Secondary Neoplasms Subsequent to Berlin-Frankfurt-Muenster (BFM) Therapy of Non-Hodgkin Lymphoma of Childhood: Significantly Higher Risk for Patients with Lymphoblastic Lymphoma Compared to Other NHL-Subtypes.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 232-232 ◽  
Author(s):  
Olga Wachowski ◽  
Martin Zimmermann ◽  
Birgit Burkhardt ◽  
Olaf Determann ◽  
Ulrike Meyer ◽  
...  

Abstract We evaluated the rate and type of second malignant neoplasms (SMN) after BFM treatment of children with Non-Hodgkin lymphoma (NHL). Between January 1981 and February 2003 2451 patients (pts) <15 years (y) of age at diagnosis were enrolled into the subsequent trials NHL-BFM 81, 83, 86, 90, and 95. Pts with lymphoblastic lymphoma (LBL) (n=547) or non-anaplastic peripheral T-cell lymphoma (n=97) received acute lymphoblastic leukemia (ALL)-type therapy including cumulative doses of cyclophosphamide (max. 3g/m2), daunorubicine/doxorubicin (max. 280mg/m2), but, except few pts, no etoposide. Prophylactic cranial radiotherapy (CRT) was given in stage III/IV (omitted in NHL-BFM95). Pts with mature B-cell neoplasms (n=1597), or anaplastic large cell lymphoma (n=210) received B-type therapy, consisting of 2–8, 5-day courses including cumulative doses of cyclophosphamide (max. 7g/m2), ifosfamide (max. 8g/m2), doxorubicine max. 150mg/m2 (in trial 81 max. 200mg/m2), and etoposide (max. 1.4g/m2). CRT was omitted since trial NHL-BFM86. With a median follow-up of 6.9 (range 0.2–22.6) years the probability of survival at 15 y was 83+1%. By June 2005, 47 SMN were documented, including 16 acute myeloid leukemias/myelodysplastic syndromes (AML/MDS), 11 NHL, 2 ALL, 1 Hodgkin’s lymphoma, 7 brain tumors, and 10 other SMN. All SMN occurred in first remission after a median time of 2.9 (range: 0.4–12.3) years from diagnosis of NHL. The cumulative incidence of SMN at 15 y was 4.0% (95% confidence interval [CI]: 1.9%–6.1%) for the total group. The cumulative incidence of SMN was significantly higher among pts with LBL receiving ALL-type therapy (6.3% at 15 y [95%CI: 2.4%–10.3%] (13 AML/MDS, 2 NHL, 3 brain tumors, and 3 other SMN), as compared to pts with other NHL-entities receiving B-type therapy (3.4% at 15 y [95% CI: 0.5–6.4%] (3 AML/MDS, 9 NHL, 2 ALL, 1 Hodgkin’s lymphoma, 4 brain tumors, and 7 other SMN), p=0.002. There was no significant difference of cumulative incidence of SMN in pts who received CRT compared to pts not receiving CRT. However, 5 of 7 pts, who developed brain tumor, received CRT of 12–24 Gy. Also, there was no significant correlation between the incidence of SMN and the cumulative doses of drugs, except for anthracyclines. For pts receiving a cumulative dose of anthracyclines of >160mg/m2 (almost exclusively pts with LBL receiving ALL-type therapy) the cumulative risk for SMN at 15 y was 6.5% (95% CI: 1.5-11.5%), as compared to 2.0% (95% CI: 1.1–2.9%) for pts with lower doses, p=0.007. Exposure to etoposide was not a risk factor for secondary AML/MDS (11 of 16 pts with sec. AML/MDS did not receive etoposide). In a Cox regression analysis only diagnosis of LBL remained a significant risk factor for SMN (RR 2.5, 95% CI 1.4–4.4). Our analysis revealed a cumulative risk for SMN of 4% at 15 y after successful treatment of childhood NHL. The cumulative incidence of SMN was significantly higher in LBL-pts than in other pts. AML/MDS were the most frequent SMN following LBL while second lymphoid malignancies were the most frequent SMN following non-LBL.

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.


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.


