Sustained remission in a patient with PDGFR‐beta‐rearranged T‐lymphoblastic lymphoma and complete remission with dasatinib

2019 ◽  
Vol 67 (1) ◽  
Author(s):  
Kaustav P. Shah ◽  
Clinton M. Carroll ◽  
Claudio Mosse ◽  
Ashwini Yenamandra ◽  
Scott C. Borinstein
Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4684-4684
Author(s):  
Kian Meng Chang ◽  
Ngee Siang Lau ◽  
Lee Ping Chew ◽  
Sen Mui Tan ◽  
S. Jameela ◽  
...  

Abstract The T- and NK-cell neoplasms are a heterogenous group and are relatively uncommon. From the International Non-Hodgkin’s Lymphoma classification study, precursor T-lymphoblastic lymphoma and the mature T- and NK-cell lymphomas account for 1.7% and 12% of all NHLs. T-NHL are in general more common in Asia. The outcome of this disease is poorer with a 5-y overall survival of 20% (excluding anaplastic NHL). Hospital Kuala Lumpur is the major hematology referral centre in Malaysia. A total of 414 new cases of lymphoma was seen during 2000–2005. The majority were DLBCL 46% and follicular grade I/II lymphomas 15.8%. Only 48 cases (12%) were T-NHL. These were PTCL 19 (5%), anaplastic T-NHL 9 (2.2%), cutaneous T-NHL Stage IV 9 (2.2%), T/NK-NHL 5 (1.2%) and angioimmunoblastic NHL 3 (0.7%). Between 2002–2005, 15 patients were treated with HyperCVAD regimen alternating with Mtx/Ara-C. Three had T-lymphoblastic lymphoma, 7 had PTCL and 5 had T/NK cell. The complete remission was 40% with two proceeding to stem cell transplant. One received local DXT. The median duration of remission was 18.5mths(10–30mths) with median follow-up of 30mths(25–35mths). Alemtuzumab(Campth-1H) is a humanised monoclonal antibody that targets the CD 52 antigen including the T-cell. We combined HyperCVAD with Alemtuzumab 30mg sc on day 1 of every chemotherapy cycle. Four patients with relapsed/refractory T-ALL and 1 patient with T/NK NHL were treated. Two patients, one who relapsed six months after chemotherapy and the other after allogeneic transplant achieved complete remission. The patient with T/NK who had progressive disease with enlarging hepatosplenomegaly after 1 pair of HyperCVAD achieved remission after the addition of Campath. The median duration between each chemotherapy cycle was 25 days(23–26d). One patient had CMV reactivation with no evidence of disease. All patients are still undergoing treatment. The addition of Campath 30mg on day 1 of each chemotherapy cycle appears to be a viable option to try to improve the results in aggressive T-NHL. In view of these preliminary findings, we are starting a study protocol using Campath plus HyperCVAD in aggressive T-cell disease.


2022 ◽  
Vol 2022 ◽  
pp. 1-3
Author(s):  
Ingolf Molle ◽  
Irma Petruskevicius ◽  
Peter Kamper ◽  
Francesco d’Amore

Treatment of early relapses of T lymphoblastic leukemia/lymphoma is often unsuccessful. We tested an experimental regimen containing daratumumab and nelarabine in two young patients with early relapses of T lymphoblastic lymphoma and T-ALL, respectively. Both patients achieved a deep complete remission. Combining daratumumab with chemotherapy may have a role in relapsing T lymphoblastic leukemia/lymphoma.


Pathology ◽  
1991 ◽  
Vol 23 (4) ◽  
pp. 356-359 ◽  
Author(s):  
Edmond Kin-Wah Chiu ◽  
Shee-Loong Loke ◽  
Alex C.L. Chan ◽  
Raymond Hin-Suen Liang

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 845.1-845
Author(s):  
K. Laskari ◽  
P. Athanassiou ◽  
A. Georgiadis ◽  
C. Gerodimos ◽  
G. Gkoni ◽  
...  

