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Once the diagnosis is reached&#44; the percentage of infiltration of mast cells into the bone marrow indicates whether or not the patient has mast cell leukemia &#40;&#8805;20&#37; of infiltration&#41;&#46; If the infiltration is less than 20&#37;&#44; and there is no evidence of the findings listed under B or C &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#44; the disorder is indolent mastocytosis&#59; if only those reported under B are detected&#44; the diagnosis is SM&#59; and&#44; in the presence of those listed under C&#44; we are speaking of aggressive SM&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">2&#44;3</span></a></p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Bone disease in mastocytosis is related to the infiltration of the bone marrow by mast cells&#44; and is detected in 70&#37; of the cases&#46; The release of histamine by the mast cells induces sclerosis&#44; a fact that explains the presence of osteoblastic lesions&#44; and the release of heparin and of prostaglandins induces lysis&#46; This means that the differential diagnosis must include other processes such as myelofibrosis&#44; Paget&#39;s disease&#44; bone metastasis or fluorosis&#44; in the case of blastic lesions&#44; or cystic fibrosis-related osteoporosis&#44; thalassemia or Gaucher disease in lytic lesions&#46; The most common bone involvement consists of trabecular osteoporosis &#40;OP&#41;&#46; The bone changes have an extensive clinical variability&#44; from asymptomatic alterations that often go unnoticed&#44; to more aggressive presenting symptoms&#44; with multiple vertebral fractures&#44;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">4</span></a> as in the case we report&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Observation</span><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was a 40-year-old man with nothing remarkable in his medical records&#46; He was admitted in July 2013 with a 1-week history of intense thoracic back pain&#46; The radiological study revealed the presence of multiple signs of vertebral compression &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Bone densitometry measured by dual energy X-ray absorptiometry confirmed the presence of lumbar OP &#40;T-score &#8722;3&#46;37&#41;&#46; Causes secondary to OP in men &#40;hepatitis C virus&#44; hypogonadism&#44; hyperparathyroidism&#44; etc&#46;&#41; were ruled out&#44; as were lymphoproliferative disorders and other tumorigenic processes&#46; The patient required vertebroplasty in thoracic 7 due to thoracic back pain refractory to opioids &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#59; at that time the decision was made to initiate treatment with subcutaneous teriparatide&#46; Given the negative findings in the extensive study performed&#44; we found serum tryptase levels that were persistently high &#40;around 53&#46;9<span class="elsevierStyleHsp" style=""></span>&#956;g&#47;L &#91;normal&#44; 5&#46;5&#8211;13&#46;5&#93;&#41;&#46; A bone marrow aspirate and biopsy were consistent with mastocytosis&#46; Cytometry detected a mast cell concentration of 0&#46;13&#37;&#44; 99&#46;5&#37; of which were activated&#44; and 0&#46;5&#37; expressed CD25&#46; The c-Kit D816V mutation &#40;A7176T&#41;&#44; detected in whole bone marrow&#44; was restricted to mast cells &#40;TaqMan&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Once the diagnosis of SM had been confirmed &#40;24 months after the first vertebral fractures&#41;&#44; the patient underwent 2 years of teriparatide therapy&#44; and we opted for continuing with an antiresorptive agent &#40;denosumab every 6 months&#41;&#46; Although his bone mineral density did not get worse&#44; he had additional vertebral compression fractures&#46; Starting in July 2015&#44; with the diagnosis of indolent SM&#44; treatment with disodium chromoglycate was begun and&#44; to date &#40;December 2015&#41;&#44; he has had no new fractures&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">As the risk of OP and fractures in SM is high&#44; especially among men&#44;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">4</span></a> routine measurement of bone mineral density and morphometric study are recommended in these patients&#46; Systemic mastocytosis can appear in an indolent form&#44; with no cutaneous or organ manifestations and&#44; thus&#44; the diagnosis is not suspected&#46; We report the case of a young healthy man who had had several vertebral compression fractures in the absence of traumatic injury&#46; He had risk factors for OP&#44; and secondary causes had been ruled out&#46; The diagnosis was idiopathic osteoporosis&#44; although the diagnosis of SM was not discovered for years as there were no compatible symptoms&#46; For