About

Pediatric Non-Hodgkin Lymphoma (NHL)


About Pediatric Non-Hodgkin Lymphoma

Non-Hodgkin lymphomas comprise approximately 5% of all cancers in children, and about 800 cases of NHL are diagnosed in the United States yearly. In general, the long-term prognosis for children with NHL is excellent, and more than 80% of children are cured within a year after diagnosis.


NHL is not just one disease but a category of cancers that begin in the lymphocytes and attack the lymphatic system. The lymphatic system is part of the immune system and serves to fight disease and infections. NHL causes cells to reproduce at an abnormally fast pace, which leads to the growth of tumors. The cancerous cells can also spread to other organs and tissues in the body. Typically, NHL is an aggressive cancer, yet some patients with fast-growing NHL can be totally cured. With slow-growing NHL, treatments are often effective at stabilizing the cancer over the long-term.

The cause of NHL remains largely unknown, and most cases of NHL in children are not caused by anything that could have been prevented. However, there are some risk factors that may raise a child’s probability of developing NHL, such as:

  • Age – it is more common in older children than in younger ones
  • Gender – it is slightly more common in boys than in girls
  • Race – it is more common in white children than in the African American population
  • Weakened immune system – due to congenital or acquired immunodeficiency, such as HIV/AIDS, having taken immunosuppressive drugs after an organ transplant, or having a genetic syndrome, including:
    • Wiskott-Aldrich syndrome
    • Severe combined immunodeficiency syndrome (SCID)
    • Ataxia-telangiectasia
    • Common variable immunodeficiency
    • X-linked lymphoproliferative syndrome
  • Radiation exposure – such as having survived an atomic explosion or nuclear reactor accidents
  • Epstein-Barr virus infection – EBV has been linked with about 15% of all Burkitt lymphomas (a type of NHL) in the United States.
  • Family history – some research has suggested that having a sibling or parent with NHL might be a risk factor

The symptoms of NHL vary depending largely upon the type of lymphoma and where it is located. The location of where the tumor is growing often explains the symptoms. Common signs and symptoms in children include:

  • Swollen lymph nodes in neck, chest, abdomen, underarm, or groin; usually painless
  • Fever
  • Sore throat or cough
  • Feeling full after only a small amount of food
  • Bone and joint pain
  • Shortness of breath
  • Night sweats
  • Tiring easily (fatigue)
  • Weight loss/decreased appetite
  • Itching of the skin
  • Recurring infections, if white blood cell counts are low
  • Easy bruising or bleeding, if blood platelet counts are low
  • Anemia, if red blood cell counts are low

NHL can occur in both adults and children, but the types of NHL that are typically diagnosed in children are different from those in adults. In general, NHL is classified by how the cancer cells appear under the microscope. The key features include the size, shape, and growth pattern of the cells.

While there are many types of NHL, most cases of pediatric NHL are classified as one of the three following types, all of which are high-grade (fast-growing) but require different treatments:

  1. Lymphoblastic lymphoma (LBL): accounts for about 25% to 30% of childhood NHL in the United States. LBL can grow very rapidly and often causes trouble breathing, so it needs to be diagnosed and treated quickly.
  2. Burkitt lymphoma (small non-cleaved cell lymphoma): accounts for about 40% of childhood NHL in the United States. This lymphoma begins in lymphatic system and can spread to other organs, including the brain. It is one of the fastest growing cancers known, so it needs to be diagnosed and treated quickly.
  3. Large cell lymphoma: these lymphomas originate in more mature forms of T cells or B cells and can grow almost anywhere in the body. They do not grow as quickly as other childhood lymphomas and tend to occur more frequently in older children and teens. There are two main subtypes of large cell lymphoma – anaplastic large cell lymphoma (ALCL) and diffuse large B-cell lymphoma (DLBCL).

What is non-Hodgkin lymphoma?

NHL is not just one disease but a category of cancers that begin in the lymphocytes and attack the lymphatic system. The lymphatic system is part of the immune system and serves to fight disease and infections. NHL causes cells to reproduce at an abnormally fast pace, which leads to the growth of tumors. The cancerous cells can also spread to other organs and tissues in the body. Typically, NHL is an aggressive cancer, yet some patients with fast-growing NHL can be totally cured. With slow-growing NHL, treatments are often effective at stabilizing the cancer over the long-term.

 

What are the risk factors for non-Hodgkin lymphoma?

The cause of NHL remains largely unknown, and most cases of NHL in children are not caused by anything that could have been prevented. However, there are some risk factors that may raise a child’s probability of developing NHL, such as:

  • Age – it is more common in older children than in younger ones
  • Gender – it is slightly more common in boys than in girls
  • Race – it is more common in white children than in the African American population
  • Weakened immune system – due to congenital or acquired immunodeficiency, such as HIV/AIDS, having taken immunosuppressive drugs after an organ transplant, or having a genetic syndrome, including:
    • Wiskott-Aldrich syndrome
    • Severe combined immunodeficiency syndrome (SCID)
    • Ataxia-telangiectasia
    • Common variable immunodeficiency
    • X-linked lymphoproliferative syndrome
  • Radiation exposure – such as having survived an atomic explosion or nuclear reactor accidents
  • Epstein-Barr virus infection – EBV has been linked with about 15% of all Burkitt lymphomas (a type of NHL) in the United States.
  • Family history – some research has suggested that having a sibling or parent with NHL might be a risk factor

 

What are the symptoms of non-Hodgkin lymphoma?

The symptoms of NHL vary depending largely upon the type of lymphoma and where it is located. The location of where the tumor is growing often explains the symptoms. Common signs and symptoms in children include:

  • Swollen lymph nodes in neck, chest, abdomen, underarm, or groin; usually painless
  • Fever
  • Sore throat or cough
  • Feeling full after only a small amount of food
  • Bone and joint pain
  • Shortness of breath
  • Night sweats
  • Tiring easily (fatigue)
  • Weight loss/decreased appetite
  • Itching of the skin
  • Recurring infections, if white blood cell counts are low
  • Easy bruising or bleeding, if blood platelet counts are low
  • Anemia, if red blood cell counts are low

 

How many types of non-Hodgkin lymphoma are there?

NHL can occur in both adults and children, but the types of NHL that are typically diagnosed in children are different from those in adults. In general, NHL is classified by how the cancer cells appear under the microscope. The key features include the size, shape, and growth pattern of the cells.

While there are many types of NHL, most cases of pediatric NHL are classified as one of the three following types, all of which are high-grade (fast-growing) but require different treatments:

  1. Lymphoblastic lymphoma (LBL): accounts for about 25% to 30% of childhood NHL in the United States. LBL can grow very rapidly and often causes trouble breathing, so it needs to be diagnosed and treated quickly.
  2. Burkitt lymphoma (small non-cleaved cell lymphoma): accounts for about 40% of childhood NHL in the United States. This lymphoma begins in lymphatic system and can spread to other organs, including the brain. It is one of the fastest growing cancers known, so it needs to be diagnosed and treated quickly.
  3. Large cell lymphoma: these lymphomas originate in more mature forms of T cells or B cells and can grow almost anywhere in the body. They do not grow as quickly as other childhood lymphomas and tend to occur more frequently in older children and teens. There are two main subtypes of large cell lymphoma – anaplastic large cell lymphoma (ALCL) and diffuse large B-cell lymphoma (DLBCL).

 

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