57–year–old male came with 4 weeks of constant 6/10 diffuse abdominal pain which was aggravated by meals. He had worsening constipation with loss of appetite, early satiety and a 15 lb. weight loss. 2 months prior had a cholecystectomy for questionable gallbladder sludge. He had lightheadedness with exertion attributed to dehydration after a negative stress test and his bilateral carpal tunnel syndrome symptoms were worsening recently. He had a PMH of IgM light chain gammopathy with peripheral neuropathy and a subsequent bone marrow biopsy confirmed lymphoplasmacytic lymphoma (LPL) which was under surveillance. He had a tachycardia of 110 bpm and a BP of 95/60. He had a prominent JVP and pedal edema with a 2/6 systolic murmur at the LSB. There were decreased breath sounds at the lung bases and abdomen was diffusely tender with no guarding and hypoactive bowel sounds. Of note, his tongue appeared enlarged with scalloped edges and he alleged his voice had become hoarse recently. CBC showed a mild AOCD. Lipase and LFTs were normal except for an albumin of 2.1 g/dL. Sodium was 129, creatinine 0.9 and troponin was 0.34 on three draws with no EKG changes of ischemia. NT–proBNP was 24437 pg/dL. CXR showed bilateral pleural effusions, abdominal series was unrevealing. Urine protein–creatinine ratio was 1.3. CT abdomen was normal. Gastric emptying study showed mild gastroparesis. EGD showed a small GU with stigmata of previous ooze and colonoscopy was normal. The patient was started on enteral feeding and a PPI for symptomatic relief and constipation was aggressively treated. An echocardiogram showed biatrial enlargement with biventricular hypertrophy (with sparing of the apical segments), thickening of the mitral valve and EF of 41% suggesting a diffuse infiltrative process. He was started on diuresis with a plan for endomyocardial biopsy however the gastric and colonic biopsies showed positive congo red staining and immunohistochemistry confirmed AL type amyloidosis. A bone marrow biopsy confirmed LPL and also primary amyloid infiltration. Hematology recommended bortezomib and steroids however on day 5 of treatment he died from cardiac arrest. Autopsy revealed diffused systemic amyloidsis in stomach, small bowel, colon, myocardium, valves, kidneys with no other obvious cause of death.
AL Amyloidosis is a rare disorder associated with plasma cell dyscrasias which can affect the heart, kidney, GI tract, liver, peripheral nerves and skin. Interestingly our patient showed simultaneous severe involvement of multiple organs in the setting of a LPL. Elevated troponin, uric acid and NT–pro BNP generally predict a higher mortality Patients may have a rapid decline with SCD hence arguing for expedited evaluation and initiation of treatment.
Amyloidosis is always in the differential of multisystemic infiltrative diseases however expedited evaluation and treatment is mandatory, especially with cardiac involvement, to favorably affect outcomes
Figure 1Ibuprofen induced non caseating granuloma.