Unusual presentation of more common disease/injury
CASE REPORT
Jejunal perforation: a rare presentation of B-cell lymphoma V Santharam,1 P Kumar,2 L Y W Lee3 1
Department of General Surgery, Milton Keynes General Hospital, Milton Keynes, Bucks, UK 2 Milton Keynes General Hospital, Keynes, Bucks, UK 3 Guy’s & St Thomas’s NHS Foundation Trust, London, UK Correspondence to Dr V Santharam, vidya_santharam@hotmail. co.uk Accepted 3 February 2014
SUMMARY A 42-year-old man who was being treated for pneumonia developed severe, sudden-onset abdominal pain with features of shock and peritonism. The clinical picture combined with radiological investigations raised suspicion of a bowel perforation necessitating urgent surgical review and emergency laparotomy. This diagnosed a jejunal perforation with abnormal lymph nodes. Histological examination confirmed diffuse large B-cell lymphoma. The patient was subsequently started on a course of chemotherapy. While gastrointestinal perforation secondary to antilymphoma treatment is a well-recognised complication, primary perforation caused by the lymphoma itself must always be considered.
BACKGROUND Gastrointestinal (GI) perforation is well documented in patients who have lymphoma and are being treated with either chemotherapy and/or steroids.1 Furthermore, the small intestine is a common site of perforation in patients with diffuse large B-cell lymphoma.2 We present a case where the presenting feature of a diffuse large B-cell lymphoma was a jejunal perforation in a patient who had no history of lymphoma.
CASE PRESENTATION
To cite: Santharam V, Kumar P, Lee LYW. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013203052
A 40-year-old man presented with a 2-week history of night sweats, fever and cough on a background of general malaise. His medical history included tuberculosis (TB) and sarcoidosis. The patient’s vital signs on admission were as follows: temperature 38.8°C, heart rate 117 bpm, respiratory rate 22 breaths/minute, blood pressure 111/73 mm Hg and oxygen saturations of 97% on room air. Clinical examination demonstrated reduced air entry and coarse crackles at the right base. He also had some epigastric tenderness to palpation, although his abdomen was soft. Laboratory investigations demonstrated a white cell count 4.9×109/L, C reactive protein, 42 mg/L and haemoglobin, 10.2 g/dL. Based on this presentation, he was initially treated for a lower respiratory tract infection, however, 6 h postadmission the patient became hypotensive, tachycardic and tachypnoeic. He had also developed severe, constant abdominal pain. On examination, he displayed signs of peritonism. An erect chest X-ray demonstrated free air under the diaphragm. An urgent CT scan was performed and this confirmed intestinal perforation. An emergency laparotomy was performed and a 3 mm jejunal perforation was noted with enlarged mesenteric lymph nodes, which were not macroscopically
Santharam V, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203052
typical of TB. Samples of resected jejunal tissue were subsequently sent for histology.
INVESTIGATIONS Erect chest X-ray demonstrated free air under the right hemidiaphragm, prompting a suspicion of GI tract perforation (figure 1). CT of the abdomen/pelvis with contrast showed a small bowel perforation, hepatosplenomegaly with the spleen measuring about 19 cm, and the presence of abnormal enlarged lymph nodes within the abdomen. More specifically, abnormal lymph nodes were seen in the aortocaval group and in the small bowel mesentery. Some enlarged inguinal lymph nodes and bilaterally enlarged iliac lymph nodes were also seen. These findings are in keeping with a suspicion of lymphoma (figure 2). Histology of the resected jejunal tissue confirmed diffuse large B-cell lymphoma (figure 3).
TREATMENT Resection of the upper jejunum and reanastomosis was performed.
