case report Wien Klin Wochenschr (2014) 126:294–297 DOI 10.1007/s00508-014-0525-5
Diffuse nodular lymphoid hyperplasia of the small bowel associated with common variable immunodeficiency and giardiasis: a rare case report Sehmus Olmez · Mehmet Aslan · Alpaslan Yavuz · Gulay Bulut · Ahmet Cumhur Dulger
Received: 22 October 2013 / Accepted: 8 February 2014 / Published online: 20 March 2014 © Springer-Verlag Wien 2014
Summary Diffuse nodular lymphoid hyperplasia (DNLH) of the intestine is an extremely rare lymphoproliferative disorder of uncertain etiology. Typically, numerous polypoid nodules composed of hyperplastic benign lymphoid tissue are present in the small and/ or large intestinal mucosa. DNLH has been observed in association with common variable immunodeficiency (CVID). A 38-years-old man was admitted to our clinic due to dyspeptic complaints. An upper gastrointestinal system endoscopic examination revealed DNLH in the duodenum. A biopsy specimen showed the presence of nodular lymphoid hyperplasia and a Giardia lamblia infection in the duodenum. CVID was suspected, and the diagnosis was established by demonstrating a significant reduction in the serum gamma-globulin levels. DNLH is a rare benign condition with regards to diagnosis and treatment of unknown etiology. In patients with DNLH, screening for the immune deficiencies is being important in addition to histopathological examinations.
Dr. S. Olmez () · A. C. Dulger Department of Gastroenterology, Medical Faculty, Yuzuncu Yil Universitesi, 65000 Van, Turkey e-mail: [email protected] M. Aslan Department of Internal Medicine, Medical Faculty, Yuzuncu Yil Universitesi, Van, Turkey A. Yavuz Department of Radiology, Medical Faculty, Yuzuncu Yil Universitesi, Van, Turkey G. Bulut Department of Pathology, Medical Faculty, Yuzuncu Yil Universitesi, Van, Turkey
Keywords Diffuse nodular lymphoid hyperplasia · Common variable immunodeficiency · Giardia lamblia · Celiac disease
Diffuse noduläre lymphoide Hyperplasie des Dünndarms mit gewöhnlicher variabler Immundefizienz und Giardiasis: Fallbericht einer seltenen Erkrankung Zusammenfassung Die diffuse noduläre lymphoide Hyperplasie (DNLH) des Darms ist eine extrem seltene lymphoproliferative Erkrankung ungesicherter Ätiologie. Typisch für die Erkrankung sind zahlreiche polypoide Knoten in der Mukosa des Dünn- und/oder Dickdarms, die aus benignem hyperplastischem lymphoiden Gewebe bestehen. Es liegen Berichte über ein mit gewöhnlicher passagerer Immundefizienz (CVID) gemeinsames Auftreten vor. Ein 38-jähriger Patient wurde auf unserer Klinik wegen dyspeptischer Beschwerden aufgenommen. Eine Gastro-Duodenoskopie ergab das Vorliegen einer DNLH im Duodenum. Im bioptischen Material wurde eine noduläre lymphoide Hyperplasie, sowie eine Infektion mit Giardia lamblia festgestellt. Eine suspizierte CVID wurde durch den Nachweis einer signifikant verminderten Gammaglobulinämie bestätigt. Was die Diagnose und Therapie betrifft, ist die DNLH eine seltene benigne Erkrankung – allerdings unklarer Ätiologie. Bei Patienten mit DNLH sollte zusätzlich zur Histologie ein Screening auf Immundefizienz durchgeführt werden. Schlüsselwörter Diffuse noduläre lymphoide Hyperplasie · Gewöhnliche variable Immundefizienz · Giardia lamblia · Zöliakie
294 Diffuse nodular lymphoid hyperplasia of the small bowel associated with common variable immunodeficiency
13
