Gastrointestinal Amyloidosis in Biclonal Gammopathy

• DOI: 10.1016/j.clml.2021.02.015
• Publication Date: 2021-03-07T16:29:49Z
• Authors: Matteo Coen; Aurélie Bornand; Kaveh Samii; Flora Koegler; Jacques Serratrice
• Keywords: Gammopathy; Lymphoplasmacytic Lymphoma; Macroglobulinemia; AL amyloidosis; Plasmapheresis

•AL amyloidosis is classically associated with plasma cell disorders. Albeit rarely, it has also been described in association hematological disorders.•We describe here a case of patient gastrointestinal AA amyloidosis, which was likely the result long-standing biclonal gammopathy IgM-κ and IgG-λ.•We believe that this finding can be instrumental to raise awareness about possibility gammopathies amyloidosis. A 55-year-old woman Swiss ancestry referred our hospital for chronic diarrhea, dysphagia, weight loss. The had diagnosed Waldenström macroglobulinemia monoclonal immunoglobulin (Ig)M-kappa (κ)-type paraprotein 15 years earlier. Different treatment regimens (plasmapheresis, corticosteroids, cyclosporin A, cyclophosphamide) proved ineffective. Therefore, 3 after initial diagnosis she underwent syngenic donor hematopoietic stem transplantation. remission since She complained sicca syndrome (with positive Schirmer test sialometry) blurred vision right eye because (almost 30-year duration) bilateral chorioretinitis (posterior uveitis, chorioretinal atrophy, hyalitis, subretinal pigment epithelium infiltration). treated mycophenolate mofetil prednisone. An vitrectomy showed presence an intraocular B-cell population, absent on further biopsy. Five before admission, laparoscopic tumorectomy chromophobe renal carcinoma (pT1bc N0c M0 R0); tumor then. On physical examination, her body mass index 16.15 kg/m2. Vital parameters were normal. Laboratory tests normal red blood white counts slight thrombocytosis (370 g/L) elevated inflammatory markers: C-reactive protein, 35.70 mg/L (N < 5); fibrinogen, 8.1 g/L serum amyloid 50.4 6.8). Blood chemistry biochemical signs malnutrition (decreased levels albumin prealbumin: 32 143 mg/L, respectively). Liver kidney function unremarkable, as extensive infectious (stool culture) immune panel. Serum electrophoresis peak gamma region (size M spike, 0.2 g/L), immunofixation IgG-lambda (λ), first 6 κ λ free light chains 37.2 (normal, 3.3-19.4) 29.9 5.7-26.3), κ/λ ratio 1.24 0.26–1.65). Urinary not performed; urinalysis moderate proteinuria (A2, 397.8 mg/g) albuminuria (8.8 mg/g). Peripheral flow cytometry clonal population (CD19+, CD20+, CD5–, CD10–, CD38–), representing 87% B lymphocytes 0.06% total leukocytes. Bone marrow cytology revealed lymphoid (approximately 2% leucocyte comprised T [61%], polyclonal cells [3%], natural killer [36%]). Immunophenotypic analysis by performed bone aspirate no evidence hematologic malignancy. Biopsy cellularity trilinear hematopoiesis without neoplastic infiltration. Colonoscopy oesogastroduodenoscopy biopsy performed. Histology examination random biopsies tissue architecture inflammation. Congo stain reddish deposits small arteries duodenal, ileal, colon submucosal layers (Figure 1, Figure 2, 3, 4); protein identified vascular walls submucosa duodenum, ileum, immunohistochemistry (mouse IgG2a recognizing clone mc1) 5). Whole-body positron emission tomography/computed tomography Echocardiography did show myocardial involvement. Electromyography axonal sensorimotor length-dependent polyneuropathy, compatible neuropathy.Figure 2Slightly Ectatic Vessels Colonic Submucosa, With Wall Thickened Deposits Amorphous Eosinophilic Material; Standard Hematoxylin Eosin Staining (Magnification 400×).View Large Image ViewerDownload Hi-res image Download (PPT)Figure 3Congo Red Conventional Light Microscopy Highlighting Amyloid Within Vascular Walls 4Congo Polarized Showing Apple-Green Birefringence 5Positive Immunostaining Duodenal Submucosa (Magnifications 100×).View (PPT) We concluded patient's symptoms could ascribed intestinal Some reports amelioration or regression patients colchicine.1Hirasaki S Koide N Nakagawa K et al.Amyloid colitis: successful colchicine therapy.Dig Endosc. 1998; 10: 216-222Crossref Scopus (2) Google Scholar course (0.5 mg daily) tried, but slowly ameliorated. evaluated professional nutritionist started oral nutritional supplements; increased, finally discharged home weeks hospitalization. Gastrointestinal represents manifestation systemic misfolding disorder characterized deposition insoluble extracellular fragments. In occur close proximity vasculature, nerves, nerve plexuses within muscularis mucosae. aggregates then affect peristalsis, gut wall compliance, vascularization2Rowe Pankow J Nehme F Salyers W amyloidosis: review literature.Cureus. 2017; 9: e1228PubMed Scholar; hence, typical (ie, nausea, malabsorption) occurred patient.3Bird Guidelines management AL amyloidosis.Br Haematol. 2004; 125: 681-700Crossref PubMed (140) At least 17 types have described, they differ from type precursor promoting amyloidogenic). 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Spencer Blackett, London1890Google authors stated conflicts interest.