OUTCOME AND FOLLOW-UP Postlaparotomy, the patient was initially transferred to the intensive care unit for monitoring and made a steady recovery over the next 2 weeks. Following a histological diagnosis of diffuse large B-cell lymphoma, a six-cycle regimen of R-CHOP combination chemotherapy (rituximab,
Figure 1 Erect chest X-ray postero-anterior film, showing small amount of free gas under the right hemidiaphragm (indicated by red arrow), suggestive of perforation. 1
Unusual presentation of more common disease/injury retrospective review of all GI lymphoma patients over a 37-year period found that 9% of patients developed a perforation, of which 55% occurred after chemotherapy.1 Furthermore, the lymphoma most frequently associated with perforation was of the diffuse large B-cell variety.1 Tumour necrosis with perforation is the potential complication of chemotherapy treatment of GI lymphoma.7 Diagnosis of GI perforation is often delayed because the traditional symptoms and signs of an inflammatory process may be diminished by the anti-inflammatory effects of steroids, resulting in peritonitis going unrecognised until the development of shock.8 Bowel perforation as a consequence of antilymphoma treatment is well documented. However, this case demonstrates that bowel perforation can be a primary complication of GI lymphoma itself; or indeed, the presenting feature of an undiagnosed case of GI lymphoma. Figure 2 CT of the abdomen/pelvis, axial view, showing free air under the right hemidiaphragm and around the liver.
Learning points ▸ The presenting symptoms of lymphoma, such as fever, malaise and night sweats, are often very similar to the symptoms of septicaemia. ▸ In patients with gastrointestinal (GI) perforation, upper GI malignancies are an important differential and histology should be obtained. ▸ The most common lymphoma associated with perforation is B-cell lymphoma and clinicians should be aware that perforation may indeed be the presenting feature.
cyclophosphamide, doxorubicin, vincristine and prednisolone) was instigated.
DISCUSSION Non-Hodgkin’s lymphoma (NHL) is a condition associated with sarcoid disease3 that frequently occurs in lymph nodes of the chest, abdomen, neck, tonsils and skin. Although less common, it can also develop at other sites, such as the GI tract and the central nervous system. Meanwhile extranodal NHL is most commonly found to be a primary gastric lymphoma.4 Approximately 5% of peripheral NHL’s are primarily located in the intestine.5 Five to 10% of all GI tumours are comprised of primary GI lymphomas.6 An American single centre
Contributors VS and PK cowrote the article and LYWL reviewed it. All three authors were involved in the patient’s care. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2 3 4
5 6
7
Figure 3 CT of the abdomen/pelvis, coronal view, showing a thickened abnormal loop of small bowel with stranding and free gas in the surrounding fat. Hepatosplenomegaly is also demonstrated.
2
8
Vaidya R, Habermann TM, Donohue JH, et al. Bowel perforation in intestinal lymphoma: incidence and clinical features. Ann Oncol 2013;24:2439–43. Cengiz A, Sacid C, Cuneyt K, et al. Spontaneous intestinal perforation due to non-Hodgkin’s lymphoma: evaluation of eight cases. Dig Dis Sci 2007;52:1752–6. Papanikolaou IC, Sharma OP. The relationship between sarcoidosis and lymphoma. Eur Respir J 2010;36:1207–9. Maisey N, Norman A, Prior Y, et al. Chemotherapy for primary gastric lymphoma: does in-patient observation prevent complications? Clin Oncol (R Coll Radiol) 2004;16:48–52. Domizio P, Owen R, Shepherd N, et al. Primary lymphoma of the small intestine: a clinicopathological study of 119 cases. Am J Surg Pathol 1993;17:429–42. Daum S, Ullrish R, Heise W, et al. Intestinal non-Hodgkin’s lymphoma: a multicenter prospective clinical study from the German Study Group on intestinal non-Hodgkin’s lymphoma. J Clin Oncol2003;21:2740–6. Meyers PA, Potter VP, Wollner N, et al. Bowel perforation during initial treatment for childhood non-Hodgkin’s lymphoma. Cancer 1985;56:259–61. Wada M, Onda M, Tokunaga A, et al. Spontaneous gastrointestinal perforation in patients with lymphoma receiving chemotherapy and steroids. Report of three cases. Nihon Ika Daigaku Zasshi 1999;66:37–40.
