Universität zu Köln

Eichenauer, Dennis A. and Engert, Andreas (2017). Nodular lymphocyte-predominant Hodgkin lymphoma: a unique disease deserving unique management. Hematol.-Am. Soc. Hematol. Educ. Program. S. 324 - 329. WASHINGTON: AMER SOC HEMATOLOGY. ISSN 1520-4383

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Abstract

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma entity with an incidence of 0.1 to 0.2/100000/y.Compared with the more common subtypes of classical Hodgkin lymphoma, NLPHL is characterized by distinct pathological and clinical features. Histologically, the disease-defining lymphocyte predominant cells consistently express CD20 but lack CD30. Clinically, NLPHL mostly has a rather indolent course, and patients usually are diagnosed in early stages. The prognosis of early-stage NLPHL is excellent, with progression-free survivaI and overall survival rates exceeding 90% after involved-field radiotherapy (IF-RT) alone (stage IA) or combined modality treatment consisting of a brief chemotherapy with 2 cycles of ABVD (doxorubicin, Neomycin, vinblastine, dacarbazine) chemo therapy followed by IF-RT (early stages other than stage IA). In contrast, patients with advanced disease at diagnosis tend to relapse either with NLPHL histology or with histological transformation into aggressive B-cell non-Hodgkin lymphoma despite more aggressive first-line treatment with 6 to 8 cycles of multiagent chemotherapy. However, even NLPHL patients with multiple relapses successfully respond to salvage therapy in many cases. Salvage therapies range from single-agent anti-CD20 antibody treatment to high-dose chemotherapy followed by autologous stem cell transplantation. Treatment at disease recurrence should be chosen on the basis of various factors, including histology at relapse, time to relapse, extent of disease at relapse, and prior treatment. Because death among NLPHL patients is more often caused by therapy-related late effects than lymphoma-related complications, optimizing the risk-benefit ratio of treatment by decreasing toxicity whenever possible is the major goal of clinical research in this disease.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Eichenauer, Dennis A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Engert, Andreas UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-208238
DOI:	10.1182/asheducation-2017.1.324
Journal or Publication Title:	Hematol.-Am. Soc. Hematol. Educ. Program
Page Range:	S. 324 - 329
Date:	2017
Publisher:	AMER SOC HEMATOLOGY
Place of Publication:	WASHINGTON
ISSN:	1520-4383
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language STUDY-GROUP GHSG; LONG-TERM; PHASE-II; TRANSFORMATION; RITUXIMAB; CHEMOTHERAPY; INTENSITY Multiple languages Education, Scientific Disciplines; Hematology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/20823