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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">To the Editor&#58;</span></p><p class="elsevierStylePara">The deposition of the protein SAA &#40;serum amyloid A&#41; is responsible for systemic amyloidosis&#44; which is sometimes associated with certain neoplasias&#46;<span class="elsevierStyleSup">1</span> The association between amyloidosis and gastrointestinal stromal tumour &#40;GIST&#41;<span class="elsevierStyleSup">2</span> is extremely rare and only two cases have been reported&#46;<span class="elsevierStyleSup">3&#44;4</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">CASE REPORT</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">We report the case of a 64-year-old male with no relevant history&#44; who sought treatment due to loss of 17kg&#44; asthaenia&#44; anorexia and anaemia&#46; A colonoscopy was performed&#44; showing unspecific inflammatory changes of the colonic mucosa&#44; and a gastroscopy was performed&#44; revealing a gastric neoplasia and antral gastropathy of which biopsies were taken&#46; The gastric biopsy histology confirmed a neoplastic proliferation with solid pattern suggestive of GIST&#44; and the immunohistochemistry was positive for CD117&#46; AA amyloid deposits were observed in biopsies of the gastric mucosa&#44; the tumour and the colonic mucosa&#46;</p><p class="elsevierStylePara">The patient subsequently showed lower limb oedema and diarrhoea&#46; The analytical tests showed&#58; haemoglobin 8&#46;7g&#47;dl&#44; haematocrit 28&#37;&#44; mean corpuscular volume 75&#44; serum creatinine 1&#46;3mg&#47;dl&#44; total protein 5&#46;9g&#47;dl&#44; albumin 1&#46;36g&#47;dl&#44; cholesterol 96mg&#47;dl&#44; transferrin saturation index 13&#37;&#44; proteinuria 5&#46;1g&#47;day and sediment with 4-6 red blood cells&#47;high power field&#46; Given the poor condition of the patient&#44; renal biopsy was not performed and it was assumed that amyloidosis previously observed in gastrointestinal biopsies was responsible for the nephrotic syndrome and diarrhoea&#46; Cardiac amyloidosis was excluded by echocardiogram&#46;</p><p class="elsevierStylePara">Treatment was initiated with 100mg&#47;day of selective cKit tyrosine kinase inhibitor&#44; Imatinib &#40;Glivec<span class="elsevierStyleSup">&#174;</span>&#41;&#46; 15 months after diagnosis&#44; surgical removal was decided after observing a reduction in the tumour mass in the study by positron emission tomography&#47;computerised tomography&#46; The pathological anatomy confirmed the involvement of the removed splenic flexure of the colon and stomach&#46; In this regard&#44; it was classified as a high-risk GIST &#40;&#62;5cm in size&#44; &#62;5 mitosis per high power field&#41;&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">Renal function continued to deteriorate&#44; and as such&#44; the patient finally began periodic haemodialysis&#44; one year and seven months after the discovery of GIST with the diagnosis of chronic renal failure secondary to AA amyloidosis&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">Amyloidosis is characterised by the deposition of proteinaceous material&#44; which typically has a fibrillar ultrastructure with beta folding&#44; making it insoluble and resistant to proteolytic enzymes&#46; Depending on the fibrillar protein that is deposited&#44; several forms can be distinguished&#46;<span class="elsevierStyleSup">1</span></p><p class="elsevierStylePara">In secondary amyloidosis&#44; the fibrillar protein amyloid A&#44; derived from the precursor serum A &#40;SAA&#41; is deposited&#44; which acts as an acute phase reactant&#46; It is induced by chronic inflammatory diseases such as rheumatoid arthritis and the familial Mediterranean fever or some infections&#44; such as tuberculosis or osteomyelitis&#46; Neoplasias are also an uncommon cause of systemic amyloidosis&#44; especially in renal cell carcinoma or Hodgkin&#8217;s disease&#46;<span class="elsevierStyleSup">1&#44;5&#44;6</span></p><p class="elsevierStylePara">GIST constitutes less than 1&#37; of tumours of the digestive system&#46; The most common location is the stomach&#44; but it can be found throughout the entire gastrointestinal tract and adjacent organs&#46; Most have a mutation in the proto-oncogene cKIT &#40;CD117&#41;&#44; whose detection confirms diagnosis&#46; Specific inhibitors against the latter have become first-line drugs as coadjuvants to surgery or for tumours that are unresectable due to their