John Kuruvilla, MD, FRCPC, Princess Margaret Cancer Center, Toronto, Canada, comments on the findings of a study comparing the quality of life (QoL) of patients with relapsed/refractory (R/R) classical Hodgkin lymphoma (HL) treated with pembrolizumab vs brentuximab vedotin. The study demonstrated that pembrolizumab was more effective and better tolerated than brentuximab vedotin. This interview took place at the European Hematology Association (EHA) Congress 2022 held in Vienna, Austria.

Hodgkin lymphoma must be distinguished from non-cancerous causes of lymph node swelling (such as various infections) and from other types of cancer. Definitive diagnosis is by lymph node biopsy (usually excisional biopsy with microscopic examination). Blood tests are also performed to assess function of major organs and to assess safety for chemotherapy. Positron emission tomography (PET) is used to detect small deposits that do not show on CT scanning. PET scans are also useful in functional imaging (by using a radiolabeled glucose to image tissues of high metabolism). In some cases, a Gallium scan may be used instead of a PET scan.

TypesEdit

There are two main types of Hodgkin lymphoma: classic Hodgkin lymphoma and nodular lymphocyte predominant Hodgkin lymphoma. The prevalence of classic Hodgkin lymphoma and nodular lymphocyte Hodgkin lymphoma are approximately 90% and 10%, respectively.[22][23] The morphology, phenotype, molecular features, and, therefore, the clinical behaviour and presentation of the two types differ.[24]

ClassicEdit

Classic Hodgkin lymphoma (excluding nodular lymphocyte predominant Hodgkin lymphoma) can be subclassified into four pathologic subtypes based upon Reed–Sternberg cell morphology and the composition of the reactive cell infiltrate seen in the lymph node biopsy specimen (the cell composition around the Reed–Sternberg cell(s)).

markers (such as CD20) are not expressed on all cells,[25] Reed–Sternberg cells are usually of B cell origin.[26][27] Although Hodgkin's is now frequently grouped with other B-cell malignancies, some T-cell markers (such as CD2 and CD4) are occasionally expressed.[28] However, this may be an artifact of the ambiguity inherent in the diagnosis.

Hodgkin cells produce interleukin-21 (IL-21), which was once thought to be exclusive to T-cells. This feature may explain the behavior of classic Hodgkin lymphoma, including clusters of other immune cells gathered around HL cells (infiltrate) in cultures.[29]

Nodular lymphocyte predominantEdit

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is another subtype of Hodgkin lymphoma distinct from Classic Hodgkin lymphoma and is characterized by the presence of popcorn cells which express CD20.[23][30] Due to these differences, among others, NLPHL is often treated differently from Classic Hodgkin lymphoma, including using rituximab in combination with AVBD chemotherapy, though individual cases vary and clinical trials are ongoing.[23]

StagingEdit

The staging is the same for both Hodgkin and non-Hodgkin lymphomas.

After Hodgkin lymphoma is diagnosed, a person will be staged: that is, they will undergo a series of tests and procedures that will determine what areas of the body are affected. These procedures may include documentation of their histology, a physical examination, blood tests, chest X-ray radiographs, computed tomography (CT)/Positron emission tomography (PET)/magnetic resonance imaging (MRI) scans of the chest, abdomen and pelvis, and usually a bone marrow biopsy. Positron emission tomography (PET) scan is now used instead of the gallium scan for staging. On the PET scan, sites involved with lymphoma light up very brightly enabling accurate and reproducible imaging.[31] In the past, a lymphangiogram or surgical laparotomy (which involves opening the abdominal cavity and visually inspecting for tumors) were performed. Lymphangiograms or laparotomies are very rarely performed, having been supplanted by improvements in imaging with the CT scan and PET scan.[32]

On the basis of this staging, the person will be classified according to a staging classification (the Ann Arbor staging classification scheme is a common one):

Stage I is involvement of a single lymph node region (I) (mostly the cervical region) or single extralymphatic site (Ie);

Stage II is involvement of two or more lymph node regions on the same side of the diaphragm (II) or of one lymph node region and a contiguous extralymphatic site (IIe);

Stage III is involvement of lymph node regions on both sides of the diaphragm, which may include the spleen (IIIs) or limited contiguous extralymphatic organ or site (IIIe, IIIes);

Stage IV is disseminated involvement of one or more extralymphatic organs.

The absence of systemic symptoms is signified by adding "A" to the stage; the presence of systemic symptoms is signified by adding "B" to the stage. For localised extranodal extension from mass of nodes that does not advance the stage, subscript "E" is added. Splenic involvement is signified by adding "S" to the stage. The inclusion of "bulky disease" is signified by "X".

Omaha, Neb - Christen Nino De Guzman knew she was sick. The 21-year-old student at the University of Nebraska-Lincoln was sleeping all day and missing class because she felt so tired. She assumed she had the flu.
"I went to a hospital in Lincoln and they told me I was anemic," said Nino De Guzman. "But in the next few weeks, I developed horrible night sweats -- my sheets and clothes would just be soaked."
She soon ended up in an emergency room and was given antibiotics for bronchitis. Unfortunately, she continued to get sicker.
"I finished school for the summer and was back home in Omaha," she said. "My mom took me to a hospital here and they discovered I had tumors throughout my abdomen. But after two weeks of testing, they still didn't have a definitive diagnosis. Meanwhile, my stomach was blowing up like I was seven months pregnant. Doctors thought it was from all the fluids I was getting, but in reality it was the tumors tripling in size."
That's when her mom had Christen transferred to The Nebraska Medical Center. "Immediately, there was a team of doctors around my bed trying to figure out what was wrong," said Nino De Guzman. "Within a day or two, they told me I had a type of non-Hodgkins lymphoma called Burkitt's lymphoma. I remember one of the E.R. doctors telling me they thought it was stage four, but he said 'I think we can cure this.' I would cling to those words for the next four months."
"The kind of lymphoma Christen had accounts for only 1% of adult lymphomas," said Philip Bierman, MD, hematologist/oncologist at The Nebraska Medical Center and one of the physicians who treated Christen. "It requires aggressive chemotherapy, but it can be extremely difficult to diagnose and there are other kinds of lymphoma that can be confused with it."
"Patients like Christen should be treated at a place that has the experience with the chemotherapy that's required for this lymphoma," said Dr. Bierman. "In this part of the country, we probably have the most experience with it."

After several rounds of intense and complex chemotherapy, Christen was recently declared cancer-free. She just returned from a trip to New York City and has big plans for the future.
"I feel so thankful to live so close to the number one hospital in the world for treating my kind of lymphoma," said Nino De Guzman. "It's a place where people come from all over the world to get treated. This will definitely be a special Thanksgiving for my family and me."

For more information about lifesaving lymphoma treatment call 1-800-922-0000.