Diffuse Small-Bowel Myelomatosis
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《新英格兰医药杂志》
To the Editor: Extramedullary plasmacytomas are an unusual manifestation of multiple myeloma. Gastrointestinal plasmacytomas occur as discrete masses, usually involving either the stomach or colon, and can be mistaken for lymphoma.1,2,3 We report myeloma manifested as diffuse small-bowel myelomatosis.
A 74-year-old man presented with a one-year history of diffuse abdominal pain, diarrhea, and weight loss (16 kg [35 lb]). He did not have bone pain. Physical examination revealed no abnormalities except for cachexia; mild, diffuse abdominal distention without tenderness or a palpable mass; and stools that were positive for occult blood on guaiac testing. His blood count was normal except for a hematocrit of 28 percent. The serum creatinine level was 0.8 mg per deciliter. The total serum gamma globulin level was elevated, at 4.0 mg per deciliter, and serum immunofixation showed two IgG lambda bands. A 24-hour urine specimen contained 666 mg of protein, and urine immunofixation showed an IgG lambda band as well as a free lambda band. The results of a skeletal survey were normal. A marrow biopsy specimen contained 18 percent plasma cells. Both a computed tomographic scan of the abdomen and a small-bowel series showed small-bowel dilatation, with no mucosal thickening or adenopathy. The findings on upper endoscopy with biopsy of the stomach, esophagus, and duodenum were unremarkable. Colonoscopy revealed a thickened terminal ileum, but examination of a biopsy specimen showed only chronic inflammation.
Small-bowel obstruction developed, and the patient was taken to surgery. Much of the ileum appeared friable. A 50-cm segment of ileum and an adjacent 4-cm segment of cecum were resected. The entire wall of more than 40 cm of ileum showed a dense infiltrate of atypical plasma cells (Figure 1A). The adjacent colon was unremarkable. Immunohistochemical analysis showed that the cells stained strongly for CD138 (Figure 1B) and stained strongly for lambda light chain (Figure 1C). Staining for kappa light chain was negative, a finding indicative of light-chain restriction. Staining for CD20 was negative, a result that ruled out B-cell lymphoma. Staining for amyloid was negative. The patient had a complicated postoperative course and ultimately died of pneumonia and respiratory failure.
Figure 1. Specimen of the Patient's Resected Small Bowel.
The entire wall of ileum is infiltrated by a dense collection of plasma cells, many with atypical cytologic features (Panel A; hematoxylin and eosin, x400). The cellular infiltrate stained strongly for CD138, an antigenic marker for plasma cells (Panel B; murine monoclonal antibody and immunohistochemical reagents, x400). The cellular infiltrate also stained strongly for lambda light chain (Panel C; anti-human lambda polyclonal antibody, x400). Staining for kappa light chain and the B-cell CD20 antigen was negative.
Gastrointestinal involvement in myeloma is rare and occurs in less than 5 percent of cases of extramedullary plasmacytoma.4 It must be distinguished from lymphoma, amyloidosis, and immunoproliferative small-intestinal (alpha-chain) disease. Multiple myeloma should now be considered in the differential diagnosis of entities causing diffuse small-bowel disease associated with weight loss, diarrhea, and malabsorption.
Rebecca Weintraub, B.A.
Stanford University School of Medicine
Stanford, CA 94305
Sharmila Pramanik, M.D.
Santa Clara Valley Medical Center
San Jose, CA 95128
Lee Levitt, M.D.
Stanford University School of Medicine
Stanford, CA 94305
lee.levitt@hhs.co.santa-clara.ca.us
References
Griffiths AP, Shepherd NA, Beddall A, Williams JG. Gastrointestinal tumour masses due to multiple myeloma: a pathological mimic of malignant lymphoma. Histopathology 1997;31:318-323
Rao K, Yaghmai I. Plasmacytoma of the large bowel: a review of the literature and a case report of multiple myeloma involving the rectosigmoid. Gastrointest Radiol 1978;3:225-228
Morita T, Tamura S, Yokoyama Y, et al. A case of early-stage gastric plasmacytoma. J Gastroenterol 2002;37:398-401.
