Published online Jun 16, 2018. doi: 10.12998/wjcc.v6.i6.121
Peer-review started: January 14, 2018
First decision: February 9, 2018
Revised: April 8, 2018
Accepted: April 16, 2018
Article in press: April 17, 2018
Published online: June 16, 2018
Processing time: 159 Days and 3.4 Hours
Patient presented to the hospital with 3 mo of persistent intermittent headaches, fevers, nausea/vomiting, cough, swollen neck lymph nodes, and weight loss.
Patient had multilevel neck lymphadenopathy on exam, significant splenomegaly, swelling of lower extremities upon examination, concerned for malignancy.
Neoplastic etiology, such lymphoma and other malignancies were considered in the differential diagnoses.
Complete blood cell count showed anemia.
Magnetic resonance imaging of the abdomen revealed significant, small bilateral pleural effusions, splenomegaly of 19 cm and multiple retroperitoneal nodes.
A cervical lymph node biopsy revealed entirely effaced nodal architecture with scattered neoplastic large lymphoid cells in the background of small T-cell and histiocytes, consistent with T-cell/histiocyte-rich large B cell lymphoma.
The patient was treated with cyclophosphamide, oncovin, prednisone (COP), and prophylactic intrathecal therapy with methotrexate alternating with cytarabine.
T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is rare in pediatric population. Previous reports have also shown that pediatric patients with THRLBCL may also present with head and neck lymphadenopathy, splenomegaly, and similar rates of bone marrow involvement compared to adult patients. Pediatric patients tend to have a better prognosis and respond better to therapy compared to adult patients despite there being an overall 3-year survival rate of 50%-64%. Despite knowing pediatric patients do better than adult patients, it is imperative to diagnose THRLBCL as early as possible because prognosis worsens if treatment delayed. Barriers to proper diagnosis of THRLBCL in a pediatric patient are misdiagnosing it as NLPHL and cHL.
HL: Hodgkin lymphoma; NHL: Non-Hodgkin lymphoma; THRLBCL: T-cell/histiocyte-rich large B-cell lymphoma; DLBCL: Diffuse large B-cell lymphoma; LAD: Lymphadenopathy; NLPHL: Nodular lymphocyte-predominant Hodgkin lymphoma; cHL: Classic Hodgkin lymphoma; COP: Cyclophosphamide, oncovin, prednisone; H and E: Hematoxylin and Eosin stain.
THRLBCL should be considered as a differential diagnosis in a pediatric patient presenting with persistent head and neck lymphadenopathy.