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Mesenteric Lymph Node Cavitation And Hyposplenism

Mesenteric Lymph Node Cavitation. Courtesy
Mesenteric Lymph Node Cavitation.  Courtesy McBride OM, Skipworth RJ, Leitch D, Yalamarthi S.

What Is Mesenteric Lymph Node Cavitation And Hyposplenism?

[dropcap]M[/dropcap]esenteric lymph node cavitation and hyposplenism combination is a rare lymphatic entity. It is characterized by involution (degeneration) of a mesenteric lymph node (abdominal) and absence of functional spleen tissue.

Q: What is happening to the lymph nodes?

A: Lymph nodes are enlarged with central, partly cystic degeneration. When cut open either at surgery or autopsy, milky fluid exudes from the cut surface.

In regards to absent spleen function, the body is highly susceptible to bacterial invasion such as pneumonia because tissues of the spleen, called the pulp, produce specialized white blood cells that protect the body against bacterial invasion and trap foreign antigens.

What Is Mesenteric Lymph Node Cavitation And Hyposplenism 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. []

Vitiligo

What Is Vitiligo? [dropcap]V[/dropcap]itiligo is a pigmentation disorder of the skin characterized by permanent loss of melanocytes in defined areas and, in some patients, antibodies to melanin. Vitiligo has significant psychological impact if occurring before adulthood.1… 

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?

Lymphadenopathy

Lymphadenopathy.
Lymphadenopathy affecting a node in the neck.

What Is Lymphadenopathy?

[dropcap]L[/dropcap]ymphadenopathy is an alteration of lymph nodes that is characterized by enlargement of lymph nodes greater than 1.5 cm caused by proliferation (increased production) of lymphocytes within the node.

Q: What are lymph nodes?

A: 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 lymph is carried away from the nodes by lymph vessels to the bloodsteam where it helps forms blood plasma. Lymph nodes produce various blood cells needed to fight infection which includes lymphocytes.

Lymphocytes are small white blood cells that plays a major role in defending the body against disease. There are two types of lynphocytes: B cells, which make antibodies that attack bacteria and toxins, and T cells which attack body cells themselves when they have been taken over by viruses or become cancerous.

Lymph nodes that become enlarged doing battle with an infection or as a result of injury nearby usually resolve with treatment of the infection or injury. However, if the cause is cancer, the nodes would need to be treated as well as the cancer.

What Is Lymphadenopathy In Celiac Disease and/or Gluten Sensitivity?