extensive proliferation&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">The link between GIST and amyloidosis was initially described by Jaakkola et al&#46;<span class="elsevierStyleSup">3</span> in a 59-year-old male with normal renal function who presented with a pelvic mass&#44; whose histopathology coincided with that of a GIST&#46; Three months later&#44; he displayed renal function deterioration and began periodic haemodialysis&#46; AA amyloidosis was found in the renal biopsy&#46; Later&#44; Overstreet et al&#46;<span class="elsevierStyleSup">4</span> described the case of a 69-year-old male who was diagnosed with a GIST&#44; with amyloid deposits in the spleen&#44; adrenal glands and liver&#46;</p><p class="elsevierStylePara">Both cases involved a GIST with high mitotic index and large size&#46; Perhaps this is associated with an increased cytokine-mediated inflammatory response and therefore an increased production of SAA protein as an acute phase reactant&#46; Similar hypotheses have been suggested in other cases of amyloidosis linked to tumours&#46; Thus&#44; for example&#44; it is believed that the secretion of interleukin-6 in the germinal centres of lymph nodes could stimulate the synthesis of SAA and be involved in secondary amyloidosis in Castleman&#8217;s disease&#46;<span class="elsevierStyleSup">5</span> However&#44; the pathophysiology of the association with GIST is yet to be studied due to its rarity&#46;</p><p class="elsevierStylePara">In conclusion&#44; GIST is an extremely rare cause of secondary amyloidosis that can result in rapidly progressive renal failure that requires renal replacement therapy within a few months&#46; Perhaps a more thorough study of the pathophysiology linking these two entities would open the way for the development of more effective therapeutic tools&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">The authors declare that they have no conflicts of interest related to the contents of this article&#46;</p>"
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Chronic renal failure secondary to systemic amyloidosis associated with gastrointestinal stromal tumour
Insuficiencia renal crónica secundaria a amiloidosis sistémica asociada a tumor del estroma gastrointestinal
Laura Muñiz-Paciosa, Enrique Morales-Ruiza, Fernando Aguilarb, Florencio García-Martína
a Servicio de Nefrología, Hospital 12 de Octubre, Madrid,
b Servicio de Medicina Interna, Hospital 12 de Octubre, Madrid,
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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">To the Editor&#58;</span></p><p class="elsevierStylePara">The deposition of the protein SAA &#40;serum amyloid A&#41; is responsible for systemic amyloidosis&#44; which is sometimes associated with certain neoplasias&#46;<span class="elsevierStyleSup">1</span> The association between amyloidosis and gastrointestinal stromal tumour &#40;GIST&#41;<span class="elsevierStyleSup">2</span> is extremely rare and only two cases have been reported&#46;<span class="elsevierStyleSup">3&#44;4</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">CASE REPORT</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">We report the case of a 64-year-old male with no relevant history&#44; who sought treatment due to loss of 17kg&#44; asthaenia&#44; anorexia and anaemia&#46; A colonoscopy was performed&#44; showing unspecific inflammatory changes of the colonic mucosa&#44; and a gastroscopy was performed&#44; revealing a gastric neoplasia and antral gastropathy of which biopsies were taken&#46; The gastric biopsy histology confirmed a neoplastic proliferation with solid pattern suggestive of GIST&#44; and the immunohistochemistry was positive for CD117&#46; AA amyloid deposits were observed in biopsies of the gastric mucosa&#44; the tumour and the colonic mucosa&#46;</p><p class="elsevierStylePara">The patient subsequently showed lower limb oedema and diarrhoea&#46; The analytical tests showed&#58; haemoglobin 8&#46;7g&#47;dl&#44; haematocrit 28&#37;&#44; mean corpuscular volume 75&#44; serum creatinine 1&#46;3mg&#47;dl&#44; total protein 5&#46;9g&#47;dl&#44; albumin 1&#46;36g&#47;dl&#44; cholesterol 96mg&#47;dl&#44; transferrin saturation index 13&#37;&#44; proteinuria 5&#46;1g&#47;day and sediment with 4-6 red blood cells&#47;high power field&#46; Given the poor condition of the patient&#44; renal biopsy was not performed and it was assumed that amyloidosis previously observed in gastrointestinal biopsies was responsible for the