Diffuse nodular lymphoid hyperplasia (DNLH) is a benign rare condition of unknown etiology characterized microscopically by diffuse hyperplasia of the lymphoid follicles of the gastrointestinal tract (GIT). It is grossly seen during endoscopy as numerous visible mucosal nodules measuring <0.5 cm in diameter. It can involve any part of the GIT, mainly the small intestine, but it may also involve the colon and rarely the stomach. It may have diffuse pattern which is the most common former focal pattern which is much less common. The disease is usually associated with immunodeficiency syndromes such as common variable immunodeficiency or selective IgA deficiency syndrome. Its prognosis is usually benign but it carries the risk of malignant transformation characteristically to lymphoma.

1. Shaimaa Elkholy, M.D.
Cairo University, Egypt
Shaimaa Elkholy, M.D. Cairo University

2. :
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. :
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. :
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. :
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. :
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. :
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. :
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. 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. 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. :
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. :
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. :
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. .
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. .
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. .
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.
Shaimaa Elkholy, M.D. Cairo University

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

18. .
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. .
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. .
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. .
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