Nodular Sclerosing Hodgkin Lymphoma

Lymphatic system
Artistic depiction of the lymphatic system including lymph nodes and lymph vessels. Red illustrates the concept that particular lymph nodes may be involved, depending on the disease.

Nodular sclerosing Hodgkin lymphoma, a cancer of lymph cells, is a type of Hodgkin disease. It is the most common type of Hodgkin lymphoma in the developed countries, accounting for 60 to 80 percent of all individuals affected by this disease.

Nodular sclerosing Hodgkin lymphoma affects mostly adolescents and young adults. It is also slightly more common in girls than boys. The name ‘nodular sclerosing’ is derived from the appearance of lymph node tissue under the microscope in this type of Hodgkin lymphoma.

The main symptoms of nodular sclerosing Hodgkin lymphoma are enlarged lymph nodes in the neck, armpits or within the chest. The diagnosis of lymphoma is made with a lymph node biopsy.

Most individuals are diagnosed in early stages and can be cured with treatment that includes chemotherapy and radiation therapy.

One of Several Different Hodgkin Lymphomas

The names of all the lymphoma types and subtypes are confusing even to doctors who do not deal with them regularly. Hodgkin disease and Hodgkin lymphoma mean exactly the same thing, however, there are many different types lymphoma, of which Hodgkin is just one. And there are several different types of Hodgkin lymphoma, too. So, here is a run-down of nodular sclerosing Hodgkin lymphoma in terms of where it fits into the bigger picture of all lymphomas:

The 2 basic categories of lymphoma are Hodgkin and Non-Hodgkin lymphoma.

Hodgkin lymphoma can then be further broken down into 2 groups:

  • Classic Hodgkin lymphoma – which has several subtypes
  • Nodular lymphocyte-predominant Hodgkin lymphoma

“Nodular lymphocyte predominant” and “nodular sclerosing” are not the same thing – they refer to two different kinds of Hodgkin lymphoma. Nodular Sclerosing Hodgkin lymphoma – the lymphoma currently being explained – is a subtype of Classic Hodgkin lymphoma. Classic Hodgkin lymphoma has the following four subtypes:

  • Nodular sclerosing
  • Mixed cellularity
  • Lymphocyte-rich
  • Lymphocyte-depleted.

Updates: Nodular Sclerosing Classic Hodgkin Lymphoma

Nodular sclerosis Hodgkin lymphoma (NSHL) is the most common tissue-pathology-based subtype among adolescents and young adults in industrialized countries. NSHL also has a strong inheritable component, with a 100-fold increased risk to identical twins.

Recently, there was a large study done on the association of ultraviolet radiation from the sun and different subtypes of Hodgkin lymphoma. In the study by Emily Bowen and colleagues, Hodgkin lymphoma incidence was lowest in the groups of people with the highest ultraviolet sun exposure. Though this finding was a trend, only, this trend did appear to hold true in people with the nodular sclerosis subtype.

Other recent studies have been exploring a particular therapy, immune checkpoint inhibition, and how it may interact with different processes that are known to be going on in certain forms of classical HL.

A study by Peter Hollander and colleagues noted that immune checkpoint inhibition targeting the programmed death receptor (PD)-1 pathway is a new treatment approach in relapsed and refractory classical Hodgkin lymphoma. This group found that certain characteristics of the cells found all around the tumor, and not of the tumor cells themselves, were linked to prognosis. The study found that outcomes in classic HL were worse when these surrounding white blood cells, or microenvironment cells, were in large proportions making the PD-1 and PD-L1. They proposed that these subgroups of patients might benefit from more aggressive chemotherapy regimens and, possibly, from PD-1 inhibitors as front-line treatment.

View Article Sources
  • Cozen W, Li D, Best T, et al. A genome-wide meta-analysis of nodular sclerosing Hodgkin lymphoma identifies risk loci at 6p21.32. Blood. 2012;119(2):469-475.
  • Hollander P, Kamper P, Ekstrom K, et al. High proportions of PD-1+ and PD-L1+ leukocytes in classical Hodgkin lymphoma microenvironment are associated with inferior outcome. Blood Adv. 2017; 1(18): 1427–1439.
  • Bowen EM, Pfeiffer RM, Linet MS, et al. Relationship between ambient ultraviolet radiation and Hodgkin lymphoma subtypes in the United States. British Journal of Cancer. 2016;114(7):826-831.