NHL-BFM Registry 2012

B-Cell Non-Hodgkin-Lymphoma (B-NHL), mature B-cell leukeamea/ Burkitt leukeamia (B-AL), Anaplastic large cell lymphoma (ALCL), Lymphoblastic lymphoma (LBL), rare subtypes of Non-Hodgkin lymphoma

Registry

It is the intention of the NHL-BFM Registry 2012 to maintain the optimal treatment for all children and adolescents with Non-Hodgkin-Lymphoma also in times, when no clinical trial for theses patients is open. The registry is open for all centers of the NHL-BFM group in Germany, Austria, Switzerland and the Czech Republic.
The registry does not start new therapies, but offers the best available therapy. Treatment recommendations are offered for those NHL-subtypes without currently active clinical trial. After the registration of a patient the registry offers assurance of continuous high standard of initial diagnostics by reference diagnostics (pathology, immunology, morphology, genetics).
It is intended by the NHL-BFM group to run clinical trials aiming at treatment optimization in children and adolescents with NHL. These clinical trials will be restricted to defined histological subgroups and run in parallel to the NHL-BFM Registry 2012. If an open clinical trial is available for a NHL-subgroup, treatment according to this trial should be offered to the patient/family.

AIMS OF THE NHL-BFM REGISTRY 2012

• continuous registration of all pediatric and adolescent NHL patients in the BFM group
• assurance of continuous high standard of initial diagnostics by reference diagnostics (pathology, immunology, morphology, genetics)
• assurance of correct allocation of treatment group and stratification arm
• to control patients’ safety by yearly analyses of o recruitment, overall survival (OS), event free survival (EFS) and cumulative incidence of events (relapse, treatment related mortality, secondary malignancy, death)
• to provide treatment recommendations for NHL patients for whom no clinical trial is open for recruitment at the time of diagnosis. These treatment recommendations are based on treatment strategies established by the clinical trials of the BFM-group or on published data from other cooperative groups showing a significantly higher survival rate or less toxicity compared to BFM-strategies.
• to allow structured and evidence based consultation of the NHL-BFM study centers for medical advice
• to allow biological studies on NHL in children and adolescents

Lymphoblastic Lymphoma

The treatment consists of two therapy parts: the intensive phase of the chemotherapy, requiring ca. seven month and a maintenance therapy with two oral medicaments which ends after two years of total therapy. All patients with an initial CNS-desease are treated with CNS-radiation.

B-NHL/B-AL

The patients are treated depending of the stage of the desease, initial LDH and CNS-desease with two to seven therapy-blocks, lasting 5 days each. The etablished criteria for the stratification and the combination and number of the therapy-blocks are continued anlaog to the B-NHL BFM 2004 trial.

ALCL

Patienten mit komplett reseziertem Stadium I erhalten nach einer Vorphase drei Chemotherapiekurse. Mit Ausnahme der Patienten mit isoliertem Hautbefall oder ZNS-Beteiligung erhalten alle Patienten höherer Risikogruppen nach der Vorphase sechs Chemotherapiekurse.
Die sehr kleine Patientengruppe mit isoliertem Hautbefall erhält nach eindeutiger Klärung des Stadiums keine Chemotherapie. Die Entscheidung für eine "watch and wait"-Strategie bei diesen Patienten sollte mit der Studienzentrale diskutiert werden. Die wenigen Patienten mit einer Beteiligung des zentralen Nervensystems werden entsprechend der Therapieempfehlung für Patienten mit ZNS-positivem reifen B-Zell NHL behandelt, gefolgt von einer Schädelbestrahlung.

Andere seltenen NHL-Subtypen

Für die Behandlung seltener NHL-Subtypen liegt zurzeit keine validierte Standardtherapie vor, die in das Register NHL-BFM Registry 2012 aufgenommen werden kann. Für die Diagnostik und Therapie dieser Patienten steht die Studienzentrale gerne als Ansprechpartner zur Verfügung. Dies betrifft z.B. periphere T-Zell Lymphome, primär mediastinale B-Zell Lymphome, Follikuläre Lymphome, Marginalzonen-Lymphome oder Lymphome bei Kindern mit schwerem Immundefekt.

• alle neu diagnostizierten Non-Hodgkin-Lymphome oder Non-Hodgkin-Lymphome als Sekundärmalignom
• Diagnose und Behandlung in einem teilnehmenden Zentrum
• Alter bei Diagnose < 18 Jahre
• Unterschriebene Einwilligungserklärungen (einsichtsfähiger Patient, Erziehungsberechtigte) über die Teilnahme am Register und zur Speicherung / Weitergabe von Daten
• Follow-up von wenigstens 2 Jahren ab Diagnose möglich