this reason&#44; we consider that SM should be included in the diagnostic study of OP in adults in whom an identifiable cause or risk factors are not detected&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">At the present time&#44; the most adequate treatment according to histomorphometric studies seems to be antiresorptive therapy&#59; however&#44; although the bone mass increases&#44; certain patients are still at risk for fractures&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusions</span><p id="par0035" class="elsevierStylePara elsevierViewall">Systemic mastocytosis is more common than we actually detect in clinical practice&#46; Even in patients with cutaneous mastocytosis&#44; the average time between the onset of symptoms and the diagnosis is 1 year&#46; In the absence of clinical signs&#44; SM should be suspected in men with OP and in patients with osteoblastic or osteolytic bone lesions of unknown origin&#46; In these cases&#44; repeatedly high serum tryptase levels make it possible to identify patients who should undergo a bone marrow study&#44; to provide early support in the definitive diagnosis&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Ethical Disclosures</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Protection of human and animal subjects</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Confidentiality of data</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Right to privacy and informed consent</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conflicts of Interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare they have no conflicts of interest&#46;</p></span></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La mastocitosis sist&#233;mica &#40;MS&#41; es una enfermedad clonal de los progenitores mastoc&#237;ticos de la m&#233;dula &#243;sea&#46; El cuadro cl&#237;nico var&#237;a desde una forma asintom&#225;tica &#40;indolente&#41; hasta una forma altamente agresiva con una supervivencia muy corta &#40;leucemia de mastocitos&#41;&#46; Un 28-34&#37; de los pacientes con MS tiene s&#237;ntomas relacionados con la afecci&#243;n &#243;sea en el momento del diagn&#243;stico y el 16&#37; fracturas&#46; La presentaci&#243;n de MS como fracturas vertebrales cl&#237;nicas en varones j&#243;venes no es frecuente&#46; A continuaci&#243;n describimos un caso de osteoporosis establecida como &#250;nica manifestaci&#243;n de MS&#46;</p></span>"
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                  \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Diagnostic criteria &#40;laboratory&#41;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Mastocytosis is considered to be systemic if a mayor and a minor criterion are met&#44; or 3 minor criteria coexist</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Severity criteria &#40;clinical&#41;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>B manifestations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Indicative of a high tumor burden and expansion of the genetic defect to several myeloid cell lines<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Bone marrow&#47;extracutaneous mastocytosis &#62;30&#37; with serum tryptase &#60;200<span class="elsevierStyleHsp" style=""></span>ng&#47;mL<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Hypercellular bone marrow with loss of lipid material&#44; subtle signs of dysmyelopoiesis in the absence of cytopenia&#44; or the World Health Organization criteria for myelodysplastic or myeloproliferative syndrome&#46;<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Organomegaly&#58; palpable hepatomegaly&#47;splenomegaly or lymphadenopathy &#62;2<span class="elsevierStyleHsp" style=""></span>cm according to images&#44; with no evidence of functional involvement&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>C manifestations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Indicative of organic functional changes owing to the infiltration<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Cytopenia&#58; neutrophils &#60;1000&#47;&#956;L&#44; platelets &#60;100&#44;000&#47;&#956;L&#44; hemoglobin &#60;10<span class="elsevierStyleHsp" style=""></span>g&#47;dL<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Hepatomegaly with ascites and an alteration in liver function<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Palpable splenomegaly with hypersplenism<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Malabsorption syndrome with hypoalbuminemia and weight loss<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Severe osteolytic or osteoporotic lesions with pathological fractures<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Enlargement of diverse organs with critical risk due to mast cell infiltration&nbsp;\t\t\t\t\t\t\n
