Two months after moving to the United States from Egypt, 67-year-old Mr. A presented to the emergency department with moderate shortness of breath that worsened on exertion, periodic cough, and fatigue. The patient had developed generalized edema along with oliguria some time ago, and he had been treated for renal insufficiency in his homeland. Treatment had been conservative; he never received dialysis. Mr. A had persistently low hemoglobin, requiring multiple transfusions. However, no workup for his chronic anemia was done prior to his coming to the United States. Mr. A was admitted for evaluation and treatment of chronic anemia and renal failure.

1. Physical examination
On admission, Mr. A’s BP was 95/66 mm Hg. His pulse was 99 beats per minute, temperature 100°F, and respiratory rate 22 breaths per minute. He appeared lethargic and in moderate respiratory distress. Lung auscultation revealed bilateral rales. Mr. A’s abdomen was soft and nontender and demonstrated a shifting dullness, reflecting a possible ascites. There was no organomegaly. Neurologically, there were no focal deficits. His extremities showed bilateral pedal edema.

2. Laboratory results
Mr. A’s admitting laboratory values were hemoglobin 9.5 g/dL; hematocrit 24.3%; RBCs 3.22×106/µL; WBCs 9.8×103/µL; platelets 120×103/µL; and mean corpuscular volume 4 fL. The patient’s metabolic panel showed sodium 129 mmol/L, potassium 5.3 mmol/L, chloride 101 mmol/L, blood urea nitrogen 144 mg/dL, and creatinine 4.2 mg/dL. Cardiac enzymes were within normal range.

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3. Hospital course
Mr. A was put on hemodialysis for his declining renal status, and a complete workup was started for his chronic anemia and coagulopathy. Flow cytometry studies revealed a monoclonal l-positive plasma cell population consistent with a plasma cell dyscrasia. Protein electrophoresis showed multiple restriction bands. Mr. A’s serum b2-microglobulin was 83.0 mg/L. Serum/urine electrophoresis showed immunoglobulin (Ig) A l monoclonal proteins. IgA was elevated at 3,577 mg/dL, IgG was 291 mg/dL, and IgM was <33 mg/dL.

Bone marrow aspirate revealed nearly complete replacement of normal marrow elements by sheets of plasma cells manifesting varying degrees of cytologic atypia. Iron stores were present. Bone survey showed lucency within the anterior aspect of the parietal bone and multiple scattered lucencies within the humerus and femur. A diagnosis of multiple myeloma was reached.

Before Mr. A could be discharged to the care of an oncologist, however, he developed severe upper and lower GI bleeding. After becoming hypotensive, he was started on fluids and, eventually, norepinephrine bitartrate (Levophed) and dopamine. The patient received multiple transfusions of packed RBCs and fresh frozen plasma. A GI bleeding scan (tagged RBCs) showed accumulation of isotope in the small bowel, suggesting that the source of the bleeding was in the stomach and duodenum. Esophagogastroduodenoscopy (EGD) detected a Dieulafoy ulcer, described as an ectatic submucosal artery that, with pressure over time, erodes through the gastric mucosa, resulting in hemorrhage. This arterial vessel of abnormal size reaches the mucosa, causing a tiny ulceration by permanent compression of the mucosal layer. Although found predominantly in the stomach, Dieulafoy ulcers have rarely occurred in the colon and the rectum.

4. Analysis
Multiple myeloma and amyloidosis are closely related. The immunoglobulin-related form of amyloidosis is a monoclonal plasma cell disorder in which the secreted monoclonal immunoglobulin protein forms insoluble fibrillar deposits in one or more organs. The deposits almost always contain immunoglobulin light (L) chains or L-chain fragments, termed amyloid L (AL)-chain type.

In most patients, the monoclonal L chain remains soluble in the bloodstream. In some patients, the physiochemical characteristics of the L-chain fragment lead to its deposition as amyloid. These AL deposits can develop in any organ system. In a minority of cases, localized amyloid deposits (including amyloid masses [amyloidomas]) may be found in various sites, even in the absence of systemic disease. The pathogenesis involved is not well understood, but a small, localized clone of plasma cells apparently produces immunoglobulins, which form deposits near the site of synthesis.

The unique association found in Mr. A’s case was the detection of the Dieulafoy ulcer. Most patients with AL have histologic evidence of infiltration of the gut, particularly in the blood vessels. However, deposition is symptomatic in only a minority of patients. A prior case report has shown Dieulafoy ulcer giving rise to severe hemorrhage in a patient with benign biclonal gammopathy. It is highly likely that deposition of amyloid fibrils in Mr. A’s gastric artery hastened the hemorrhagic process with hematemesis and later severe rectal bleeding.

5. Treatment
The area of the Dieulafoy ulcer was injected with 7 cc of epinephrine in a 1:10,000 dilution and completely cauterized with a gold probe using the 14-watt setting. Mr. A’s hemodynamic status was restored to normal, with no further hematemesis or hematochezia.

As far as treatment of Mr. A’s multiple myeloma was concerned, we started  him on the steroid melphalan (Alkeran) and thalidomide (Thalomid).

Once he was hemodynamically stable, Mr. A was discharged and advised to follow up with oncology.

Dr. Sen is attending physician at Betsy Johnson Regional Hospital and Harnett Family Medicine, both in Harnett County, N.C.

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