Non-Hodgkin Lymphomas in Children and Adolescents

Author(s):  
Véronique Minard-Colin ◽  
Catherine Patte

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

Author(s):  
Tahani Awad Elkarim Elfadl ◽  
Ibrahim Abosoudah ◽  
Mohammed Bayoumy ◽  
Ali Al Harbi ◽  
Muhammad Matloob Alam ◽  
...  

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.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5299-5299
Author(s):  
Yonghong Zhang ◽  
Ling Jin ◽  
Jing Yang ◽  
Yanlong Duan ◽  
Chunjv Zhou ◽  
...  

Abstract One hundred and nineteen children with non-Hodgkin lymphoma were treated between February 2003 and December 2006 in Beijing Children’s Hospital on BCH-2003-NHL protocol. The diagnosis was made by histopathology of the biopsied tissue and/or bone marrow, and disease was classified according to WHO-2001 pathologic classification. We applied modified LMB89 protocol to cases with B-cell lymphoma; modified BFM90-ALL protocol for lymphoblastic lymphoma and cutaneous T-cell/NK cell lymphoma; and modified BFM90-ALCL protocol for anaplastic large-cell lymphoma (ALCL). There were 50 cases (42%) of B cell lymphoma including 32 cases of Burkitt¡’s lymphoma, 10 cases of Burkitt-like lymphoma and 8 cases of diffuse large B cell lymphoma; 44 cases (37%) of lymphoblastic lymphoma; 19 cases (16%) of ALCL; and 6 cases (5%) of cutaneous T-cell/NK cell lymphoma. The 85 boys and 34 girls (ratio, 2.5:1) ranged in age from 2 to 15 years (median, 7.8 years) at diagnosis. B cell lymphoma typically presented as abdomen mass and acute abdomen; nasopharynx and tonsil were also common sites of involvement. Lymphoblastic lymphoma generally presented with mediastinal mass and bone marrow involvement. There was no typical presentation for ALCL. According to the St. Jude staging system, 19 cases had stage I–II, and 94 cases stage III–VI diseases (exclude 6 cases of cutaneous T-cell/NK cell lymphoma). Seven cases had CNS involvement and 25 cases involved bone marrow. The treatment duration was 2 to 8 months for B-cell lymphoma, 2.5 to 3 years for lymphoblastic lymphoma and 1 to 1.5 years for ALCL. The follow-up rate was 100% and median observation period was 23 months. The overall survival (OS) at 3 years was 90.7% and the 3-year event-free survival (EFS) estimate was 82.3%. For B-cell lymphoma, 3-year OS was 88.68% and 3-year EFS was 81.8%. For lymphoblastoma lymphoma, the rates were 89.3% and 69.4%, respectively. All cases of ALCL are alive with on undergoing treatment for relapse. Patients with ALCL achieved the best 3-year OS (100%) and had 3-year EFS of 94.2%. Grade 3 or 4 bone marrow suppression occurred in 97.5% of patients with B-cell lymphoma, 100% of those with lymphoblastic lymphoma and 89.5% of cases with ALCL. As of to date, 11 patients have died, the causes of death include infection (n=4), abandonment of therapy (n=6) and relapse (n=1). Univarate analysis showed that stage IV disease, failure to achieve complete remission after 3 months of treatment, and bulky mass are were associated with poor prognosis £all P values <0.05£©. In summary, we have achieved excellent treatment results using modified international protocols. Infection and financial problem remained the main reasons of treatment failure.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3590-3590 ◽  
Author(s):  
Kieron Dunleavy ◽  
Stefania Pittaluga ◽  
Nicole Grant ◽  
Seth Steinberg ◽  
Margaret Shovlin ◽  
...  

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%)


2017 ◽  
Vol 35 (9) ◽  
pp. 955-962 ◽  
Author(s):  
Andrea B. Moffitt ◽  
Sandeep S. Dave

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.


2019 ◽  
Vol 8 (1S) ◽  
pp. 69
Author(s):  
Ninda Septia Yuspar ◽  
Irza Wahid

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.


2015 ◽  
Vol 33 (27) ◽  
pp. 2963-2974 ◽  
Author(s):  
Véronique Minard-Colin ◽  
Laurence Brugières ◽  
Alfred Reiter ◽  
Mitchell S. Cairo ◽  
Thomas G. Gross ◽  
...  

Non-Hodgkin lymphoma is the fourth most common malignancy in children, has an even higher incidence in adolescents, and is primarily represented by only a few histologic subtypes. Dramatic progress has been achieved, with survival rates exceeding 80%, in large part because of a better understanding of the biology of the different subtypes and national and international collaborations. Most patients with Burkitt lymphoma and diffuse large B-cell lymphoma are cured with short intensive pulse chemotherapy containing cyclophosphamide, cytarabine, and high-dose methotrexate. The benefit of the addition of rituximab has not been established except in the case of primary mediastinal B-cell lymphoma. Lymphoblastic lymphoma is treated with intensive, semi-continuous, longer leukemia-derived protocols. Relapses in B-cell and lymphoblastic lymphomas are rare and infrequently curable, even with intensive approaches. Event-free survival rates of approximately 75% have been achieved in anaplastic large-cell lymphomas with various regimens that generally include a short intensive B-like regimen. Immunity seems to play an important role in prognosis and needs further exploration to determine its therapeutic application. ALK inhibitor therapeutic approaches are currently under investigation. For all pediatric lymphomas, the intensity of induction/consolidation therapy correlates with acute toxicities, but because of 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/computed tomography and minimal disseminated and residual disease, using new biologic technologies to improve risk stratification, and developing innovative therapies, both in the first-line setting and for relapse.


