P-293: SYSTEMIC AMYLOIDOSIS WITH GASTROINTESTINAL INVOLVEMENT: LOW SUSPICIOUS PRESENTATION, LATE TREATMENT, MULTIORGAN DISFUNCTION AND HIGHER THE RISK OF DEATH AFTER AUTOLOGOUS STEM CELL TRANSPLANT.

Introduction:

Introduction:
Light chain amyloidosis (AL) is a heterogeneous and life-threatening disease caused by extracellular deposition of misfolded free light chains (FLC) that typically originate from small plasma cell clones. The majority of these patients have a significant impairment of vital organs, such as the heart, kidney, and liver. However gastrointestinal (GI) involvement is an uncommon manifestation of systemic amyloidosis. The most common clinical signs are nausea, vomiting, dysphagia, gastroparesis, gastro-esophageal reflux, loss of appetite, constipation, abdominal pain, bloating. The Gl bleeding may occur from any site of amyloid deposition and can be seen in up to 57% of patients. Few data are available, with the exception of some case reports. GI direct biopsy verification of the disease is needed to identify amyloid deposit.





Methods: NA

Results:

Case Report: 49 years old, female, without previous comorbidities was admitted to an emergency unit hospital in January 2020 to investigate epigastric pain and postprandial fullness that started eight months before diagnosis. The blood count was normal, abdominal ultrasound showed hepatomegaly. Endoscopy showed infiltrative lesion, of entire anterior region of the gastric body, with enanthematic and friable mucosa. Biopsy was congo red positive. Bone marrow aspirate showed 2,4% of clonal plamocytes Lambda monoclonal light chain. Mass spectrometry detected lambda-type immunoglobulin light chain.. She had she left Cardiac ventricle fibrosis due to cardiac Amyloid deposition, New York Heart Association Scored 3 (NYHA 3), hepatomegaly, GI and renal involvement. August 2020 patient was treated with cyclophosphamide, bortezomib, and dexamethasone, 6 cycles. Daratumumab was added for 4 cycles to improve response. According to Mayo Clinic organ response criteria the patient improved cardiac function (NYHA2), decreased pro-BNP, presented hematological response (very good parcial response), but no of liver or kidney improvement. June 2022 She was submitted to an autologous stem cell transplant (ASCT), at D+9 after ASCT she had a major GI bleeding, hypovolemic shock, progressing to organ failure and death.



Conclusions: CONCLUSION
It is important to draw attention to GI symptoms in amyloidosis involvement and its nonspecific symptoms could delay diagnosis and favor advanced organ failure. The lack of a risk score to estimate massive bleeding in GI involvement makes the management of these patients more difficult. This case had a late diagnose of systemic amyloidosis with GI, cardiac, hepatic and renal involvement. Despite of Cardiac and hematologic response, she progressed with hepatic and renal disease increasing the risk of death after transplant.