Hodgkin lymphoma- childhood

Overview

Hodgkin's lymphoma — formerly known as Hodgkin's disease — is a cancer of the lymphatic system, which is part of your immune system.

In Hodgkin's lymphoma, cells in the lymphatic system grow abnormally and may spread beyond the lymphatic system. As Hodgkin's lymphoma progresses, it compromises your body's ability to fight infection.

Hodgkin's lymphoma is one of two common types of cancers of the lymphatic system. The other type, non-Hodgkin's lymphoma, is far more common.

Most Hodgkin lymphomas are the classical type. The classical type is broken down into the following four sub-types:

  • Nodular sclerosing Hodgkin lymphoma.
  • Mixed cellularity Hodgkin lymphoma.
  • Lymphocyte depletion Hodgkin lymphoma.
  • Lymphocyte-rich classical Hodgkin lymphoma

Symptoms

These and other symptoms may be caused by childhood Hodgkin lymphoma or by other conditions. A doctor should be consulted if any of the following problems occur: Painless, swollen lymph nodes in the neck, underarm

  • Fever for no known reason
  • Drenching night sweats
  • Weight loss for no known reason
  • Itchy skin
  • Feeling very tired

Causes

It's not clear what causes Hodgkin's lymphoma.

Doctors know that most Hodgkin's lymphoma occurs when an infection-fighting cell called a B cell develops a mutation in its DNA. The mutation tells the cells to divide rapidly and to continue living when a healthy cell would die. The mutation causes a large number of oversized, abnormal B cells to accumulate in the lymphatic system, where they crowd out healthy cells and cause the signs and symptoms of Hodgkin's lymphoma.

Prevention

  • A family history of lymphoma: Having a close family member who has Hodgkin's lymphoma or non-Hodgkin's lymphoma increases your risk of developing Hodgkin's lymphoma.
  • Your sex: Males are slightly more likely to develop Hodgkin's lymphoma.
  • Past Epstein-Barr infection: People who have had illnesses caused by the Epstein-Barr virus, such as infectious mononucleosis, are more likely to develop Hodgkin's lymphoma than are people who haven't had Epstein-Barr infections.
  • A weakened immune system.:Having a compromised immune system, such as from HIV/AIDS or from having an organ transplant requiring medications to suppress the immune response, increases the risk of Hodgkin's lymphoma.

Diagnosis

Tests and procedures used to diagnose Hodgkin's lymphoma include:

  • Physical exam. Your doctor checks for swollen lymph nodes, including in your neck, underarm and groin, as well as a swollen spleen or liver.
  • Blood tests. A sample of your blood is examined in a lab to see if anything in your blood indicates the possibility of cancer.
  • Imaging tests. Imaging tests used to diagnose Hodgkin's lymphoma include X-ray, computerized tomography (CT) scan and positron emission tomography (PET-scan).
  • Surgery to remove a swollen lymph node. Minor surgery may be done to remove all or part of an enlarged lymph node for testing. The lymph node is sent to a laboratory for testing. A diagnosis of Hodgkin's lymphoma is made if the abnormal Reed-Sternberg cells are found within the lymph node.
  • A procedure to collect bone marrow for testing. A bone marrow biopsy may be used to look for signs of cancer in the bone marrow. During this procedure, a small amount of bone marrow, blood and bone are removed through a needle.

After your doctor has determined the extent of your Hodgkin's lymphoma, your cancer will be assigned a stage. Your cancer's stage helps determine your prognosis and your treatment options.

Stages of Hodgkin's lymphoma include:

  • Stage I. The cancer is limited to one lymph node region or a single organ.
  • Stage II. In this stage, the cancer is in two lymph node regions or the cancer has invaded one organ and the nearby lymph nodes. But the cancer is still limited to a section of the body either above or below the diaphragm.
  • Stage III. When the cancer moves to lymph nodes both above and below the diaphragm, it's considered stage III. Cancer may also be in one portion of tissue or an organ near the lymph node groups or in the spleen.
  • Stage IV. This is the most advanced stage of Hodgkin's lymphoma. Cancer cells are in several portions of one or more organs and tissues. Stage IV Hodgkin's lymphoma affects not only the lymph nodes but also other parts of your body, such as the liver, lungs or bones.

Additionally, your doctor uses the letters A and B to indicate whether you're experiencing symptoms of Hodgkin's lymphoma:

  • A means that you don't have any significant symptoms as a result of the cancer.
  • B indicates that you may have significant signs and symptoms, such as a persistent fever, unintended weight loss or severe night sweats.

Prognosis

The prognosis (chance of recovery) and treatment options depend on the following:

  • The stage of the cancer.
  • The size of the tumor and how quickly it shrinks after initial treatment.
  • The patient's symptoms when diagnosed.
  • Certain features of the cancer cells.

Whether the cancer is newly diagnosed, does not respond to initial treatment, or has recurred.

Treatment

Patients 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 controls the tumors better and provides a better chance for survival than chemotherapy alone. Patients with later disease (III, IVA, or IVB) are treated with combination chemotherapy alone. Patients of any stage with a large mass in the chest are usually treated with combined chemotherapy and radiation therapy.

It should be noted that the common non-Hodgkin's treatment, rituximab (which is a monoclonal antibody against CD20) is not routinely used to treat Hodgkin's lymphoma due to the lack of CD20 surface antigens in most cases. The use of rituximab in Hodgkin's lymphoma, including the lymphocyte predominant subtype has been reviewed recently.

Although increased age is an adverse risk factor for Hodgkin's lymphoma, in general elderly patients without major comorbidities are sufficiently fit to tolerate standard therapy, and have a treatment outcome comparable to that of younger patients. However, the disease is a different entity in older patients and different considerations enter into treatment decisions.

For Hodgkin's 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 linear accelerator which produces high energy X Rays and Electrons. Patients usually describe treatments as painless and similar to getting an X-ray. Treatments last less than 30 minutes each.

For lymphomas, there are a few different ways radiation oncologists target the cancer cells. Involved field radiation is when the radiation oncologists give radiation only to those parts of the patient's body known to have the cancer. Very often, this is combined with chemotherapy. Radiation therapy directed above the diaphragm to the neck, chest and/or underarms is called mantle field radiation. Radiation to below the diaphragm to the abdomen, spleen and/or pelvis is called inverted-Y field radiation. Total nodal irradiation is when the therapist gives radiation to all the lymph nodes in the body to destroy cells that may have spread.

Adverse effects:

The high cure rates and long survival of many patients with Hodgkin's lymphoma has led to a high concern with late adverse effects of treatment, including cardiovascular disease and second malignancies such as acute leukemias, lymphomas, and solid tumors within the radiation therapy field. Most patients with early-stage disease are now treated with abbreviated chemotherapy and involved-field radiation therapy rather than with radiation therapy alone. Clinical research strategies are exploring reduction of the duration of chemotherapy and dose and volume of radiation therapy in an attempt to reduce late morbidity and mortality of treatment while maintaining high cure rates. Hospitals are also treating those who respond quickly to chemotherapy with no radiation.

In childhood cases of Hodgkin's lymphoma, long-term endocrine adverse effects are a major concern, mainly gonadal dysfunction and growth retardation. Gonadal dysfunction seems to be the most severe endocrine long-term effect, especially after treatment with alkylating agents and/or pelvic radiotherapy.