Clinical presentation, evolution, and prognosis of precursor B‐cell lymphoblastic lymphoma in trials LMT96, EORTC 58881, and EORTC 58951

• Published in: British journal of haematology 2011-02, Vol.152 (4), p.441-451
• Main Authors: Ducassou, Stéphane, Ferlay, Céline, Bergeron, Christophe, Girard, Sandrine, Laureys, Geneviève, Pacquement, Hélène, Plantaz, Dominique, Lutz, Patrick, Vannier, Jean‐Pierre, Uyttebroeck, Anna, Bertrand, Yves
• Format: Article
• Language: English
• Subjects: Acute lymphatic leukemia; Adolescent; Age; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Bone marrow; Cancer; Central nervous system; Child; Child, Preschool; Children; Clinical trials; Disease Progression; double prime B-cell lymphoma; Drug Administration Schedule; Female; Follow-Up Studies; Hematologic and hematopoietic diseases; Humans; Infant; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; lymphoblastic lymphoma; Lymphocytes B; Male; Medical sciences; Neoplasia; Neoplasm Staging; precursor B‐cell; Precursor Cell Lymphoblastic Leukemia-Lymphoma - diagnosis; Precursor Cell Lymphoblastic Leukemia-Lymphoma - drug therapy; Precursor Cell Lymphoblastic Leukemia-Lymphoma - pathology; Prognosis; Recurrence; Remission; Survival; Survival Analysis; Treatment Outcome
• ISSN: 0007-1048; 1365-2141
• DOI: 10.1111/j.1365-2141.2010.08541.x

Summary In children, lymphoblastic lymphomas represent 30% of Non‐Hodgkin lymphomas (NHL), and approximately 15% are precursor B‐cell lymphomas (PBLL). Our study evaluated their main clinical characteristics, evolution, and prognosis in three trials. From 1989 to 2008, 53 children with PBLL (median age 7·75 years) were included in three protocols: Malignant Lymphoma Therapy (LMT) 96, European Organization for Research and Treatment of Cancer (EORTC) 58881, and EORTC 58951 using Berlin‐Frankfürt‐Münster‐derived acute lymphoblastic leukaemia (ALL) therapy. There were 10 stage I disease, 9 stage II, 9 stage III and 25 stage IV. Clinical presentation was heterogeneous with a majority of bone lesions and cutaneous or subcutaneous manifestations. At diagnosis 23 patients had bone marrow involvement, and only three had central nervous system involvement. The median follow‐up was 74 months. At last follow‐up, 45 patients were in continuous complete remission, whereas eight had progressed or had relapsed (7 Stages IV and 1 Stage III) and died. Two patients had a secondary neoplasia, and are still alive. Disease stage was a major prognostic factor, with better overall survival (OS) and event‐free survival (EFS) (P