Hodgkin disease

Other Names: Hodgkin disease; Hodgkin's lymphoma; Lymphoma, Hodgkin's

Hodgkin disease (HOJ-kin dih-ZEEZ) is a cancer of the immune system that is marked by the presence of a type of cell called the Reed-Sternberg cell. Hodgkin lymphoma (HL) is a heterogeneous group of malignant lymphoid neoplasms of B-cell origin characterized histologically by the presence of Hodgkin and Reed-Sternberg (HRS) cells in the vast majority of cases.

Micrograph of a classic Reed–Sternberg cell

Types

HL is comprised of 2 major forms: classical Hodgkin lymphoma (CHL; see this term), seen in 95% of all HL cases, and nodular lymphocyte predominant Hodgkin lymphoma (NLPHL; ), seen in only 5% of all HL cases. Disease onset occurs most frequently in young adults (age 15-35) followed by older adults (over the age of 55) and usually begins with the painless swelling of a lymph node in the upper body. Disease can spread to multiple lymph node regions or lymphoid structures and associated systemic symptoms (weight loss > 10% of baseline, fevers, night sweats) are observed in about 20% of patients.

Cause

The exact cause is unknown but immunological, genetic and environmental factors are thought to be involved.

Risk factors

There are two major types of Hodgkin lymphoma: classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. About half of cases of Hodgkin lymphoma are due to Epstein–Barr virus (EBV) and these are generally the classic form. The virus-positive cases are classified as a form of the Epstein-Barr virus-associated lymphoproliferative diseases. Other risk factors include a family history of the condition and having HIV/AIDS. Exposure to benzene

Epidemiology

HL is an uncommon cancer with an incidence of about 1/40,000 in North America and Europe. There are about 8500 new cases reported in the U.S. each year.

Symptoms

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Fatigue(Tired)
• Immunodeficiency(Decreased immune function)
• Lymphadenopathy(Swollen lymph nodes)
• Lymphoma(Cancer of lymphatic system)

30%-79% of people have these symptoms

• Anorexia
• Chest pain
• Cough(Coughing)
• Fever
• Hyperhidrosis(Excessive sweating)
• Pruritus(Itching)
• Weight loss

5%-29% of people have these symptoms

• Ataxia
• Bone pain
• Dyspnea(Trouble breathing)
• Headache(Headaches)
• Hemoptysis(Coughing up blood)
• Hepatomegaly(Enlarged liver)
• Peripheral neuropathy
• Splenomegaly(Increased spleen size)

Staging

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.

Diagnosis

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.

Additional images

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  Stage 1 Hodgkin lymphoma

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  Stage 2 Hodgkin lymphoma

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  Stage 3 Hodgkin lymphoma

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  Stage 4 Hodgkin lymphoma

Treatment

People with early stage disease (IA or IIA) are effectively treated with radiation therapy or chemotherapy. The choice of treatment depends on the age, sex, bulk and the histological subtype of the disease.

Adding localised radiation therapy after the chemotherapy regimen may provide a longer progression-free survival compared with chemotherapy treatment alone.

People with later disease (III, IVA, or IVB) are treated with combination chemotherapy alone. People of any stage with a large mass in the chest are usually treated with combined chemotherapy and radiation therapy. The original treatment for Hodgkin's was MOPP. The abbreviation stands for the four drugs Mustargen (also known as chlormethine), Oncovin (also known as vincristine), Prednisone and Procarbazine (also known as Matulane). The treatment is usually administered in four week cycles, often for six cycles. MSD and VCR are administered intravenously, while procarbazine and prednisone are pills taken orally. MOPP was the first combination chemotherapy brought in that achieved a high success rate. Currently, the ABVD chemotherapy regimen is the standard treatment of Hodgkin's disease in the US.

BEACOPP is a form of treatment for stages > II mainly used in Europe.

For Hodgkin lymphomas, radiation oncologists typically use external beam radiation therapy (sometimes shortened to EBRT or XRT). Radiation oncologists deliver external beam radiation therapy to the lymphoma from a machine called a linear accelerator which produces high energy X-rays and electrons. People usually describe treatments as painless and similar to getting an X-ray. Treatments last less than 30 minutes each. The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Brentuximab vedotin (Brand name: Adcetris)brentuximab vedotin (Adcetris) was approved for the treatment of adult patients with previously untreated Stage III or IV classical Hodgkin lymphoma (cHL), in combination with chemotherapy.

In August 2015, it was approved for treatment of patients with classical Hodgkin lymphoma at high risk of relapse or progression as post-autologous hematopoietic stem cell transplantation (auto-HSCT). In August 2011, it was approved for the treatment of patients with Hodgkin lymphoma after failure of autologous stem cell transplant (ASCT) or after failure of at least two prior multi-agent chemotherapy regimens in patients who are not ASCT candidates.

• Pembrolizumab (Brand name: Keytruda) Treatment of adult and pediatric patients with refractory classical Hodgkin lymphoma (cHL), or those who have relapsed after three or more prior lines of therapy.
• Nivolumab (Brand name: Opdivo) Treatment of adult patients with Classical Hodgkin lymphoma that has relapsed or progressed after 3 or more lines of systemic therapy that includes autologous HSCT.

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