Santharam V, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203052
Unusual presentation of more common disease/injury
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Santharam V, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203052
3
CASE REPORT
Jejunal perforation: a rare presentation of B-cell lymphoma V Santharam,1 P Kumar,2 L Y W Lee3 1
Department of General Surgery, Milton Keynes General Hospital, Milton Keynes, Bucks, UK 2 Milton Keynes General Hospital, Keynes, Bucks, UK 3 Guy’s & St Thomas’s NHS Foundation Trust, London, UK Correspondence to Dr V Santharam, vidya_santharam@hotmail. co.uk Accepted 3 February 2014
SUMMARY A 42-year-old man who was being treated for pneumonia developed severe, sudden-onset abdominal pain with features of shock and peritonism. The clinical picture combined with radiological investigations raised suspicion of a bowel perforation necessitating urgent surgical review and emergency laparotomy. This diagnosed a jejunal perforation with abnormal lymph nodes. Histological examination confirmed diffuse large B-cell lymphoma. The patient was subsequently started on a course of chemotherapy. While gastrointestinal perforation secondary to antilymphoma treatment is a well-recognised complication, primary perforation caused by the lymphoma itself must always be considered.
BACKGROUND Gastrointestinal (GI) perforation is well documented in patients who have lymphoma and are being treated with either chemotherapy and/or steroids.1 Furthermore, the small intestine is a common site of perforation in patients with diffuse large B-cell lymphoma.2 We present a case where the presenting feature of a diffuse large B-cell lymphoma was a jejunal perforation in a patient who had no history of lymphoma.
CASE PRESENTATION
To cite: Santharam V, Kumar P, Lee LYW. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013203052
A 40-year-old man presented with a 2-week history of night sweats, fever and cough on a background of general malaise. His medical history included tuberculosis (TB) and sarcoidosis. The patient’s vital signs on admission were as follows: temperature 38.8°C, heart rate 117 bpm, respiratory rate 22 breaths/minute, blood pressure 111/73 mm Hg and oxygen saturations of 97% on room air. Clinical examination demonstrated reduced air entry and coarse crackles at the right base. He also had some epigastric tenderness to palpation, although his abdomen was soft. Laboratory investigations demonstrated a white cell count 4.9×109/L, C reactive protein, 42 mg/L and haemoglobin, 10.2 g/dL. Based on this presentation, he was initially treated for a lower respiratory tract infection, however, 6 h postadmission the patient became hypotensive, tachycardic and tachypnoeic. He had also developed severe, constant abdominal pain. On examination, he displayed signs of peritonism. An erect chest X-ray demonstrated free air under the diaphragm. An urgent CT scan was performed and this confirmed intestinal perforation. An emergency laparotomy was performed and a 3 mm jejunal perforation was noted with enlarged mesenteric lymph nodes, which were not macroscopically
Santharam V, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203052
typical of TB. Samples of resected jejunal tissue were subsequently sent for histology.
INVESTIGATIONS Erect chest X-ray demonstrated free air under the right hemidiaphragm, prompting a suspicion of GI tract perforation (figure 1). CT of the abdomen/pelvis with contrast showed a small bowel perforation, hepatosplenomegaly with the spleen measuring about 19 cm, and the presence of abnormal enlarged lymph nodes within the abdomen. More specifically, abnormal lymph nodes were seen in the aortocaval group and in the small bowel mesentery. Some enlarged inguinal lymph nodes and bilaterally enlarged iliac lymph nodes were also seen. These findings are in keeping with a suspicion of lymphoma (figure 2). Histology of the resected jejunal tissue confirmed diffuse large B-cell lymphoma (figure 3).
TREATMENT Resection of the upper jejunum and reanastomosis was performed.
