Localized Gastric Amyloidosis with Kappa and Lambda Light Chain Co-Expression

Esophagogastroduodenoscopy for cancer screening was performed in a 55-year-old woman as part of a health screening program, and revealed a depressed lesion approximately 20 mm in diameter in the lesser curvature of the mid-gastric body. Several biopsy specimens were collected as the lesion resembled early gastric cancer; however, histopathologic evaluation revealed chronic active gastritis with an ulcer and amorphous eosinophilic material deposition. Congo red staining identified amyloid proteins, and apple-green birefringence was shown using polarized light microscopy. Immunohistochemical staining revealed the presence of kappa and lambda chain-positive plasma cells. There was no evidence of underlying plasma cell dyscrasia or amyloid deposition in other segments of the gastrointestinal tract. Echocardiography and computed tomography of the chest, abdomen, and pelvis did not show any significant findings. Thus, the patient was diagnosed with localized gastric amyloidosis with kappa and lambda light chain coexpression.

Amiloidosis por cadenas livianas de inmunoglobulina con compromiso gástrico al debut: revisión de la literatura basada en un caso clínico / Gastric AL amyloidosis as initial manifestation: literature review from a case report

Systemic amyloidosis comprises a group of diseases characterized by low molecular weight subunit protein deposit in organs, including the gastrointestinal tract. The most frequent clinical manifestations are gastrointestinal bleeding, malabsorption syndrome, protein-losing enteropathy and chronic intestinal dysmotility. The diagnosis is confirmed with gastrointestinal tissue biopsy positive to Congo red stain or recognizing the amyloid fibrils by electron microscopy. The treatment is based in the management of gastrointestinal symptoms and chemotherapeutic drugs, including melphalan and prednisone or cyclophosphamide, bortezomib and prednisone. The bone marrow transplant is reserved for 70-year-old patients or younger without advanced comorbidities. We present a case of a patient with weight loss, anorexia, nausea and early satiety.

La amiloidosis sistémica a un conjunto de enfermedades caracterizadas por el depósito de subunidades fibrilares proteicas de bajo peso molecular en órganos, incluyendo el sistema digestivo. Sus manifestaciones clínicas más frecuentes son la hemorragia digestiva, síndrome malabsortivo, gastro-enteropatía perdedora de proteínas y dismotilidad gastrointestinal crónica. El diagnóstico se confirma con una biopsia de tejido gastrointestinal positiva a tinción rojo Congo o la visualización de fibrillas de amiloide mediante microscopia electrónica. El tratamiento está basado el manejo de los síntomas gastrointestinales y el oncológico, donde destacan esquemas quimioterapéuticos que incluyen melfalan y prednisona o ciclofosfamida, bortezomib y prednisona. El trasplante de médula ósea está reservado a pacientes menores de 70 años sin comorbilidades avanzadas. Presentamos en este artículo el caso de un paciente con baja de peso, anorexia, náuseas y saciedad precoz.

Three Cases of Amyloidosis Diagnosed by Endoscopic Biopsy of Stomach / 대한소화기내시경학회지

Since amyloidosis is usually diagnosed later in the disease process, a high index of suspicion is therefore necessary for earlier diagnosis. Confirmative diagnosis rests on a biopsy of the involved organ. Gastrointestinal amyloidosis causes a variety of symptoms including intestinal obstruction, ulcers, malabsorption, hemorrhaging, protein loss, diarrhea, anorexia, nausea, vomiting, and dysphagia. We confirmed amyloid deposits in the stomach in three patients with epigastric pain through a biopsy of erosive gastritis documented on a gastrofiberscopy. One patient with primary amyloidosis which had invaded his kidney, stomach, and heart, expired, although aggressive treatment with a pacemaker insertion, peritoneal dialysis, and ventilator care was performed. Another patient with multiple myeloma died on the 38th day, after having started systemic chemotherapy. The other patient with secondary amyloidosis due to rheumatoid arthritis, is currently receiving colchicine at our out patient clinic.

A Case of Multiple Myeloma of Kappa Light Chain Type Associated with Gastric Amyloidosis and Acute Renal Failure and Pathologic Fracture Due to Femur Plasmacytoma / 대한신장학회잡지

Primary amyloidosis has been reported to develop in 6 to 15% of patients with multiple myeloma and especially in 20 to 24% of patients with light chain myeloma. Although deposition of amyloid in the gastric mucosa is common in primary systemic amyloidosis(AL), gastric amyloidosis in AL type is rarely symptomatic. Also, pathologic fracture of the femur secondary to plasmacytoma is quite rare for the first manifestation of multiple myeloma. A case of gastric amyloidosis associated with gastric outlet obstruction and femur plasmacytoma in multiple myeloma is reported with review of literatures.