Open Questions in the Management of Nodular Lymphocyte Predominant Hodgkin Lymphoma

Localized Nodular Lymphocyte Predominant Hodgkin Lymphoma is a rare disease with an overall good prognosis but frequent late relapses. Due to it’s rarity there is no standard therapeutic approach and pathological diagnosis may be hard. In this paper we discuss the technical aspects of the radiation therapy and histological issues. The new fields reductions proposed for classical Hodgkin lymphoma cannot be applied to early stages Nodular Lymphocyte Predominant Hodgkin lymphomas which are usually treated with radiation therapy without systemic chemotherapy. 1. Introduction In this paper we describe two cases of failure in the management of nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). Both treated outside our center, they were referred back to our unit for early relapse after “involved node” radiotherapy (INRT). NLPHL is a rare disease (3–8% of all Hodgkin lymphomas) with distinct histology and clinical course. Despite frequent late relapses, NLPHL have paradoxically an overall good prognosis. Due to the rarity of the disease there is no standard therapeutic approach. In this short paper, we discuss the technical aspects of the radiation therapy and histological issues correlated with these two early relapses. 2. Case Presentation: First Case The patient is a 53-year-old male who presented a submaxillary right tumefaction (IIA area). The lymph node biopsy (June 2011) demonstrated NLPHL. The Ann Arbor classification was stage IA. The PET scanner confirmed the unique uptake in the cervical area IIA. An INRT started in August 2011 and delivered 36？Gy within 18 fractions using conformational technique and was well tolerated (Figure 1). Figure 1: Dose distribution of a three-dimensional conformal radiotherapy: CTV (blue), PTV (red), and isodoses lines of 37 Gray (green), 36 Gray (cyan), 34 Gray (pink), and 20 Gray (yellow). During the followup the evaluation was assessed using PET scanners. The first PET scanner (January 2012) showed a complete response in the IIA area but a suspicious uptake in the subclavicular area (outside treated fields), confirmed as a relapse on the PET of July 2012 (Figure 2). Figure 2: PET scanner showing the relapse in the subclavicular area and mapping. The biopsy of this early recurrence (5 months) demonstrated a relapse of NLPHL, confirmed in our hematological malignancies expert laboratory. The performance status (PS) of the patient was still 0; biology and clinical examination were normal. 3. Second Case The patient is a 46-year-old male who presented a left cervical (III area) tumefaction. After biopsy he was