Nodular Lymphocyte-Predominant Hodgkin Lymphoma (NLPHL)

Hodgkin lymphoma (HL) is a blood cancer that develops in the lymphatic system and affects a type of white blood cells called B-lymphocytes. In normal circumstances, these B-lymphocytes produce antibodies that target viruses, bacteria, cancer cells, and other foreign substances. Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare subtype of Hodgkin lymphoma that accounts for about 5% of all HL cases and is characterized by large ‘popcorn-like’ cancerous cells.

NLPHL usually starts in lymph nodes in the neck or armpit. It can occur in people of any age, although it is most common in people between 30 and 50 years old. NLPHL is much more common in males than in females

The diagnostic process for NLPHL starts with a physical examination, during which the doctor palpates lymph nodes and other areas to check for swelling. The doctor will also assess the medical history of the patient to better understand the general health status and any potential risk factors. If a lymphoma is suspected, a lymph node biopsy is performed to make a final diagnosis. This is a simple procedure during which all, or part of a lymph node is removed from the body and sent to the laboratory for a microscopic evaluation. If this analysis reveals the presence of ‘popcorn cells’, additional blood tests can be performed. In patients with an advanced disease stage, a bone marrow biopsy and imaging tests are used to look for signs of NLPHL in other areas of your body (X-ray, CT, PET, MRI, ultrasound).

In most patients, NLPHL is diagnosed in an early disease stage, meaning that the lymphoma is only growing in one place (or a few places close together). For these patients, the treatment can often be limited to the area that is affected by the lymphoma. If the lymphoma is growing in an easy-to-reach place, surgery can be offered. However, when surgery is not an option, radiotherapy is the preferred treatment strategy. Of note, radiotherapy can also be offered following surgery when there is a suspicion that some lymphoma cells are left behind.

For patients with advanced-stage NLPHL, the standard treatment consists of chemotherapy, often in combination with the monoclonal antibody rituximab. For frail patients, rituximab may also be given as monotherapy. For a proportion of patients, chemotherapy is followed by radiotherapy.

Although the treatment for NLPHL is usually effective, the disease can come back after a while (relapse) or may respond poorly to the initial treatment (refractory). For patients with relapsed/refractory NLPHL, the treatment mainly consists of chemotherapy (with different agents than the ones used in first line) in combination with rituximab. If the relapse is local, radiotherapy can also be considered. For patients with a very rapid relapse, or for patients with multiple, consecutive relapses, a stem cell transplantation can be an option (only if the patient is fit enough to undergo this intensive treatment).