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Primary retroperitoneal amyloidosis – A challenging diagnostic
Amiloidosis retroperitoneal primaria – Un desafío diagnóstico
Claudia-Gabriela Moldovanua,b,
Corresponding author
moldovanu_claudia@yahoo.com

Corresponding author.
, Thibaut Zverc
a Department of Radiology, Hospital of Haute Saone, René Heymes Street, n. 2, 70000 Vesoul, France
b Department of Radiology, University of Medicine, Pharmacy, Science and Technology of Târgu Mureș, Gheorghe Marinescu Street, n. 38, 540139 Târgu Mureș, Romania
c Department of Radiology, Centre Hospitalier Universitaire de Besançon, Boulevard Fleming, 25030 Besançon, France
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We report the case of a 60-year-old man presenting with weight loss &#40;15<span class="elsevierStyleHsp" style=""></span>kg in the last month&#41;&#44; lack of energy&#44; abdominal pain and discomfort and lymphedema of the legs&#46; He had a previous medical history of gout&#44; systemic arterial hypertension &#40;diseases which had been diagnosed 10 years earlier and had been medicated and controlled&#41;&#44; and benign prostatic hyperplasia&#46; He had prostate biopsy and appendectomy in the past&#46; He did not smoke cigarettes and occasionally drinks alcohol&#46; His medical family history were unremarkable&#46; Initial investigations revealed with leukopenia&#44; white blood cell count&#44; 3&#46;37&#47;mm<span class="elsevierStyleSup">3</span> with 55&#46;5&#37; neutrophils&#44; 30&#47;mm<span class="elsevierStyleSup">3</span> eosinophils and 28&#46;5&#37; lymphocytes&#46; Other blood investigations&#44; including creatinine&#44; electrolytes&#44; liver function&#44; and random and fasting blood sugar levels&#44; were normal&#46; Also&#44; tumor markers such as CA19-9&#44; CEA and AFP were in the normal range&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Computed tomography of the abdomen revealed massive thickening of the retroperitoneum with calcifications that encased the abdominal vessels and kidneys&#44; but without compressing them &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; and also&#44; pelvic lymphadenopathy&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Extensive laboratory examinations &#40;serum and urinary protein electrophoresis&#41; were performed&#44; showing a monoclonal spike in the gamma region&#44; which indicated IgG and Bence&#8211;Jones protein lambda light chains and therefore the possibility of amyloidosis was considered&#46; Serum-free kappa and lambda chains were 36&#46;85 and 42&#46;90&#44; respectively&#46; The analysis of a biopsy of pelvic lymphadenopathy showed deposits of amorphous eosinophilic material&#44; hyalinization and clusters of small lymphocytes&#46; Congo red staining demonstrated amyloid deposition&#46; Consequently&#44; the diagnosis of lambda light chain retroperitoneal amyloidosis was confirmed by immunohistochemistry&#46; The patient was subsequently investigated for the underlying diseases&#44; but there was no evidence of systemic amyloidosis&#44; plasmacytoma&#44; or multiple myeloma&#46; Our patient was referred to our department of haematology and subsequently started on chemotherapy with seven cycles of bortezomib 0&#46;7<span class="elsevierStyleHsp" style=""></span>mg&#47;m<span class="elsevierStyleSup">2</span> and neofordex 40<span class="elsevierStyleHsp" style=""></span>mg&#44; and his condition improved with good drug tolerance&#46; Currently&#44; the patient is stable and the disease has stagnated&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Amyloidosis represents a group of diseases that occurs as a consequence of an extracellular deposition of amyloid protein in organs and&#47;or tissues&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Historically&#44; it can be classified as primary and secondary or systemic&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">1&#44;2</span></a> Primary amyloidosis&#44; also known as AL amyloidosis&#44; is a rare entity that is difficult to diagnose&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Retroperitoneal involvement is one of the rarest forms of presentation&#44; being a challenge&#44; a clinical and imaging dilemma&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">2&#44;3</span></a> Once the diagnosis of amyloidosis is suspected&#44; it must be confirmed by biopsy on the affected organ&#44; with histopathological assessment using the Congo red staining&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Treatment is largely based on steroids and with autologous stem cell transplantation in a select group of non-responsive patients&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Early diagnosis and treatment are essential in stabilizing the disease&#44; thereby improving the outcome and the prognosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">1&#44;3</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Acknowledgement</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors of this paper like to thank Cyril Faure from the Groupe Hospitalier de la Haute-Sa&#244;ne&#44; Hospital of Haute Saone&#44; Vesoul&#44; France&#44; Internal Medicine Department&#44; for his contribution to the diagnosis of this case&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflict of interest</span><p id="par0025" class="elsevierStylePara elsevierViewall">None&#46;</p></span></span>"
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Article information
ISSN: 02105705
Original language: English
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos