The Coexistence of Multiple Myeloma-associated Amyloid Light-chain Amyloidosis and Fabry Disease in a Hemodialysis Patient

نویسندگان

  • Kensei Taguchi
  • Atsuo Moriyama
  • Goh Kodama
  • Yosuke Nakayama
  • Kei Fukami
چکیده

Fabry disease (FD) is an inherited lysosomal disorder caused by an X-linked α-galactosidase A deficiency. We report the case of a 50-year-old male FD patient on hemodialysis who presented with macroglossia-related speaking difficulty and gastrointestinal symptoms. An endoscopic analysis revealed multiple gastric ulcers, and a histological examination led to a diagnosis of amyloid light-chain amyloidosis. Serum free light-chain and bone marrow analyses detected multiple myeloma (MM). Treatment with bortezomib and dexamethasone significantly improved the patient's symptoms. This is the first case to demonstrate a potential pathogenic relationship between FD and MM. The similar gastrointestinal manifestations might have contributed to the diagnostic difficulty.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

A Rare Presentation of Multiple Myeloma: A Case Report of Hepatic Amyloidosis

The clinically reported case of liver involvement with multiple myeloma (MM) is rare. Amyloidosis, defined as a tissue deposition of clonal light-chain fibrils, has been reported in 10-15% of the MM patients. We described a rare MM patient with the primal presentation of fulminant hepatic failure and biliary system involvement due to amyloidosis. Our patient had the primal symptoms of hyperbili...

متن کامل

Bullous skin lesions in a patient with end-stage renal disease combined with myeloma and primary amyloidosis

Amyloidosis is a systemic disease involving multiple orchain lambda-type multiple myeloma and combined amyloid gans such as heart, kidney, and skin. It can also present with diverse skin lesions such as pinch hemorrhages and maculopapular eruptions; however, hemorrhagic bullous lesions are very rare. Here, I introduce a case of bullous amyloidosis undergoing hemodialysis for the treatment of en...

متن کامل

Myocardial 99mTc-MDP uptake on the bone scintigraphy in the hemodialysis-associated amyloidosis: A case report [Persian]

Amyloidosis is characterized by an abnormal extracellular deposition of amyloid in different organs, where it usually causes some type of dysfunction. Its cause is unknown. Five different types of amyloidosis have been described according to the underlying disease; immunoglobulin amyloidosis, familial amyloidosis, senile systemic amyloidosis, secondary amyloidosis and hemodialysis-associa...

متن کامل

PRIMARY LOCALIZED RETROPERITONEAL AMYLOID TUMOR WITH OSSEOUS META PLASIA: CASE REPORT AND REVIEW OF THE LITERATURE

Amyloidoma or amyloid tumor is a tumor-like localized deposit of amyloid encountered occasionally in association with multiple myeloma, various chronic inflammatory diseases and primary amyloidosis. Amyloid tumors unassociated with plasmacytoma or other disease are extremely rare in soft tissues and few cases in various situations have been reported. Histologic examination discloses amorp...

متن کامل

Gastrointestinal Tract Amyloidosis Presenting With Pneumatosis Intestinalis

Pneumatosis intestinalis is a radiographic finding of gas pockets within the bowel wall. It can be associated with a range of diagnoses, but the most life-threatening causes are mesenteric ischemia, bowel necrosis, and bowel obstruction. Here we present the case of a patient with multiple myeloma who had pneumatosis intestinalis due to gastrointestinal amyloidosis, which is a rare manifestation...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 56  شماره 

صفحات  -

تاریخ انتشار 2017