Childhood Hodgkin Lymphoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Cellular Classification and Biologic Correlates
Nodular Lymphocyte-Predominant Hodgkin Lymphoma
There are variable estimates for the relative frequency of nodular lymphocyte-predominant Hodgkin lymphoma in the pediatric population, ranging from 5% to 10%. The relative frequency is higher for children younger than 10 years compared with children aged 10 to 19 years. Nodular lymphocyte-predominant Hodgkin lymphoma is most common in males younger than 18 years.
Patients with nodular lymphocyte-predominant Hodgkin lymphoma generally present with localized, nonbulky disease that infrequently involves the mediastinum. Almost all patients are asymptomatic.
Nodular lymphocyte-predominant Hodgkin lymphoma is characterized by molecular and immunophenotypic evidence of B-lineage differentiation with the following distinctive features:
Nodular lymphocyte-predominant Hodgkin lymphoma is characterized by large cells with multilobed nuclei, referred to as popcorn cells. These cells express B-cell antigens, such as CD19, CD20, CD22, and CD79A, and are negative for CD15 and may or may not express CD30.
The OCT-2 and BOB.1 oncogenes are both expressed in nodular lymphocyte-predominant Hodgkin lymphoma; they are not expressed in the cells of patients with classical Hodgkin lymphoma.
Reliable discrimination from non-Hodgkin lymphoma is problematic in diffuse subtypes with lymphocytic and histiocytic cells set against a diffuse background of reactive T-cells.
Nodular lymphocyte-predominant Hodgkin lymphoma can be difficult to distinguish from progressive transformation of germinal centers and/or T-cell-rich B-cell lymphoma.
Chemotherapy and/or radiation therapy produce excellent long-term progression-free survival and overall survival in patients with nodular lymphocyte-predominant Hodgkin lymphoma; however, late recurrences have been reported up to 10 years after initial therapy.[19,20]
Deaths observed among individuals with nodular lymphocyte-predominant Hodgkin lymphoma are more frequently related to treatment complications and/or the development of subsequent neoplasms (including non-Hodgkin lymphoma), underscoring the importance of judicious use of chemotherapy and radiation therapy at initial presentation and after recurrent disease.[19,20]
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