We report here a case of a 35-year-old male who presented with multi-system disease, which on evaluation was found to be due to primary systemic amyloidosis. We present the myriad manifestations of this uncommon disease entity.

Clinical manifestations of primary amyloidosis can be subtle, particularly when the gastrointestinal tract is involved. We present two cases that illustrate the diversity of such involvement and the subsequent problems with diagnosis. Gastrointestinal effects of primary systemic amyloidosis are reviewed and clinical pointers towards diagnosis are discussed. In particular these cases illustrate ...

BACKGROUND Amyloidosis is a heterogeneous disease caused by deposition of amyloid fibrils in organs and thereby interfering with physiological functions. Hardly any incidence data are available and most survival data are limited to specialist clinics. METHODS Amyloidosis patients were identified from the Swedish Hospital Discharge and Outpatients Registers from years 2001 through 2008. RESU...

Cardiac amyloidosis is a prognostically critical condition, since it is a frequent cause of death in patients with systemic amyloidosis. Lee et al. reported, for the first time, describing systemic amyloidosis in Korea from the perspective of cardiologists. In this paper, the authors enrolled 129 systemic amyloidosis patients retrospectively from 1999 to 2011. They showed that cardiac involve w...

despite the fact that hepatic involvement is frequently seen in systemic primary amyloidosis, major hepatic symptoms as primary manifestation and severe impaired liver function are rare. herein, we report a 38-year-old woman with primary hepatic amyloidosis, and severe  portal hypertension . the patient had ascites and markedly elevated alkaline phosphatase level at presentation. she had a rapi...

PURPOSE OF REVIEW This review aims to summarize recent developments in the area of systemic amyloidoses with emphasis on pathologic diagnosis. RECENT FINDINGS In recent years, management of amyloidosis has shifted from a purely supportive approach to quite diverse, radical and aggressive treatments. The central issue is the understanding that treatment of systemic amyloidoses depends on the m...

Amyloidosis is characterised by the accumulation of poorly soluble fibrous proteins in the extracellular space of various bodily organs. Light chain amyloidosis (AL) is recognised as the most common form of systemic amyloidosis. Light chains are deposited in the majority of bodily organs, and accumulation of them in the liver produces hepatomegaly. We report a case of AL-systemic amyloidosis wi...

The pattern of amyloid deposits in the femoral head is described in four cases, two of which had deposits of amyloid related to age and two of which had generalised systemic amyloidosis (one of primary amyloidosis, one of multiple myeloma). The deposition of amyloid in the articular cartilage of the femoral head was similar in all four cases. Heavy deposits of synovial amyloid were identified i...

Systemic amyloidosis was induced consistently in mice by intramuscular injection of syngeneic organ (liver and kidney) extracts mixed with CFA six times at weekly intervals. Syngeneic organ extract with CFA also induced amyloidosis of a lesser degree. All three strains of mice (C57BL/6, C3H/He, and BALB/c) injected with a syngeneic liver extract mixed with CFA developed systemic amyloidosis; th...