2021 ◽  
Vol 5 (4) ◽  
pp. 134-138
Author(s):  
Glazydia Juwita Rachma ◽  
Ugroseno Yudho Bintoro ◽  
Mia Ratwita Andarsini ◽  
Novira Widajanti

Non-Hodgkin's lymphoma is a major public health problem with over 14.1 million people are diagnosed with it (2012). In the same year there were 8.2 million deaths due to cancer. The purpose of this study was to determine the relationship between clinical manifestations and the degree of malignancy based on histopathological features in patients with Non-Hodgkin's Lymphoma. This study used a retrospective analytical method with a cross-sectional approach using the patient's medical record at RSUD Dr. Soetomo, Surabaya who was diagnosed with Non-Hodgkin Lymphoma from 1st January 2015 to 31st December 2017. In this study, there were 139 samples include those criteria, with a greater number of male samples (62.6%) compared to women (37.4%). This study showed that 49.3% of patients with non- Hodgkin's lymphoma in RSUD Dr. Soetomo with clinical manifestations without symptoms actually experience malignancy with a high degree, this showed that clinical manifestations without symptoms are not always associated with a low level of malignancy. Then, based on the Chi Square test results obtained p-value of 0.289 (>0.05), so there was no significant relationship between clinical manifestations and the degree of malignancy. Keywords: lymphoma; manifestation; histopatological


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


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4862-4862 ◽  
Author(s):  
Alaa Addasi

Abstract Abstract 4862 BACKGROUND Jordan is a small country with an estimated mid year population in 2008 of 5 850 000, 3015000 of whom are males and 2 835 000 are females (male: female ratio 1.06: 1). (Department of Statistics Jordan, 2008). About 12.7 % of the population is under 5 years old, and 37.3 % under 15 years old. 11%if the population are 15–19 year old, with a M:F ratio 1.06:1 as well) Only 3.3 % of the total population is above the age of 65 years old (sex ratio of 1.01 male per 1 female in this age group). According to the Jordan Cancer Registry Report for 2008, Lymphoma is the fourth most common Cancer in the country. A total of (4606 ) new cases of cancer were recorded among Jordanians in the year 2008, 333 (7.2%) of whom had a diagnosis of lymphoma.111 (2.4%) were diagnosed as Hodgkin's lymphoma (HL), and 222 (4.8%) as Non Hodgkin Lymphoma(NHL). OBJECTIVE In this study, we aim to characterize some of the clinico-pathological features of Hodgkin lymphoma in Jordan by analyzing the data available for patients referred to King Hussein Cancer Center over a seven year period. PATIENTS AND METHODS A retrospective analysis was conducted of adults (>18 years) lymphoma patients referred to KHCC, between 1/1/2003 and 31/12/2010. Clinical features and histological subtypes were prospectively established for all patients registered in the Lymphoma Service Database. Pathology review and original paraffin block were mandated for all patients. RESULTS Over the 8 year period of 2003–2010,1329 lymphoma patients were referred to KHCC and registered in the Lymphoma Service Database, of whom 477 (35.9%) were diagnosed with Hodgkin's lymphoma. Among this group all 477 patients were adults 18 years or older (100%), as children are treated in a different department. The median age was 35 years, (with an age range of 18–77), and 5% of patients were above the age of 60. 290 (61 %) of the patients were males, 187 (39%) were females, with a male to female (M:F) ratio of 1.55:1. 276 (57.8%) of the HL cases had a diagnosis of nodular sclerosis Hodgkin lymphoma (HDNS), making it the most common histological subtype. 120(25.2%) had mixed cellularity Hodgkin lymphoma (HDMC), 9 (1.9%) had lymphocyte-rich Hodgkin lymphoma (HDLR), and 6 (1.2%) had lymphocyte-depleted Hodgkin (HDLD). Nodular lymphocyte predominance Hodgkin lymphoma (NLPHD) cases were 33, and constituted 6.9% of the HL cohort. CONCLUSION Hodgkin lymphoma appears to constitute a bigger share of the lymphoma burden in Jordan, as opposed to Europe and the US. Clinico-pathological features, however, appear to be closer to those described in Western countries, with similar incidence of HDNS, and HDMC subtypes, but possibly with less incidence of HDLR and HDLD subtypes. Disclosures: No relevant conflicts of interest to declare.


