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Cancer Predisposition In Children 

In this impressive photo, the large round cell is a lymphocyte. Macrophages with projectile-looking surfaces are interacting with it. Photo is Courtesy of Dr. Timothy Triche. National Cancer Institute.
In this impressive photo, the large round cell is a lymphocyte. Macrophages with projectile-looking surfaces are interacting with it. Courtesy of Dr. Timothy Triche. National Cancer Institute.

What Is Cancer Predisposition In Children?

[dropcap]C[/dropcap]ancer predisposition in children signifies a higher than normal risk of developing cancer. Cancer is the uncontrolled division of abnormal cells in the body.

Among the 12 major types of childhood cancers, leukemias (blood cell cancers) and cancers of the brain and central nervous system account for more than half of the new cases. About one-third of childhood cancers are leukemias.

The most common type of leukemia in children is acute lymphoblastic leukemia. The most common solid tumors are brain tumors (e.g., gliomas and medulloblastomas), with other solid tumors (e.g., neuroblastomas, Wilms tumors, and sarcomas such as rhabdomyosarcoma and osteosarcoma) being less common according to the National Cancer Institute.

On average, 1 to 2 children develop the disease each year for every 10,000 children in the United States.

What Is Cancer Predisposition In Children In Celiac Disease and/or Gluten Sensitivity?

Lymphoma, Enteropathy-Associated T-Cell (EATL) 

EATL of Jejunum.Courtesy pubcan.org
EATL of Jejunum with Thickening And Yellowish Ulcers Visible. Courtesy pubcan.org

What Is Enteropathy-Associated T-Cell Lymphoma?

[dropcap]E[/dropcap]nteropathy associated T-cell lymphoma (EATL), although rare, is a tumor of intraepithelial lymphocytes. It is the most common primary gastrointestinal T-cell lymphoma and is characterized by its aggressive course and poor prognosis.

Primary means this malignancy starts out in the intestinal wall rather than spreading to it from a tumor somewhere else in the  body.

EATL usually affects the jejunum and grossly (visible to the eye) appears as multiple ulcers causing circumferential thickening of affected bowel wall without the formation of definite tumor masses most commonly in the proximal small bowel. As such, patients may present with intestinal perforation, obstruction or hemorrhage.1

Mesenteric lymph nodes in the abdomen are commonly involved.2

Q: How is EATL diagnosed?

A: Work-up of EATL must include immunohistology, T-cell flow cytometry, T-cell rearrangement and adequate imaging with CT and PET scanning.3

Management of EATL requires a combination of early diagnosis and treatment by surgical resection followed by chemotherapy to achieve treatment success. Overall however, the treatment completion rate remains at 50% and EATL carries a poor prognosis with a 5-year survival rate of <20%.4

What Is Enteropathy-Associated T-Cell Lymphoma In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Pun AH, Kasmeridis H, Rieger N, Loganathan A. Enteropathy associated T-cell lymphoma presenting with multiple episodes of small bowel hemorrhage and perforation. J Surg Case Rep. 2014 Mar 20;2014(3). pii: rju013. doi: 10.1093/jscr/rju013. [↩]
  2. Yang DH, Myung SJ, Chang HS, et al. A case of enteropathy-associated T-cell lymphoma presenting with recurrent hematochezia. Korean Journal of Gastroenterology = Taehan Sohwagi Hakhoe Chi. Dec 2003;42(6):527-32. [↩]
  3. Meijer JWR, Mulder CJJ, Goerres MG, Boot H, Schweizer JJ. Coeliac disease and (extra)intestinal T-cell lymphomas: definition, diagnosis and treatment. Scandanavian Journal of Gastroenterology. Dec 2004;39(Suppl 241):78,7p. [↩]
  4. Pun AH, Kasmeridis H, Rieger N, Loganathan  Enteropathy associated T-cell lymphoma presenting with multiple episodes of small bowel hemorrhage and perforation.A. J Surg Case Rep. 2014 Mar 20;2014(3). pii: rju013. doi: 10.1093/jscr/rju013. [↩]

Lymphoma, B-Cell Non-Hodgkin’s

Malignant lymphoma high grade B-cell. Courtesy Wikimedia
Malignant lymphoma high grade B-cell. Courtesy Wikimedia

What Is B-Cell Non-Hodgkin’s Lymphoma?

[dropcap]B[/dropcap]cell non-Hodgkin’s lymphoma is a malignant, monoclonal (arising from a single cell) proliferation of lymphocytes that is preceded by lymphadenopathy and characterized by varying, less predictable spread than Hodgkin’s disease.

Lymphadenopathy is enlargement of lymph nodes greater than 1.5 cm caused by activation and increased production of lymphocytes and phagocytes (type of white blood cell that engulfs pathogens during infection) or invasion by a tumor.

Q: How does this type of lymphoma develop?

A: 80% to 85% of non-Hodgkin’s lymphoma arise from B-lymphocytes (B-cells).

What Is B Cell Non-Hodgkin’s Lymphoma In Celiac Disease and/or Gluten Sensitivity?

