Treatment of nodular lymphocyte-predominant Hodgkin lymphoma
- Peter M Mauch, MD
Peter M Mauch, MD
- Professor of Radiation Oncology
- Harvard Medical School
- Ann S LaCasce, MD
Ann S LaCasce, MD
- Assistant Professor of Medicine
- Harvard Medical School
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is an uncommon subtype of Hodgkin lymphoma (HL). This disorder is differentiated from all other forms of HL, which are commonly referred to as classical Hodgkin lymphomas (CHL), by characteristic pathologic and clinical features.
NLPHL represents a more indolent disease than CHL, and therefore is managed uniquely. Since this disease is rare, most information concerning treatment and outcome has come from reports of single institutions or pooled, multi-institutional retrospective analyses.
Treatment and prognosis of NLPHL are reviewed here. The epidemiology, pathogenesis, clinical presentation, pathology, and diagnosis of NLPHL are discussed separately. (See "Clinical manifestations, pathologic features, and diagnosis of nodular lymphocyte-predominant Hodgkin lymphoma".) Treatment of the other Hodgkin lymphoma subtypes is discussed separately.
After the diagnosis of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is confirmed, the patient should undergo a work-up and staging evaluation similar to that performed for patients with classical Hodgkin lymphomas (CHL). Staging is performed using the Ann Arbor staging system with Cotswolds modifications (table 1) [1,2]. This system is described at length separately. (See "Staging and prognosis of Hodgkin lymphoma" and "Clinical manifestations, pathologic features, and diagnosis of nodular lymphocyte-predominant Hodgkin lymphoma".)
Historically, patients with nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) have been treated like patients with classical Hodgkin lymphomas (CHL), and many of the large randomized studies that have guided treatment of patients with CHL have included small numbers of patients with NLPHL. NLPHL differs from CHL in terms of natural history, response to treatment, and prognosis , and therapeutic approaches may be distinct from CHL. Our approach is generally consistent with that proposed by the National Comprehensive Cancer Network (NCCN) .
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- STAGE IA/IIA DISEASE
- Natural history
- Surgical excision alone
- Radiation therapy
- - Reduced volume RT
- - RT technique
- RT with or without chemotherapy
- Chemotherapy alone
- Recurrent disease
- Treatment related complications
- STAGE IB/IIB DISEASE
- STAGE III/IV DISEASE
- Combination chemotherapy
- Palliative RT
- Relapsed/refractory disease
- Impact of histopathology
- Transformation to NHL
- SUMMARY AND RECOMMENDATIONS