case report Introduction Diffuse nodular lymphoid hyperplasia (DNLH) is an extremely rare lymphoproliferative disorder of uncertain etiology, which is characterized by the presence of multiple nodular lesions. Typically, numerous polypoid nodules composed of hyperplastic benign lymphoid tissue are present in the small and/or large intestinal mucosa [1, 2]. Mucosal polypoid lesions can range in size from 2 to 10 mm but rarely exceed 5 mm in diameter [1, 3]. DNLH can present as an asymptomatic disease or manifest with gastrointestinal symptoms, such as abdominal pain, chronic diarrhea, occult bleeding, and rarely intestinal obstruction [3]. DNLH has been suggested to be a risk factor for primary lymphoma of the small intestine [4]. It has been reported on a large cohort of patients with diffuse duodenal nodular lymphoid hyperplasia (DDNLH), etiologically related to Helicobacter pylori infection [1]. DNLH of the gastrointestinal tract is often associated with immunodeficiency syndromes. Approximately 20 % of adults with Common variable immunodeficiency (CVID) are found to have DNLH [1, 4, 5]. CVID is considered to be a collection of primary immune deficiency diseases characterized by reduced serum immunoglobulin (Ig)G, IgA, and/or IgM levels [3]. An association between hypogammaglobulinemia and DNLH has been reported by several investigators [1, 6]. However, to our knowledge, there are only limited case reports of DNLH in the duodenum in association with Giardia lamblia infection in immunocompromised patients [2]. In this case report, we describe a case of a nodular mucosal pattern in the duodenum concomitant Giardia lamblia infection in an immunocompromised patient.
Case report A 38-year-old man was admitted to the gastroenterological clinic with a 1-month history of dyspeptic complaints. He had no history of diarrhea or weight loss. In addition, his past medical history included celiac disease that was diagnosed 15 months ago. His family history was unremarkable, and his social history was negative for alcohol and smoking.
On admission, the patient’s blood pressure, body temperature, and pulse rate were 120/75 mmHg, 36.5 °C, and 92/min, respectively. On physical examination, he presented a medium general status. Physical examination revealed no significant features. The liver and spleen were not palpable. Laboratory studies on admission showed the following results: white blood cell count, 5.900/µl; hemoglobin, 14.7 g/dl; platelet count, 259,000/mm3; albumin, 4.37 g/ dl; AST, 25 U/L (normal
Diffuse nodular lymphoid hyperplasia of the small bowel associated with common variable immunodeficiency and giardiasis: a rare case report Sehmus Olmez · Mehmet Aslan · Alpaslan Yavuz · Gulay Bulut · Ahmet Cumhur Dulger
Received: 22 October 2013 / Accepted: 8 February 2014 / Published online: 20 March 2014 © Springer-Verlag Wien 2014
Summary Diffuse nodular lymphoid hyperplasia (DNLH) of the intestine is an extremely rare lymphoproliferative disorder of uncertain etiology. Typically, numerous polypoid nodules composed of hyperplastic benign lymphoid tissue are present in the small and/ or large intestinal mucosa. DNLH has been observed in association with common variable immunodeficiency (CVID). A 38-years-old man was admitted to our clinic due to dyspeptic complaints. An upper gastrointestinal system endoscopic examination revealed DNLH in the duodenum. A biopsy specimen showed the presence of nodular lymphoid hyperplasia and a Giardia lamblia infection in the duodenum. CVID was suspected, and the diagnosis was established by demonstrating a significant reduction in the serum gamma-globulin levels. DNLH is a rare benign condition with regards to diagnosis and treatment of unknown etiology. In patients with DNLH, screening for the immune deficiencies is being important in addition to histopathological examinations.
