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Non-Hodgkin-Lymphome bei Kindern und Jugendlichen

Von der Diagnostik bis zur Nachsorge

Non-Hodgkin’s lymphoma in children and adolescents

From diagnostics to follow-up

Zusammenfassung

In Deutschland beträgt die jährliche Inzidenz der Non-Hodgkin-Lymphome (NHL) 0,8/100.000 Kinder unter 15 Jahren. Die im Kindesalter vorherrschenden NHL-Formen sind Burkitt-Lymphome, lymphoblastische sowie anaplastische großzellige Lymphome. Häufigstes NHL-Leitsymptom bei Kindern und Jugendlichen sind schmerzlose Lymphknotenschwellungen. Bei malignen Ergüssen oder signifikantem Knochenmarkbefall kann die Diagnose ohne Operation gesichert werden, ansonsten ist ein operativer Eingriff erforderlich. Eine exakte Klassifikation des NHL ist die absolute Voraussetzung für die Wahl der geeigneten Therapieform, denn für die 3 Subgruppen lymphoblastische Lymphome, B-Zell-Lymphome mit komplettem B-Immunphänotyp und anaplastische großzellige Lymphome kommen unterschiedliche Chemotherapiestrategien zum Einsatz, mit welchen ≥80% der Betroffenen überleben. Geheilte ehemalige onkologische Patienten bedürfen einer lebenslangen Nachsorge (Erkennen von Rezidiven bzw. Spätfolgen der Therapie).

Abstract

The annual incidence of non-Hodgkin’s lymphoma (NHL) in Germany is 0.8/100,000 children under 15 years of age. The dominant forms of NHL in childhood are Burkitt’s lymphoma, lymphoblastic and anaplastic large cell lymphomas. The commonest main symptom of NHL in children and adolescents is painless lymph node swelling. The diagnosis can be confirmed without the necessity of an operation when malignant effusions or significant bone marrow participation occur, otherwise an operative intervention is necessary. An exact classification of NHL is an absolute priority for selection of a suitable form of therapy as different chemotherapy strategies are employed for the three subgroups lymphoblastic lymphoma, peripheral B cell lymphoma and anaplastic large cell lymphoma by which ≥80 % of patients survive. Cured oncological patients need a lifelong follow-up for recognition of recurrent or late complications of therapy.

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Abbreviations

ALCL:

Anaplastisches großzelliges Lymphom

ALL:

Akute lymphoblastische Leukämie

ALPS:

Autoimmunes lymphoproliferatives Syndrom (Canale-Smith-Syndrom)

AML:

Akute myeloische Leukämie

AP:

Alkalische Phosphatase

Ara-C:

Arabinofuranosylcytosin

B-ALL:

Akute B-Zell-Leukämie

BFM:

Berlin, Frankfurt, Münster

B-NHL:

B-Zell-Non-Hodgkin-Lymphom

BSG:

Blutkörperchensenkungsgeschwindigkeit

CMV:

Zytomegalievirus

COMP:

Cyclophosphamid, Oncovin (Vincristin), Methotrexat, Prednison

CT:

Computertomographie

DLBCL:

Diffuses großzelliges B-Zell-Lymphom

EBV:

Epstein-Barr-Virus

EKG:

Elektrokardiogramm

EMA:

Epitheliales Membranantigen

FAB:

Französisch-amerikanisch-britisch

FISH:

Fluoreszenz-in-situ-Hybridisierung

HAV:

Hepatitis-A-Virus

HBV:

Hepatitis-B-Virus

HCV:

Hepatitis-C-Virus

HHV-8:

Humanes Herpesvirus 8

HIV:

Humanes Immundefizienzvirus

HL:

Hodgkin-Lymphom

HNO:

Hals, Nasen, Ohren

Ig:

Immunglobulin

IPT:

Immunophänotypisierung

LDH:

Laktatdehydrogenase

LSA2-L2:

Lymphosarkom-2-Protokoll

MALT:

„Mucosa associated lymphatic tissue“

MOTT:

„Mycobacteria other than tuberculosis“

MRT:

Magnetresonanztomographie

NHL:

Non-Hodgkin-Lymphom

NK:

„Natural killer“

PMLBL:

Primär mediastinales (thymisches) großzelliges B-Zell-Lymphom

PTLD:

„Posttransplantant lymphoproliferative disease“

T3:

Trijodthyronin

T4:

Thyroxin

TSH:

Thyreoidea stimulierendes Hormon

VZV:

Varizella-Zoster-Virus

WHO:

Weltgesundheitsorganisation

XLP:

X-chromosomales lymphoproliferatives Syndrom (Purtilo-Syndrom)

ZNS:

Zentralnervensystem

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Correspondence to J. Ritter.

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Der vorliegende aktualisierte Beitrag erschien ursprünglich in der Zeitschrift best practice onkologie 6/2011.

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Ritter, J. Non-Hodgkin-Lymphome bei Kindern und Jugendlichen. Monatsschr Kinderheilkd 160, 1147–1162 (2012). https://doi.org/10.1007/s00112-012-2818-y

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Schlüsselwörter

  • Non-Hodgkin-Lymphom
  • Burkitt-Lymphom
  • Lymphoblastisches Lymphom
  • Anaplastisches großzelliges Lymphom
  • Chemotherapiestrategie

Keywords

  • Non-Hodgkin’s lymphoma
  • Burkitt’s lymphoma
  • Lymphoblastic lymphoma
  • Lymphoma, large cell, anaplastic
  • Chemotherapy strategy