Author(s):  
V.V. Paramonov ◽  
I.S. Dyagil

The aim is to compare the cumulative incidence of Hodgkin's lymphoma and non-Hodgkin's lymphomas between conditionally clean and polluted regions of the Cherkasy region in 2001, 2014. Materials and methods. The incidence of Hodgkin's lymphoma and non-Hodgkin's lymphomas in conditionally clean, radioactive, chemical and radioactively and chemically contaminated regions of the Cherkasy region per 100 thousand population according to the All-Ukrainian census conducted in 2001 and published by the State Statistical Service of Ukraine in 2014. Results. According to the results of our study, in 2001, in the chemically contaminated region of the Cherkasy region, there was an increase in the incidence of mainly diffuse large B-cell lymphoma by 3.781 (p = 0.043) times compared to its conditionally clean territory (1.076; 0.022-2.130 versus 4.070; 0.082-8.058 per 100 thousand population, respectively). In the chemically contaminated area in 2014, a 3.314 times (p = 0.035) higher level of cumulative incidence of unspecified lymphomas was also revealed (1.793; 0.358-3.228 versus 5.945; 0.734-11.156 per 100 thousand population, respectively) compared to clean areas of the Cherkasy region. Conclusions. Thus, the results of our study showed that in 2001, living in a chemically contaminated area increased the growth of the cumulative incidence of diffuse B-cell lymphoma, and in 2014 - lymphomas of an unspecified type.


2009 ◽  
Vol 150 (35) ◽  
pp. 1649-1653
Author(s):  
Balázs Kollár ◽  
Péter Rajnics ◽  
Béla Hunyady ◽  
Erika Zeleznik ◽  
János Jakucs ◽  
...  

A felnőttkori non-Hodgkin-lymphoma előfordulása az elmúlt évtizedekben jelentősen nőtt. A betegcsoport nagyon heterogén, változatos klinikai és morfológiai megjelenéssel. A legjellemzőbb nodalis érintettség mellett gyakoriak az extranodalis formák, amelyek leggyakrabban a gastrointestinalis traktust, a központi idegrendszert és a bőrt érintik. A gastrointestinalis traktus non-Hodgkin-lymphomáinak kezelési stratégiája változott az elmúlt évtizedben, a kemoimmunoterápia háttérbe szorította a korábban jóval gyakrabban végzett sebészeti beavatkozásokat. Módszerek: A szerzők Kaposváron, a Kaposi Mór Oktató Kórházban és Gyulán, a Pándy Kálmán Megyei Kórházban kezelt 48, gastrointestinalis traktust érintő non-Hodgkin-lymphomás betegük adatait mutatják be. A betegek közül 27 nő és 21 férfi, átlagéletkoruk 67,8 év. A leggyakoribb lokalizáció a gyomor ( n = 26), a leggyakoribb szövettani típus diffúz nagy B-sejtes lymphoma (DLBCL) volt. A betegek rizikófaktorait a nemzetközi prognosztikai index (IPI) alapján állapították meg. Negyvenhat beteg kapott kemoimmunoterápiás kezelést, 6 esetben érintett mezős sugárkezelés, 3 esetben Helicobacter pylori -eradikáció, 4 betegnél gyomorreszekció történt. Eredmények: Az összes beteg 68%-ában sikerült komplett, 13%-ában parciális remissziót elérni, 19% nonreszponder volt. A nemzetközi prognosztikai index alapján a betegek többsége az alacsony, illetve magas intermedier rizikócsoportba tartozott (IPI-átlag: 2,68). A tápcsatorna felső szakaszát érintő lymphomás betegek prognózisa volt a legjobb (IPI: 2,0), ugyanakkor a gyomorlymphomás betegeknél volt a legmagasabb a komplett remisszió aránya (73%). Következtetés: Kemoimmunoterápiával a betegek gyógyulási esélyei javultak az elmúlt évtizedben, a gastrointestinalis traktust érintő non-Hodgkin-lymphomák jelentős hányada meggyógyítható. Az IPI a legelfogadottabb mutató a non-Hodgkin-lymphoma prognózisának megítélésére. A komplett remisszióba jutott betegek prognosztikai indexe volt a legalacsonyabb, de az IPI-n kívül egyéb tényezők is befolyásolhatják a kezelésre adott választ.


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