Dr. S. Olmez () · A. C. Dulger Department of Gastroenterology, Medical Faculty, Yuzuncu Yil Universitesi, 65000 Van, Turkey e-mail: [email protected] M. Aslan Department of Internal Medicine, Medical Faculty, Yuzuncu Yil Universitesi, Van, Turkey A. Yavuz Department of Radiology, Medical Faculty, Yuzuncu Yil Universitesi, Van, Turkey G. Bulut Department of Pathology, Medical Faculty, Yuzuncu Yil Universitesi, Van, Turkey
Keywords Diffuse nodular lymphoid hyperplasia · Common variable immunodeficiency · Giardia lamblia · Celiac disease
Diffuse noduläre lymphoide Hyperplasie des Dünndarms mit gewöhnlicher variabler Immundefizienz und Giardiasis: Fallbericht einer seltenen Erkrankung Zusammenfassung Die diffuse noduläre lymphoide Hyperplasie (DNLH) des Darms ist eine extrem seltene lymphoproliferative Erkrankung ungesicherter Ätiologie. Typisch für die Erkrankung sind zahlreiche polypoide Knoten in der Mukosa des Dünn- und/oder Dickdarms, die aus benignem hyperplastischem lymphoiden Gewebe bestehen. Es liegen Berichte über ein mit gewöhnlicher passagerer Immundefizienz (CVID) gemeinsames Auftreten vor. Ein 38-jähriger Patient wurde auf unserer Klinik wegen dyspeptischer Beschwerden aufgenommen. Eine Gastro-Duodenoskopie ergab das Vorliegen einer DNLH im Duodenum. Im bioptischen Material wurde eine noduläre lymphoide Hyperplasie, sowie eine Infektion mit Giardia lamblia festgestellt. Eine suspizierte CVID wurde durch den Nachweis einer signifikant verminderten Gammaglobulinämie bestätigt. Was die Diagnose und Therapie betrifft, ist die DNLH eine seltene benigne Erkrankung – allerdings unklarer Ätiologie. Bei Patienten mit DNLH sollte zusätzlich zur Histologie ein Screening auf Immundefizienz durchgeführt werden. Schlüsselwörter Diffuse noduläre lymphoide Hyperplasie · Gewöhnliche variable Immundefizienz · Giardia lamblia · Zöliakie
294 Diffuse nodular lymphoid hyperplasia of the small bowel associated with common variable immunodeficiency
13
case report Introduction Diffuse nodular lymphoid hyperplasia (DNLH) is an extremely rare lymphoproliferative disorder of uncertain etiology, which is characterized by the presence of multiple nodular lesions. Typically, numerous polypoid nodules composed of hyperplastic benign lymphoid tissue are present in the small and/or large intestinal mucosa [1, 2]. Mucosal polypoid lesions can range in size from 2 to 10 mm but rarely exceed 5 mm in diameter [1, 3]. DNLH can present as an asymptomatic disease or manifest with gastrointestinal symptoms, such as abdominal pain, chronic diarrhea, occult bleeding, and rarely intestinal obstruction [3]. DNLH has been suggested to be a risk factor for primary lymphoma of the small intestine [4]. It has been reported on a large cohort of patients with diffuse duodenal nodular lymphoid hyperplasia (DDNLH), etiologically related to Helicobacter pylori infection [1]. DNLH of the gastrointestinal tract is often associated with immunodeficiency syndromes. Approximately 20 % of adults with Common variable immunodeficiency (CVID) are found to have DNLH [1, 4, 5]. CVID is considered to be a collection of primary immune deficiency diseases characterized by reduced serum immunoglobulin (Ig)G, IgA, and/or IgM levels [3]. An association between hypogammaglobulinemia and DNLH has been reported by several investigators [1, 6]. However, to our knowledge, there are only limited case reports of DNLH in the duodenum in association with Giardia lamblia infection in immunocompromised patients [2]. In this case report, we describe a case of a nodular mucosal pattern in the duodenum concomitant Giardia lamblia infection in an immunocompromised patient.
Case report A 38-year-old man was admitted to the gastroenterological clinic with a 1-month history of dyspeptic complaints. He had no history of diarrhea or weight loss. In addition, his past medical history included celiac disease that was diagnosed 15 months ago. His family history was unremarkable, and his social history was negative for alcohol and smoking.
On admission, the patient’s blood pressure, body temperature, and pulse rate were 120/75 mmHg, 36.5 °C, and 92/min, respectively. On physical examination, he presented a medium general status. Physical examination revealed no significant features. The liver and spleen were not palpable. Laboratory studies on admission showed the following results: white blood cell count, 5.900/µl; hemoglobin, 14.7 g/dl; platelet count, 259,000/mm3; albumin, 4.37 g/ dl; AST, 25 U/L (normal