Author(s):  
Nikita Mulchandani ◽  
Suma Mysore Narayana ◽  
Chennagiri S Premalata ◽  
Mohit Agrawal ◽  
Venkateshaiah Reddihalli Pallavi

Introduction: The involvement of the Female Genital Tract (FGT) by lymphoma is extremely rare, with ovaries being most commonly affected. Less than 1% of lymphomas present with ovarian involvement and less than 1.5% of ovarian neoplasms are of lymphoid origin. Secondary involvement of ovary by systemic lymphoma is more common than Primary Ovarian Lymphomas (POL) which is usually Primary Ovarian Non-Hodgkin lymphoma (PONHL) of B-cell lineage. Aim: To understand the clinicopathological and immuno- morphological features of Primary Ovarian Non-Hodgkin lymphoma. Materials and Methods: This was a descriptive retrospective study conducted at Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka. India, for a duration of 14 years between July 2006 to June 2020. Eight cases of PONHL were identified from departmental archives and clinicopathological and Immunohistochemistry (IHC) findings of these tumours were analysed. Results: The PONHL constituted 0.4% of all Non-Hodgkin lymphoma (NHL) reported during the study period. The patients age ranged from 13-60 years with a mean age of 34 years. Among eight cases of PONHL, two cases were of Diffuse Large B-Cell Lymphoma (DLBCL), followed by one case each of High- Grade B-Cell Lymphoma, Not Otherwise Specified (HGBL, NOS), Follicular Lymphoma (FL), Burkitt Lymphoma (BL), Plasmablastic Lymphoma (PBL), Precursor B-Lymphoblastic Lymphoma (B-LBL), and precursor T-Lymphoblastic Lymphoma (T-LBL). Seven cases were staged IE (Ann Arbor staging system) while one case was designated as stage IIE. Conclusion: This was probably the first study on PONHL from India. The diagnosis of PONHL is challenging unless there is a high index of suspicion as these patients present with non specific pelvic symptoms and can be misdiagnosed as other epithelial, stromal or germ cell ovarian neoplasm which differs in treatment and prognosis. Histological examination with IHC and molecular testing are essential to establish a diagnosis.


2019 ◽  
Vol 24 (3) ◽  
Author(s):  
Ewa Krasuska-Sławińska ◽  
Izabela Królik-Elgas ◽  
Marzena Stypińska ◽  
Anna Matosek-Rutkowska

B-cell lymphoblastic lymphoma which is a type of non-Hodgkin lymphoma is rather uncommon in children. Focal changes in bones in the course of non-Hodgkin lymphoma are mostly secondary changes and their primal location in a bone is rare. PBL (primary bone lymphoma) mainly concerns a thighbone and a tibial bone; the primary location in jaw bones is quite sporadic. In diagnostics, there is mainly magnetic resonance, medical scan (tomography), and above all – histopathological test. There is also chemotherapy by choice, and primary location in a jaw or a mandible significantly advances the prognosis. The aim of the work is to introduce a patient who was definitively diagnosed B-cell lymphoblastic lymphoma from the early B-cells. The girl reported to Laryngological Clinic, Dental Surgery Clinic for Children, Oncological Clinic of Children’s Memorial Health Institute. The cause of the visit was an elevation on the right side of a nose base, present for two months and misdiagnosed by doctors as a post-traumatic swelling in this region. After introducing laboratory and scan diagnostics and taking biopsy from the lesion, a final conclusion was made. Also, a proper treatment according to the protocol for B-cell lymphoblastic lymphoma was introduced. Non-specific B-cell lymphoma picture, as mentioned in the described case, specifically due to location in a jaw bone and a slow pace of growing, may both constitute huge diagnostic problems and deteriorate prognosis. Therefore, it is important to take into account also lymphoma – in such location of a lesion. Moreover, it is worth remembering that the final diagnosis may only be passed on the basis of histopathological examination.


Hematology ◽  
2007 ◽  
Vol 2007 (1) ◽  
pp. 297-303 ◽  
Author(s):  
John T. Sandlund

Abstract The SEER (Surveillance, Epidemiology, and End Results) data for the years 1975–1998 show that children with non-Hodgkin lymphoma (NHL) have a better treatment outcome than do adults. Many factors may contribute to this age-related difference. Some factors are related to the patient (e.g., drug distribution and clearance, performance status, compliance, sex) whereas others pertain to tumor histology and biology. The spectrum of NHL subtypes is well known to differ in children and adults. From ages 5 through 14 years, Burkitt lymphoma is the predominant histologic subtype, whereas diffuse large B-cell lymphoma is most common in the 15- to 29-year age range. Because different treatment strategies are often used in children and adults with NHL, the choice of therapy for adolescents and young adults (ages 15 through 29 years) is challenging and somewhat controversial. It is reasonable to consider pediatric strategies for some adolescents and very young adults with NHL, and pediatric strategies are currently used to treat adults with certain subtypes of NHL (Burkitt lymphoma, lymphoblastic lymphoma). However, the use of pediatric strategies in adults does not guarantee a comparable outcome, as illustrated by trials for adult lymphoblastic lymphoma. There is clearly a need for further biologic study of NHL in children, adolescents, and young adults. Age-related differences in tumor biology have been demonstrated in anaplastic large-cell lymphoma (ALCL) and diffuse large B-cell lymphoma (DLBCL). Additional biologic data will not only improve prognosis and treatment stratification but, more important, will lead to the identification of specific molecular targets for therapy.


2019 ◽  
Vol 42 (3) ◽  
pp. 303-318 ◽  
Author(s):  
Julieta Afonso ◽  
Tatiana Pinto ◽  
Susana Simões-Sousa ◽  
Fernando Schmitt ◽  
Adhemar Longatto-Filho ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document