Diffuse Nodular Lymphoid Hyperplasia (DNLH)

Shaimaa Elkholy, M.D.

Cairo University, EgyptShaimaa Elkholy, M.D. Cairo University

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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

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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.


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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.


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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.


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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.


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Types :Children type:

It is a very benign condition that regresses spontaneously.

Its reported to be associated with delayed

type of food hypersensitivity.


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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 .


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.

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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.

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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.


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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.


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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.


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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.


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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.


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Clinical manifestations:85-90% of the patients are asymptomatic.10 -15 % of the patients have symptoms:•Recurrent sinopulmonary infections•Autoimmune disorders •Gastrointestinal infections •Anaphylactic transfusion reactions• Food allergies and respiratory allergies are prominent.


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SIgAD & GI disorders:

•Recurrent infections e.g. Giardia lambila•Non infectious :Celiac disease (8%)IBDDNLH


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Case reports:Aymer et al ;2009 presented a rare case of selective IgA deficiency with diffuse NLH in patient presenting with manifestations of malabsorbtion.


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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.



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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.


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Ersoy etal ;2008 reported a case of DNLH of the colon presenting with bleeding per rectum not associated with immunodeficiency.



Health & Medicine

Diffuse Nodular Lymphoid Hyperplasia (DNLH)