Alexiou C, Kau RJ, Dietzfelbinger H, et al. Extramedullary plasmacytoma: tumor occurrence and therapeutic concepts. Cancer 1999;85:2305-2314.
A 74-year-old man presented with a one-year history of diffuse abdominal pain, diarrhea, and weight loss (16 kg [35 lb]). He did not have bone pain. Physical examination revealed no abnormalities except for cachexia; mild, diffuse abdominal distention without tenderness or a palpable mass; and stools that were positive for occult blood on guaiac testing. His blood count was normal except for a hematocrit of 28 percent. The serum creatinine level was 0.8 mg per deciliter. The total serum gamma globulin level was elevated, at 4.0 mg per deciliter, and serum immunofixation showed two IgG lambda bands. A 24-hour urine specimen contained 666 mg of protein, and urine immunofixation showed an IgG lambda band as well as a free lambda band. The results of a skeletal survey were normal. A marrow biopsy specimen contained 18 percent plasma cells. Both a computed tomographic scan of the abdomen and a small-bowel series showed small-bowel dilatation, with no mucosal thickening or adenopathy. The findings on upper endoscopy with biopsy of the stomach, esophagus, and duodenum were unremarkable. Colonoscopy revealed a thickened terminal ileum, but examination of a biopsy specimen showed only chronic inflammation.
Small-bowel obstruction developed, and the patient was taken to surgery. Much of the ileum appeared friable. A 50-cm segment of ileum and an adjacent 4-cm segment of cecum were resected. The entire wall of more than 40 cm of ileum showed a dense infiltrate of atypical plasma cells (Figure 1A). The adjacent colon was unremarkable. Immunohistochemical analysis showed that the cells stained strongly for CD138 (Figure 1B) and stained strongly for lambda light chain (Figure 1C). Staining for kappa light chain was negative, a finding indicative of light-chain restriction. Staining for CD20 was negative, a result that ruled out B-cell lymphoma. Staining for amyloid was negative. The patient had a complicated postoperative course and ultimately died of pneumonia and respiratory failure.
Figure 1. Specimen of the Patient's Resected Small Bowel.
The entire wall of ileum is infiltrated by a dense collection of plasma cells, many with atypical cytologic features (Panel A; hematoxylin and eosin, x400). The cellular infiltrate stained strongly for CD138, an antigenic marker for plasma cells (Panel B; murine monoclonal antibody and immunohistochemical reagents, x400). The cellular infiltrate also stained strongly for lambda light chain (Panel C; anti-human lambda polyclonal antibody, x400). Staining for kappa light chain and the B-cell CD20 antigen was negative.
Gastrointestinal involvement in myeloma is rare and occurs in less than 5 percent of cases of extramedullary plasmacytoma.4 It must be distinguished from lymphoma, amyloidosis, and immunoproliferative small-intestinal (alpha-chain) disease. Multiple myeloma should now be considered in the differential diagnosis of entities causing diffuse small-bowel disease associated with weight loss, diarrhea, and malabsorption.
Rebecca Weintraub, B.A.
Stanford University School of Medicine
Stanford, CA 94305
Sharmila Pramanik, M.D.
Santa Clara Valley Medical Center
San Jose, CA 95128
Lee Levitt, M.D.
Stanford University School of Medicine
Stanford, CA 94305
lee.levitt@hhs.co.santa-clara.ca.us
References
Griffiths AP, Shepherd NA, Beddall A, Williams JG. Gastrointestinal tumour masses due to multiple myeloma: a pathological mimic of malignant lymphoma. Histopathology 1997;31:318-323
Rao K, Yaghmai I. Plasmacytoma of the large bowel: a review of the literature and a case report of multiple myeloma involving the rectosigmoid. Gastrointest Radiol 1978;3:225-228
Morita T, Tamura S, Yokoyama Y, et al. A case of early-stage gastric plasmacytoma. J Gastroenterol 2002;37:398-401.
Alexiou C, Kau RJ, Dietzfelbinger H, et al. Extramedullary plasmacytoma: tumor occurrence and therapeutic concepts. Cancer 1999;85:2305-2314.