Syncytial Variant Nodular Sclerosis Classical Hodgkin Lymphoma in an Adolescent and Review of the Literature: A Unique Entity. Issue 3 (April 2019)

• Record Type: Journal Article
• Authors: Al-Antary, Eman; Jesudas, Rohith; George, Amy; Poulik, Janet; Savaşan, Süreyya
• Is Part Of: Journal of pediatric hematology/oncology. Volume 41:Issue 3(2019)
• Publication Date: 2019-04
• Subjects: Hodgkin lymphoma -- syncytial variant nodular sclerosis subtype -- chest wall bone involvement -- neutrophil emperipolesis by Reed-Sternberg-like cells; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000001245
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : Syncytial variant of nodular sclerosis (SV-NS) classical Hodgkin lymphoma (cHL) with its histologic features and clinical presentation is uncommon in adults and extremely rare in children. Here, we report a female teenager presenting with long-standing B symptoms, prominent soft tissue and bone involvement mimicking sarcoma and significant nodal disease who is diagnosed with advanced SV-NS cHL. Rare Reed-Sternberg-like cells displaying neutrophil and erythrocyte emperipolesis were seen on bone marrow aspiration slides. Despite initial complete response to chemotherapy and radiotherapy, the patient experienced early relapse suggestive of high-risk biology. This variant may constitute a unique entity.