hsct ◽  
Non Hodgkin Lymphoma ◽  
Number Of Patients

Abstract Patients with relapsed or refractory aggressive B-cell Non-Hodgkin lymphoma (B-NHL) have a poor outcome. Nivolumab (NV) targeting the PD-1 receptor has shown promising results in several malignancies like melanoma, renal and lung carcinoma and Hodgkin lymphoma. Data for aggressive B-NHL are just emerging. PD-L1 expression does not necessarily correlate with response. Among lymphoma subtypes, the expression of PD-L1 seems to vary. So far there are no valid data on PD-L1 expression in aggressive lymphoma under NV treatment. We retrospectively analyzed seven patients who had aggressive B-NHL with a refractory or relapsed disease after at least 3 regimens and were thereupon treated with NV 3mg/kg once a fortnight. Treatment was initiated after signed informed consent for off-label use. The histologic specimens were analyzed for PD-L1 expression by immunohistochemistry (IHC). The median age at NV treatment start was 51 years (27-71). The histopathological diagnoses were primary mediastinal B-cell lymphoma (PMBL; n=2), composite lymphoma with diffuse large B-cell (DLBCL) and Hodgkin lymphoma components (n=2), follicular lymphoma transformed into a DLCBL (n=2) and one mediastinal grey zone lymphoma (MGZL). A median of 5 (3-7) prior therapy regimens were given including hematopoietic stem cell transplantation (HSCT) for 3 patients: autologous (n=2) and allogeneic HSCT (n=1), respectively. The lymphoma status was refractory in 2 patients and relapsed in 5 patients. PD-L1 expression was detected in 5 cases, 3 of them showing a positive staining of 10%, 30%, and 50%, respectively, and 2 cases even of 100% of the lymphoma cells. NV was mostly well tolerated (1 pneumonitis completely resolved after systemic steroid therapy, 1 unrelated death caused by septic shock) with a median of 3 (1-17) cycles administered. Response assessment (clinical examination, ultrasound, CT or PET-CT scans) were performed after a median of 3 cycles (2-7), showing regressive and refractory disease in 3 patients, respectively, while 1 patient died prior to response assessment. Among the three responders (2xCR, 1xPR) MGZL and PBML their tumors had high PD-L1 expression of 30% (n=1) and 100% (n=2), respectively. Two responders (one after autologous, one after allogeneic HSCT) are still under treatment. After 14 weeks, one responding patient showed signs of progression of a previously known cerebral lesion (or pseudo progression under immunotherapy). The third responder proceeded to allogeneic HSCT without relevant complications. The PD-L1 expression among the non-responders were 0% (n=2) with progressive disease being the cause of death and 10% (n=1) for the other who is currently receiving alternative therapy. In conclusion, NV induces remission in heavily pretreated, PD-L1 high expression aggressive B-NHL. The significance of these data are limited due to the small number of patients. Therefore, future results of current phase II trials (NCT02038933) will reveal the role of PD-1 blockade in aggressive B-NHL. Disclosures Viardot: Roche: Honoraria; Takeda: Other: travel support; Pfizer: Honoraria; BMS: Consultancy; Janssen: Consultancy; Amgen: Consultancy.

Evaluation of R-CHOP and R-CVP in the treatment of elderly patient with non-Hodgkin lymphoma

MedPharmRes ◽  
2019 ◽  
Vol 3 (3) ◽  
pp. 1-6
Author(s):  
Truc Phan ◽  
Tram Huynh ◽  
Tuan Q. Tran ◽  
Dung Co ◽  
Khoi M. Tran
Keyword(s):  
Elderly Patients ◽  
Hodgkin Lymphoma ◽  
B Cell Lymphoma ◽  
Complete Response ◽  
The Elderly ◽  
Free Survival ◽  
Non Hodgkin Lymphoma ◽  
Common Sign ◽  
Related Mortality

Introduction: Little information is available on the outcomes of R-CHOP (rituximab with cyclophosphamide, doxorubicin, vincristine and prednisone) and R-CVP (rituximab with cyclophosphamide, vincristine and prednisone) in treatment of the elderly patients with non-Hodgkin lymphoma (NHL), especially in Vietnam. Material and methods: All patients were newly diagnosed with CD20-positive non-Hodgkin lymphoma (NHL) at Blood Transfusion and Hematology Hospital, Ho Chi Minh city (BTH) between 01/2013 and 01/2018 who were age 60 years or older at diagnosis. A retrospective analysis of these patients was perfomed. Results: Twenty-one Vietnamese patients (6 males and 15 females) were identified and the median age was 68.9 (range 60-80). Most of patients have comorbidities and intermediate-risk. The most common sign was lymphadenopathy (over 95%). The proportion of diffuse large B cell lymphoma (DLBCL) was highest (71%). The percentage of patients reaching complete response (CR) after six cycle of chemotherapy was 76.2%. The median follow-up was 26 months, event-free survival (EFS) was 60% and overall survival (OS) was 75%. Adverse effects of rituximab were unremarkable, treatment-related mortality accounted for less than 10%. There was no difference in drug toxicity between two regimens. Conclusions: R-CHOP, R-CVP yielded a good result and acceptable toxicity in treatment of elderly patients with non-Hodgkin lymphoma. In patients with known cardiac history, omission of anthracyclines is reasonable and R-CVP provides a competitive complete response rate.

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