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Nodular lymphoid hyperplasia

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Presentation on theme: "Nodular lymphoid hyperplasia"— Presentation transcript:

1 Nodular lymphoid hyperplasia
Shaimaa Elkholy, M.D. Cairo University, Egypt Shaimaa Elkholy, M.D. Cairo University

2 Diffuse Nodular lymphoid hyperplasia:
Definition: Its diffuse hyperplasia of the lymphoid follicles that is grossly characterized by the presence of numerous visible mucosal nodules measuring up to, and rarely exceeding 0.5 cm in diameter. Shaimaa Elkholy, M.D. Cairo University

3 Diffuse Nodular lymphoid hyperplasia:
Incidence : It is a benign rare condition . It may involve the small intestine ,colon or both. It may be diffuse pattern or focal form involving the terminal ileum or rectum . It very rarely involves the stomach. Shaimaa Elkholy, M.D. Cairo University

4 Diffuse Nodular lymphoid hyperplasia:
Etiology: Its of un known cause. Its lymph proliferative disease that is characterized by a stimulation of the B cell compartment that results in abnormal proliferation of secondary follicles and occurs principally in the cortex without broaching the lymph node capsule. Shaimaa Elkholy, M.D. Cairo University

5 Diffuse Nodular lymphoid hyperplasia:
Etiology: There is a theory of the local immune response to the antigens as a stimulators in GI tract, but still no antigen is defined . Another theory that it may be related to plasma cell precursors due to a maturational defect in the development of B-lymphocytes in order to compensate for functionally inadequate GI tissue. Shaimaa Elkholy, M.D. Cairo University

6 Diffuse Nodular lymphoid hyperplasia:
Histologically : there is enlargement of the mucosal B cell follicles. These hyperplastic follicles are confined to the mucosa and surrounded by a normal appearing mantle zone. The follicles are cytologically polymorphous, are often polarized, and vary in size and shape. Shaimaa Elkholy, M.D. Cairo University

7 Diffuse Nodular lymphoid hyperplasia:
Types : Children type: It is a very benign condition that regresses spontaneously. Its reported to be associated with delayed type of food hypersensitivity. Shaimaa Elkholy, M.D. Cairo University

8 Diffuse Nodular lymphoid hyperplasia:
Types : Adult type: Its usually associated with immunodeficiency : Selective IgA deficiency syndrome. 20% of patients wz Common variable immunodeficiency (IgG+IgA +_IgM). Giardia lambila. The risk of malignancy in patient with coexisting hypogammaglobulinemia, especially the risk of lymphoma and gastric carcinoma . Shaimaa Elkholy, M.D. Cairo University

9 Diffuse Nodular lymphoid hyperplasia:
RELATION TO H.PYLORI: Khuro et al, performed a cohort study from 2005 till 2010 on 40 patients with doudenal DLNH infected with H.pylori. After total eradication of H. pylori infection in 26 patients. Follow up duodenoscopies in these patients showed significant reduction of duodenal nodular lesions. 14 patients with resistant H. pylori infection showed no significant reduction of nodular lesions score. The study postulated that DDNLH in our patients was etiologically related to H. pylori infection. Shaimaa Elkholy, M.D. Cairo University

10 Diffuse Nodular lymphoid hyperplasia:
RELATION TO H.PYLORI: We believe the duodenal lesions were as a result of immune stimulation of prolonged and heavy H. pylori infection . This was supported by elevated immunoglobulins in a number of patients in our series. There are no other published reports of association of NLH with Helicobacter pylori (H. pylori) infection. Shaimaa Elkholy, M.D. Cairo University

11 Diffuse Nodular lymphoid hyperplasia:
Manifestations : Usually it causes no symptoms. The frequent gastrointestinal symptoms described by patients usually result from underlying conditions like malabsorption syndrome. coexisting diseases like immunodeficiences e.g. Repeated infections. Full blown picture of malignancy. Very rare causes bleeding or intestinal obstruction. Shaimaa Elkholy, M.D. Cairo University

12 Diffuse Nodular lymphoid hyperplasia:
Differential diagnosis: Nodular lymphoid hyperplasia always requires precise differential diagnosis from the other polyposis conditions malignant lymphoma. familial adenomatous polyposis. multiple lymphomatous polyposis. juvenile or hamartomatous polyposis. Puetz juegr syndrome. Shaimaa Elkholy, M.D. Cairo University

13 Diffuse Nodular lymphoid hyperplasia:
Treatment options : DLNH in cases with no complications does not require any special treatment, however, the patients should undergo the prophylactic examinations. This causes controversy when deciding the treatment options. Following patients without any treatment may lead to malignant progression, while surgical treatment may result in unnecessary radical resections because of obscurity in the diagnosis. Shaimaa Elkholy, M.D. Cairo University

14 DNLH with selective IgA deficiency syndrome (SIgAD).
SIgAD is the most common primary immunoglobulin deficiency. SIgAD defined as the isolated deficiency of serum IgA (ie, in the setting of normal serum levels of IgG and IgM) in a patient older than four years of age, in whom other causes of hypogammaglobulinemia have been excluded. Shaimaa Elkholy, M.D. Cairo University

15 DNLH with selective IgA deficiency syndrome (SIgAD).
Severe deficiency/definitive diagnosis —with a serum IgA < 7 mg/dL the lower limit of detection for most assays. Partial deficiency/probable diagnosis — with a serum IgA > 7 mg/dL but below the lower limit of normal (defined as 2 standard deviations below the age-adjusted mean value). serum levels of IgG and IgM must be normal. Shaimaa Elkholy, M.D. Cairo University

16 DNLH with selective IgA deficiency syndrome (SIgAD).
Clinical manifestations: 85-90% of the patients are asymptomatic. % of the patients have symptoms: Recurrent sinopulmonary infections Autoimmune disorders Gastrointestinal infections Anaphylactic transfusion reactions Food allergies and respiratory allergies are prominent. Shaimaa Elkholy, M.D. Cairo University

17 DNLH with selective IgA deficiency syndrome (SIgAD).
SIgAD & GI disorders: Recurrent infections e.g. Giardia lambila Non infectious : Celiac disease (8%) IBD DNLH Shaimaa Elkholy, M.D. Cairo University

18 DNLH with selective IgA deficiency syndrome (SIgAD).
Case reports: Aymer et al ;2009 presented a rare case of selective IgA deficiency with diffuse NLH in patient presenting with manifestations of malabsorbtion. Shaimaa Elkholy, M.D. Cairo University

19 DNLH with selective IgA deficiency syndrome (SIgAD).
Case reports: Mee joo etal ;2009 presented a rare case of selective IgA deficiency with diffuse NLH and various histologic changes in the small and large intestine. The histological features were very similar to those of celiac disease, collagenous sprue, and lymphocytic colitis. Shaimaa Elkholy, M.D. Cairo University

20 Shaimaa Elkholy, M.D. Cairo University

21 DNLH with selective IgA deficiency syndrome (SIgAD).
Piaścik etal ;2007 reported a case of diffuse nodular lymphoid hyperplasia of the GI tract in a patient with sarcoid-like syndrome as a rare manifestation of selective IgA deficiency. Shaimaa Elkholy, M.D. Cairo University

22 DNLH with selective IgA deficiency syndrome (SIgAD).
Ersoy etal ;2008 reported a case of DNLH of the colon presenting with bleeding per rectum not associated with immunodeficiency. Shaimaa Elkholy, M.D. Cairo University

23 Shaimaa Elkholy, M.D. Cairo University


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