nephrotic syndrome and diarrhoea&#46; Cardiac amyloidosis was excluded by echocardiogram&#46;</p><p class="elsevierStylePara">Treatment was initiated with 100mg&#47;day of selective cKit tyrosine kinase inhibitor&#44; Imatinib &#40;Glivec<span class="elsevierStyleSup">&#174;</span>&#41;&#46; 15 months after diagnosis&#44; surgical removal was decided after observing a reduction in the tumour mass in the study by positron emission tomography&#47;computerised tomography&#46; The pathological anatomy confirmed the involvement of the removed splenic flexure of the colon and stomach&#46; In this regard&#44; it was classified as a high-risk GIST &#40;&#62;5cm in size&#44; &#62;5 mitosis per high power field&#41;&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">Renal function continued to deteriorate&#44; and as such&#44; the patient finally began periodic haemodialysis&#44; one year and seven months after the discovery of GIST with the diagnosis of chronic renal failure secondary to AA amyloidosis&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">Amyloidosis is characterised by the deposition of proteinaceous material&#44; which typically has a fibrillar ultrastructure with beta folding&#44; making it insoluble and resistant to proteolytic enzymes&#46; Depending on the fibrillar protein that is deposited&#44; several forms can be distinguished&#46;<span class="elsevierStyleSup">1</span></p><p class="elsevierStylePara">In secondary amyloidosis&#44; the fibrillar protein amyloid A&#44; derived from the precursor serum A &#40;SAA&#41; is deposited&#44; which acts as an acute phase reactant&#46; It is induced by chronic inflammatory diseases such as rheumatoid arthritis and the familial Mediterranean fever or some infections&#44; such as tuberculosis or osteomyelitis&#46; Neoplasias are also an uncommon cause of systemic amyloidosis&#44; especially in renal cell carcinoma or Hodgkin&#8217;s disease&#46;<span class="elsevierStyleSup">1&#44;5&#44;6</span></p><p class="elsevierStylePara">GIST constitutes less than 1&#37; of tumours of the digestive system&#46; The most common location is the stomach&#44; but it can be found throughout the entire gastrointestinal tract and adjacent organs&#46; Most have a mutation in the proto-oncogene cKIT &#40;CD117&#41;&#44; whose detection confirms diagnosis&#46; Specific inhibitors against the latter have become first-line drugs as coadjuvants to surgery or for tumours that are unresectable due to their extensive proliferation&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">The link between GIST and amyloidosis was initially described by Jaakkola et al&#46;<span class="elsevierStyleSup">3</span> in a 59-year-old male with normal renal function who presented with a pelvic mass&#44; whose histopathology coincided with that of a GIST&#46; Three months later&#44; he displayed renal function deterioration and began periodic haemodialysis&#46; AA amyloidosis was found in the renal biopsy&#46; Later&#44; Overstreet et al&#46;<span class="elsevierStyleSup">4</span> described the case of a 69-year-old male who was diagnosed with a GIST&#44; with amyloid deposits in the spleen&#44; adrenal glands and liver&#46;</p><p class="elsevierStylePara">Both cases involved a GIST with high mitotic index and large size&#46; Perhaps this is associated with an increased cytokine-mediated inflammatory response and therefore an increased production of SAA protein as an acute phase reactant&#46; Similar hypotheses have been suggested in other cases of amyloidosis linked to tumours&#46; Thus&#44; for example&#44; it is believed that the secretion of interleukin-6 in the germinal centres of lymph nodes could stimulate the synthesis of SAA and be involved in secondary amyloidosis in Castleman&#8217;s disease&#46;<span class="elsevierStyleSup">5</span> However&#44; the pathophysiology of the association with GIST is yet to be studied due to its rarity&#46;</p><p class="elsevierStylePara">In conclusion&#44; GIST is an extremely rare cause of secondary amyloidosis that can result in rapidly progressive renal failure that requires renal replacement therapy within a few months&#46; Perhaps a more thorough study of the pathophysiology linking these two entities would open the way for the development of more effective therapeutic tools&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">The authors declare that they have no conflicts of interest related to the contents of this article&#46;</p>"
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