Nodular lymphoid hyperplasia: rare case of lymphoproliferative disease in the lung

Nodular lymphoid hyperplasia (NLH) is a lymphoproliferative disease caused by non-clonal expansion of lymphoid cells in the gut mucosa. Little is known about the pathogenesis of NLH, which is often disregarded as an insignificant or para-physiologic phenomenon. We present the case of a girl with isolated diffuse NLH (extending from the stomach to the rectum) caused by activated PI3Kδ syndrome (APDS) due to the novel p.Glu525Gly variant in PIK3CD. The gain-of-function effect of the variant was confirmed by demonstration of over activation of the Akt/mTOR pathway in the patient's cells. APDS diagnosis led to treatment with sirolimus, which resulted in the complete remission of NLH and in the prevention of extra intestinal complications. In conclusion, we identify APDS as a novel cause of isolated NLH and suggest that patients with severe pan-enteric NLH should be screened for this disorder that may not be apparent on first-line immunological testing.

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Diffuse nodular lymphoid hyperplasia of the small bowel associated with common variable immunodeficiency and giardiasis: a rare case report

Wiener klinische Wochenschrift ◽

10.1007/s00508-014-0525-5 ◽

2014 ◽

Vol 126 (9-10) ◽

pp. 294-297 ◽

Cited By ~ 7

Author(s):

Sehmus Olmez ◽

Mehmet Aslan ◽

Alpaslan Yavuz ◽

Gulay Bulut ◽

Ahmet Cumhur Dulger

Keyword(s):

Case Report ◽

Small Bowel ◽

Common Variable Immunodeficiency ◽

Rare Case ◽

Lymphoid Hyperplasia ◽

Nodular Lymphoid Hyperplasia ◽

Rare Case Report ◽

Common Variable

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Hypogammaglobulinemia with nodular lymphoid hyperplasia of the small bowel

Archives of Internal Medicine ◽

10.1001/archinte.121.3.273 ◽

1968 ◽

Vol 121 (3) ◽

pp. 273-277 ◽

Cited By ~ 1

Author(s):

C. H. Kirkpatrick

Keyword(s):

Small Bowel ◽

Lymphoid Hyperplasia ◽

Nodular Lymphoid Hyperplasia

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Nodular lymphoid hyperplasia of the small intestine and sarcoidosis

Archives of Internal Medicine ◽

10.1001/archinte.126.4.668 ◽

1970 ◽

Vol 126 (4) ◽

pp. 668-672 ◽

Cited By ~ 5

Author(s):

S. D. Davis

Keyword(s):

Small Intestine ◽

Lymphoid Hyperplasia ◽

Nodular Lymphoid Hyperplasia

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COMMON VARIABLE IMMUNODEFICIENCY DISORDER DIAGNOSED TROUGH GASTROSCOPY REVEALING NODULAR LYMPHOID HYPERPLASIA PRIMARY ASSESSED AS FAMILIAL ADENOMATOUS POLYPOSIS

10.26226/morressier.57c5383ad462b80296c9c853 ◽

2016 ◽

Author(s):

Anna Maria Pietrzak

Keyword(s):

Familial Adenomatous Polyposis ◽

Common Variable Immunodeficiency ◽

Lymphoid Hyperplasia ◽

Adenomatous Polyposis ◽

Nodular Lymphoid Hyperplasia ◽

Immunodeficiency Disorder ◽

Common Variable

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RARE CASE OF NODULAR LYMPHOID HYPERPLASIA OF LEFT LUNG IN THE PATIENT WITH PREVIOUS PULMONARY TUBERCULOSIS

Experimental Oncology ◽

10.31768/2312-8852.2018.40(4):332-335 ◽

2018 ◽

Vol 40 (4) ◽

pp. 332-335

Author(s):

P V Kuzyk ◽

M A Savchyna ◽

S G Gychka

Keyword(s):

Pulmonary Tuberculosis ◽

Lymphoproliferative Disorder ◽

Clinical Laboratory ◽

Left Lung ◽

Lymphoid Hyperplasia ◽

Morphological Examination ◽

Nodular Lymphoid Hyperplasia ◽

Lymph Nodes Dissection ◽

The Right ◽

Immunohistochemical Studies

Aim: To describe the case of rare benign lymphoproliferative disorder — pulmonary nodular lymphoid hyperplasia in the patient with previous pulmonary tuberculosis. Materials and Methods: In the case of pulmonary nodular lymphoid hyperplasia clinical, laboratory, instrumental and morphological examination was performed. Results: 44-year-old woman in 7 years after successfully treated infiltrative drug-susceptible tuberculosis of the right lung, was hospitalized with a suspected tumor of the left lung root. The patient underwent left-sided pneumonectomy with lymph nodes dissection. The results of histopathological and immunohistochemical studies evidenced on nodular lymphoid hyperplasia of the left lung. Conclusion: Pulmonary nodular lymphoid hyperplasia is a rare lymphoproliferative disorder of the lung with favorable prognosis. For the purpose of differential diagnosis, it is necessary to apply immunohistochemistry.

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