Celiac Disease, Refractory

A small intestinal mucosa from a case of refractory coeliac disease immunostained sequentially for CD3 (alkaline phosphatase-blue) and CD8 (peroxidase-brown). Most intraepithelial lymphocytes are CD3+, CD8-.Courtesy pubcan.org
Mucosa  in refractory celiac  disease immunostained sequentially for CD3 (alkaline phosphatase-blue) and CD8 (peroxidase-brown). Most intraepithelial lymphocytes are CD3+, CD8-. Courtesy pubcan.org

What Is Refractory Celiac Disease?

[dropcap]R[/dropcap]efractory celiac disease, formerly called refractory sprue, is a severe complication characterized by persistence of symptoms and intestinal inflammation despite gluten free diet after 12 months.1

Refractory celiac disease appears in two forms, ulcerative jejunitis (RCD I) and cryptic intestinal T-cell lymphoma (RCD II).

Patients with RCD I seem to profit from immunosuppressive treatment, but positive response to corticosteroid treatment does not exclude underlying enteropathy–associated T-cell lymphoma (EATL).

Patients with RCD II have a high percentage of abberant T-cells and is usually resistant to medical therapies. The presence of an aberrant clonal intraepithelial T-cell population has led to the designation of refractory celiac disease with this population as a cryptic intestinal T-cell lymphoma, characterized by frequent dissemination to the blood and the entire gastrointestinal lining.2

  • Refractory sprue may occur after an initial response to gluten free diet or without any evidence of preexisting celiac disease. All other causes of malabsorption must be excluded, such as collagenous colitis.
  • In a subgroup of patients with enteropathy-associated T-cell lymphoma (EATL) there is progressive deterioration of a refractory form of celiac disease. The prognosis is poor, although some patients respond to corticosteroids and immunosuppressive agents.3
  • A nationwide Finnish study showed that patients of male gender, older age, severe symptoms or seronegativity (negative antibody result) at the diagnosis of celiac disease are at risk of future refractory coeliac disease and should be followed up carefully.4
  • Chorea has been described as a paraneoplastic phenomenon in patients with non-Hodgkin’s lymphoma and has been described as associated with lymphoma arising from a background of refractory celiac disease. The finding of chorea in association with celiac disease should prompt a search for possible underlying intestinal T-cell lymphoma.5

How Prevalent Is Refractory Celiac Disease?

Sources:
  1. Murray JA, The widening spectrum of celiac disease. American Journal of Clinical Nutrition. Mar 1999;69 (3):354-365. [↩]
  2. Culliford AN, Green PH. Refractory sprue. Current Gastroenterology Reports. Oct 2003;5(5):373-8. [↩]
  3. Culliford AN, Green PH. Refractory sprue. Current Gastroenterology Reports. Oct 2003;5(5):373-8. [↩]
  4. Ilus T, Kaukinen K, Virta LJ, Huhtala H, Mäki M, Kurppa K, Heikkinen M, Heikura M, Hirsi E, Jantunen K, Moilanen V, Nielsen C, Puhto M, Pölkki H, Vihriälä I, Collin P. Refractory coeliac disease in a country with a high prevalence of clinically-diagnosed coeliac disease. Aliment Pharmacol Ther. 2014 Feb;39(4):418-25. doi: 10.1111/apt.12606. [↩]
  5. Kitiyakara T, Jackson M, Gorard DA. Refractory coeliac disease, small-bowel lymphoma and chorea. J R Soc Med. 2002 Mar;95(3):133-4. [↩]

Lymphomas, Extraintestinal

Lymph System. National Cancer Institute.What Are Extraintestinal Lymphomas?

[dropcap]E[/dropcap]xtraintestinal lymphomas (non-Hodgkin’s) are malignancies that arise in peripheral lymphatic tissue outside the intestinal tract from B-cell and T-cell lymphocytes.

Q: What is peripheral lymphatic tissue?

A: Peripheral lymphatic tissue includes lymph vessels, lymph, lymph nodes, and lymphocytes.

Lymph vessels branch into all the tissues of the body, carrying lymph, a clear fluid that contains white blood cells, especially B-cell and T-cell lymphocytes.

Lymph vessels are connected to lymph nodes which are small, round masses of tissue that store white blood cells. They also trap and remove bacteria or other harmful substances that may be in the lymph. Groups of lymph nodes are found in the neck, underarms, chest, abdomen, and groin.

Ninety percent (90%) of extraintestinal lymphomas are B-cell type and ten percent (10%) are T-cell type. In this malignancy, lymph nodes are replaced by cancer cells. Some are more aggressive than others.

In 2010, there were an estimated 509,065 people living with non-Hodgkin lymphoma in the United States according to the National Cancer Institute.

What Are Extraintestinal Lymphomas In Celiac Disease and/or Gluten Sensitivity?

Lymph Node: definition

Lymph nodes are part of the lymphatic system, acting to protect body fluids by filtering out and destroying bacteria and other harmful substances from lymph that is continually carried to them by lymph vessels.  Cleaned…