OUTCOME AND FOLLOW-UP Postlaparotomy, the patient was initially transferred to the intensive care unit for monitoring and made a steady recovery over the next 2 weeks. Following a histological diagnosis of diffuse large B-cell lymphoma, a six-cycle regimen of R-CHOP combination chemotherapy (rituximab,
Figure 1 Erect chest X-ray postero-anterior film, showing small amount of free gas under the right hemidiaphragm (indicated by red arrow), suggestive of perforation. 1
Unusual presentation of more common disease/injury retrospective review of all GI lymphoma patients over a 37-year period found that 9% of patients developed a perforation, of which 55% occurred after chemotherapy.1 Furthermore, the lymphoma most frequently associated with perforation was of the diffuse large B-cell variety.1 Tumour necrosis with perforation is the potential complication of chemotherapy treatment of GI lymphoma.7 Diagnosis of GI perforation is often delayed because the traditional symptoms and signs of an inflammatory process may be diminished by the anti-inflammatory effects of steroids, resulting in peritonitis going unrecognised until the development of shock.8 Bowel perforation as a consequence of antilymphoma treatment is well documented. However, this case demonstrates that bowel perforation can be a primary complication of GI lymphoma itself; or indeed, the presenting feature of an undiagnosed case of GI lymphoma. Figure 2 CT of the abdomen/pelvis, axial view, showing free air under the right hemidiaphragm and around the liver.
Learning points ▸ The presenting symptoms of lymphoma, such as fever, malaise and night sweats, are often very similar to the symptoms of septicaemia. ▸ In patients with gastrointestinal (GI) perforation, upper GI malignancies are an important differential and histology should be obtained. ▸ The most common lymphoma associated with perforation is B-cell lymphoma and clinicians should be aware that perforation may indeed be the presenting feature.
cyclophosphamide, doxorubicin, vincristine and prednisolone) was instigated.
DISCUSSION Non-Hodgkin’s lymphoma (NHL) is a condition associated with sarcoid disease3 that frequently occurs in lymph nodes of the chest, abdomen, neck, tonsils and skin. Although less common, it can also develop at other sites, such as the GI tract and the central nervous system. Meanwhile extranodal NHL is most commonly found to be a primary gastric lymphoma.4 Approximately 5% of peripheral NHL’s are primarily located in the intestine.5 Five to 10% of all GI tumours are comprised of primary GI lymphomas.6 An American single centre
Contributors VS and PK cowrote the article and LYWL reviewed it. All three authors were involved in the patient’s care. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2 3 4
5 6
7
Figure 3 CT of the abdomen/pelvis, coronal view, showing a thickened abnormal loop of small bowel with stranding and free gas in the surrounding fat. Hepatosplenomegaly is also demonstrated.
2
8
Vaidya R, Habermann TM, Donohue JH, et al. Bowel perforation in intestinal lymphoma: incidence and clinical features. Ann Oncol 2013;24:2439–43. Cengiz A, Sacid C, Cuneyt K, et al. Spontaneous intestinal perforation due to non-Hodgkin’s lymphoma: evaluation of eight cases. Dig Dis Sci 2007;52:1752–6. Papanikolaou IC, Sharma OP. The relationship between sarcoidosis and lymphoma. Eur Respir J 2010;36:1207–9. Maisey N, Norman A, Prior Y, et al. Chemotherapy for primary gastric lymphoma: does in-patient observation prevent complications? Clin Oncol (R Coll Radiol) 2004;16:48–52. Domizio P, Owen R, Shepherd N, et al. Primary lymphoma of the small intestine: a clinicopathological study of 119 cases. Am J Surg Pathol 1993;17:429–42. Daum S, Ullrish R, Heise W, et al. Intestinal non-Hodgkin’s lymphoma: a multicenter prospective clinical study from the German Study Group on intestinal non-Hodgkin’s lymphoma. J Clin Oncol2003;21:2740–6. Meyers PA, Potter VP, Wollner N, et al. Bowel perforation during initial treatment for childhood non-Hodgkin’s lymphoma. Cancer 1985;56:259–61. Wada M, Onda M, Tokunaga A, et al. Spontaneous gastrointestinal perforation in patients with lymphoma receiving chemotherapy and steroids. Report of three cases. Nihon Ika Daigaku Zasshi 1999;66:37–40.
Santharam V, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203052
Unusual presentation of more common disease/injury
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Santharam V, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-203052
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