Gastrointestinal Plasmacytoma Causing Anemia in a Patient With Multiple Myeloma

Tuba Esfandyari; Susan C Abraham; Amindra S Arora


Nat Clin Pract Gastroenterol Hepatol. 2007;4(2):111-115. 

In This Article

The Case

A 70-year-old white male, who had been diagnosed with IgA lambda multiple myeloma 4 months previously, was referred to the Mayo Clinic for further evaluation of anemia. The patient had required over 15 U of packed red blood cells within a 2-month period for his anemia, despite being on recombinant erythropoietin (8,000 U, subcutaneously, three times a week), and his hemoglobin level was persistently below 9 g/dl.

The diagnosis of multiple myeloma was made using standard criteria: the presence of M-protein in serum, clonal bone marrow plasma cells and lytic bone lesions. The patient complained of progressive fatigue, generalized weakness, lower rib and shoulder pain and dyspnea on exertion. He had no gastrointestinal symptoms.

The patient's medical history was significant for osteoarthritis, coronary artery disease with coronary artery bypass graft surgery, hypertension and hypercholesterolemia. He stopped smoking about 20 years ago, and rarely consumed alcohol. Family history was remarkable for colon cancer (in his brother aged 65 years) coronary artery disease and Alzheimer's disease (in his sister). Current medications included furosemide 40 mg daily, carvedilol 12.5 mg twice daily, enalapril 20 mg daily, pantoprazole 40 mg daily, and atorvastatin 10 mg at bedtime.

On physical examination, the patient's blood pressure was 130/70 mmHg, heart rate 96 beats per minute, respiratory rate 12 breaths per minute, and body temperature 36.8 ºC. Abdominal examination showed no hepatosplenomegaly, normoactive bowel sounds, and no tenderness. The rest of the physical examination, which included cardiac and lung function and lymph node examination was unremarkable except for a 4.0 × 7.0 cm mass in the left lower rib cage and osteoarthritic changes to the hands and feet.

Laboratory studies showed a hemoglobin level of 89 g/l (normal range: 138–172 g/l), a normal mean corpuscular volume, and normal white blood cell and platelet counts. Serum protein electrophoresis showed an elevated total protein level of 97 g/dl, a normal albumin level, and an M-spike of 44.8 g/l (which corresponded to the level of monoclonal proteins). A bone marrow aspirate and blood clot examination demonstrated a myeloid to erythroid ratio of 1:1, with an overall cellularity of 40%, as well as a marked increase in plasma cells, which comprised 50–60% of bone marrow cellularity. An esophagogastroduodenoscopy showed a dilated Schatzki's ring.

In view of the patient's history of coronary artery disease, which included a myocardial infarction, coronary artery bypass graft, and left ventricular dysfunction, he was not a candidate for vincristin, doxorubicin and dexamethasone chemotherapy. Instead, he received standard therapy with melphalan and prednisone. After two cycles of this treatment regimen, his serum protein electrophoresis pattern improved with a total protein level of 78 g/l, albumin level 26 g/l, and M spike 29 g/l.

Further work-up included a cytogenetic analysis (which was normal) as well as an iron stain of the bone marrow aspirate (which showed normal iron stores). Congo red stains of the subcutaneous fat aspirate and bone marrow biopsy were negative for amyloidosis. Immunophenotyping was positive for CD38 (a surface protein that influences differentiation and proliferation of myeloma cells) and CD45 (leukocyte common antigen), but negative for B-cell-associated antigens, CD19 and CD20; these results, therefore, supported the diagnosis of multiple myeloma. As part of his work-up for recalcitrant anemia, hemolysis was excluded by a normal lactate dehydrogenase result, a peripheral blood smear, haptoglobin and bilirubin assays. In addition, the patient's red blood cell mean corpuscular volume, platelet count, coagulation tests, iron tests, and vitamin B12 and folate levels were within normal limits.

The patient then underwent upper endoscopy, which showed multiple small hemorrhagic friable plaques in the stomach body as well as the second portion of the duodenum (Figure 1a–c). The plaques were quite friable. The duodenum bulb looked normal. The pathologic review clearly demonstrated that these plaques had dense, atypical, plasma-cell infiltrates consistent with multiple myeloma involvement of the upper gastrointestinal tract (Figure 2a–c). The monoclonal nature of the plasma cell infiltrates was demonstrated both by in situ hybridization and by immunohistochemical staining of the biopsies for immunoglobulin kappa and lambda light chains, which revealed distinct labeling for lambda light chain in the plasma cell cytoplasm and negative labeling for kappa light chain (Figure 3a–d). Congo red stains of gastric and duodenal biopsy specimens were negative for amyloidosis. Gastric biopsies were negative for Helicobacter pylori.

Endoscopic images of a 70-year-old white male with multiple myeloma. Multiple small hemorrhagic friable plaques can be seen in (A) the stomach body and (B,C) two views of the second portion of the duodenum.

Histologic images of tissue specimens from a 70-year-old white male with multiple myeloma and gastrointestinal plasmacytoma. (A) Duodenal involvement of multiple myeloma. There is expansion of the lamina propria and coarsening of the villi (left side), which contrasts with the normal villous architecture in an uninvolved area (right side) (stained with hematoxylin and eosin; original magnification ×10). (B) Gastric involvement of multiple myeloma. The foveolar neck regions are splayed apart by myeloma infiltrates (stained with hematoxylin and eosin; original magnification ×20). (C) Atypical plasma cells are visible at high magnification (stained with hematoxylin and eosin; original magnification ×60). The nuclear:cytoplasmic area ratio is increased and many of the cells have small nucleoli.

In situ hybridization and immunohistochemical characterization of the plasma cell infiltrate. Immunohistochemical stain for lambda immunoglobulin light chain is strongly positive within the plasma cell cytoplasm (A), whereas stain for kappa light chain is negative (B) (gastric biopsies; original magnification ×40). In situ hybridization is positive for lambda light chain (C) and negative for kappa light chain (D) (duodenal biopsies; original magnification ×20).

The diagnosis of gastrointestinal plasmacytoma was suspected endoscopically and confirmed by pathologic review of the biopsy specimens. The patient was advised to continue his PPI medication and to avoid the use of NSAIDs. The management plan for the patient was outlined by the oncologist involved in his care. Although cure is not expected, survival might be prolonged by autologous stem cell transplantation compared with chemotherapy alone. Alkylating agents have a damaging effect on hematopoietic stem cells and, therefore, melphalan and prednisone were discontinued during his evaluation for autologous stem cell transplantation. The patient was started on high-dose pulse dexamethasone therapy for a few months and discharged to his local oncologist's care. The management plan included follow-up after 3 months to re-evaluate the patient's suitability for autologous stem cell transplantation. The patient died at home after infectious complications that caused multiorgan failure.


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