Background:Interleukin-1 (IL-1) is a major mediator of the inflammatory cascade in Still’s disease and an established therapeutic target.Objectives:To assess the efficacy and safety of the IL-1b inhibitor canakinumab in adolescent and adult patients with refractory Still’s disease.Methods:We conducted a retrospective longitudinal outcome study of 50 consecutive patients aged 39 years (median, range 14-72), fulfilling the Yamaguchi disease classification criteria, with active disease despite treatment with corticosteroids (CS) (n=11) and/or methotrexate (n=9) and/or biologics (n=30) [tumor necrosis factor inhibitors (n=13), IL-6 blockade (n=7), abatacept (n=2), anakinra (n=24); ≥1 biologics (n=13)]. Canakinumab 150-300 mg was administered sc, starting every 4 (n=48) or 8 weeks (n=2), for a median of 24 months (range 3-84). Concomitant treatment included CS (n=41), methotrexate (n=12) and leflunomide (n=3).Results:Complete remission was initially achieved in 78% of patients within a median time of 3 months, irrespective of age at disease onset. Partial clinical and laboratory response was evident in 20%. Canakinumab was discontinued in one patient with resistant disease (primary failure) and in 6 out of 10 initial responders, who relapsed during treatment (secondary failure). Of 39 patients in complete remission, increase in drug administration interval and/or drug dose reduction was attempted in 7, of which only 1 relapsed, whereas drug discontinuation was attempted in 19 patients for a median time of 8 months (range 3-68), of which 8 relapsed. Overall, in half of all disease flares, canakinumab re-introduction or intensification was successful. Canakinumab had a significant CS sparing effect permitting weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient.Conclusion:In this largest so far real-life patient cohort with refractory Still’s disease, high rates of sustained remission were induced by canakinumab both in adolescent and adult patients.Disclosure of Interests:Katerina Laskari: None declared, Panagiotis Athanassiou Grant/research support from: MSD, Genesis pharma, Janssen, Consultant of: Roche, Genesis pharma, Janssen, Speakers bureau: MSD, Janssen, Roche, Genesis pharma, Athanasios Georgiadis: None declared, Charalampos Gerodimos: None declared, Georgia Gkoni: None declared, Dimitrios Daoussis: None declared, Theodoros Dimitroulas: None declared, Despoina Dimopoulou: None declared, Chrysoula Iliou: None declared, Ioannis Kallitsakis Grant/research support from: MSD, Speakers bureau: Genesis pharma, Bristol-Myers Squibb, Dimitrios Karamitsos: None declared, Christina Katsiari: None declared, Stamatis-Nick Liossis: None declared, Clio Mavragani: None declared, CHARALAMPOS PAPAGORAS: None declared, Dimitrios Pikazis: None declared, Ioannis Raftakis: None declared, Theodosios Sarikoudis: None declared, Loukas Settas: None declared, Prodromos Sidiropoulos: None declared, Despoina Soukera: None declared, Evangelos Theodorou: None declared, Panagiota Tsatsani: None declared, Eleni Tsiakou: None declared, Dimitrios Vassilopoulos: None declared, PANAYIOTIS VLACHOYIANNOPOULOS: None declared, Georgios Vosvotekas: None declared, Paraskevi V. Voulgari: None declared, Marina Zakalka: None declared, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, Petros Sfikakis Grant/research support from: Grant/research support from Abvie, Novartis, MSD, Actelion, Amgen, Pfizer, Janssen Pharmaceutical, UCB


Endoscopy ◽  
2018 ◽  
Vol 51 (04) ◽  
pp. 317-325 ◽  
Author(s):  
Joke Vliebergh ◽  
Pierre Deprez ◽  
Danny de Looze ◽  
Marc Ferrante ◽  
Hans Orlent ◽  
...  

Abstract Background Radiofrequency ablation (RFA), combined with endoscopic resection, can be used as a primary treatment for low grade dysplasia, high grade dysplasia, and early esophageal adenocarcinoma (EAC) in Barrett’s esophagus (BE). The aim of the Belgian RFA registry is to capture the real-life outcome of endoscopic therapy for BE with RFA and to assess efficacy and safety outside study protocols, in the absence of reimbursement. Patients and methods Between February 2008 and January 2017, data from 7 different expert centers were prospectively collected in the registry. Efficacy outcomes included complete remission of intestinal metaplasia (CR-IM), complete remission of dysplasia (CR-D), and durability of remission. Safety outcomes included immediate and late adverse events. Results 684 RFA procedures in 342 different patients were registered. Of these, 295 patients were included in the efficacy analysis, with CR-IM achieved in 88 % and CR-D in 93 %, in per-protocol analysis; corresponding rates in intention-to-treat analysis were 82 % and 87 %, respectively. Sustained remission was seen in 65 % with a median (interquartile range) follow-up of 25 (12 – 47) months. No risk factors for recurrent disease were identified. Immediate complications occurred in 4 % of all procedures and 6 % of all patients, whereas late complications occurred in 9 % of all procedures and in 20 % of all patients. Conclusions Data from the Belgian registry confirm that RFA in combination with endoscopic resection is an efficient treatment for BE with dysplasia or early EAC. In the absence of reimbursement, more rescue treatments are used, not compromising outcome. Since there is recurrent disease after CR-IM in 35 %, surveillance endoscopy remains necessary.


Author(s):  
Edit Porpaczy ◽  
Wolfgang R. Sperr ◽  
Renate Thalhammer ◽  
Gerlinde Mitterbauer-Hohendanner ◽  
Leonhard Müllauer ◽  
...  

AbstractMixed phenotype acute leukemia (MPAL) is an uncommon disease characterized by currently only limited knowledge concerning biology, clinical presentation, and treatment outcome. We here describe a most unusual case of simultaneous occurrence of T-lymphoblastic lymphoma in cervical and mediastinal lymph nodes and acute myeloid leukemia in the bone marrow (BM) successfully treated with allogeneic stem cell transplantation (SCT). Although the blasts in both locations showed additional aberrant expression of other lineage markers (even B-cell markers), diagnostic criteria of MPAL were not fulfilled either in the LN or in the BM. We performed next generation sequencing (NGS) with the objective to look for common genetic aberrations in both tissues. Histology, immunohistochemistry, flow cytometry, AML-associated genetic alterations (FLT3, NPM1, KIT D816V, CEPBA), and clonal T-cell receptor β and γ gene rearrangements were performed according to routine diagnostic workflows. Next generation sequencing and Sanger sequencing were additionally performed in BM and LN. Somatic mutation in the EZH2 gene (p.(Arg684Cys)) was detected in the BM by NGS, and the same mutation was found in the LN. Since an identical genetic aberration (EZH2 mutation) was detected in both locations, a common progenitor with regional dependent differentiation may be involved.


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