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                            0 => "I&#46; Alvarez-Twose"
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                            3 => "A&#46; Matito"
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Case report
Multiple Vertebral Fractures in Young Man as First Manifestation of Systemic Mastocytosis
Fracturas vertebrales múltiples en varón joven como primera manifestación de una mastocitosis sistémica
Carmen Carrasco Cuberoa,
Corresponding author
, Eugenio Chamizo Carmonab
a Servicio de Reumatología, Complejo Hospitalario Universitario de Badajoz, Hospital Perpetuo Socorro, Badajoz, Spain
b Servicio de Reumatología, Hospital de Mérida, Mérida, Spain
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Once the diagnosis is reached&#44; the percentage of infiltration of mast cells into the bone marrow indicates whether or not the patient has mast cell leukemia &#40;&#8805;20&#37; of infiltration&#41;&#46; If the infiltration is less than 20&#37;&#44; and there is no evidence of the findings listed under B or C &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#44; the disorder is indolent mastocytosis&#59; if only those reported under B are detected&#44; the diagnosis is SM&#59; and&#44; in the presence of those listed under C&#44; we are speaking of aggressive SM&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">2&#44;3</span></a></p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Bone disease in mastocytosis is related to the infiltration of the bone marrow by mast cells&#44; and is detected in 70&#37; of the cases&#46; The release of histamine by the mast cells induces sclerosis&#44; a fact that explains the presence of osteoblastic lesions&#44; and the release of heparin and of prostaglandins induces lysis&#46; This means that the differential diagnosis must include other processes such as myelofibrosis&#44; Paget&#39;s disease&#44; bone metastasis or fluorosis&#44; in the case of blastic lesions&#44; or cystic fibrosis-related osteoporosis&#44; thalassemia or Gaucher disease in lytic lesions&#46; The most common bone involvement consists of trabecular osteoporosis &#40;OP&#41;&#46; The bone changes have an extensive clinical variability&#44; from asymptomatic alterations that often go unnoticed&#44; to more aggressive presenting symptoms&#44; with multiple vertebral fractures&#44;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">4</span></a> as in the case we report&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Observation</span><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was a 40-year-old man with nothing remarkable in his medical records&#46; He was admitted in July 2013 with a 1-week history of intense thoracic back pain&#46; The radiological study revealed the presence of multiple signs of vertebral compression &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Bone densitometry measured by dual energy X-ray absorptiometry confirmed the presence of lumbar OP &#40;T-score &#8722;3&#46;37&#41;&#46; Causes secondary to OP in men &#40;hepatitis C virus&#44; hypogonadism&#44; hyperparathyroidism&#44; etc&#46;&#41; were ruled out&#44; as were lymphoproliferative disorders and other tumorigenic processes&#46; The patient required vertebroplasty in thoracic 7 due to thoracic back pain refractory to opioids &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#59; at that time the decision was made to initiate treatment with subcutaneous teriparatide&#46; Given the negative findings in the extensive study performed&#44; we found serum tryptase levels that were persistently high &#40;around 53&#46;9<span class="elsevierStyleHsp" style=""></span>&#956;g&#47;L &#91;normal&#44; 5&#46;5&#8211;13&#46;5&#93;&#41;&#46; A bone marrow aspirate and biopsy were consistent with mastocytosis&#46; Cytometry detected a mast cell concentration of 0&#46;13&#37;&#44; 99&#46;5&#37; of which were activated&#44; and 0&#46;5&#37; expressed CD25&#46; The c-Kit D816V mutation &#40;A7176T&#41;&#44; detected in whole bone marrow&#44; was restricted to mast cells &#40;TaqMan&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Once the diagnosis of SM had been confirmed &#40;24 months after the first vertebral fractures&#41;&#44; the patient underwent 2 years of teriparatide therapy&#44; and we opted for continuing with an antiresorptive agent &#40;denosumab every 6 months&#41;&#46; Although his bone mineral density did not get worse&#44; he had additional vertebral compression fractures&#46; Starting in July 2015&#44; with the diagnosis of indolent SM&#44; treatment with disodium chromoglycate was begun and&#44; to date &#40;December 2015&#41;&#44; he has had no new fractures&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">As the risk of OP and fractures in SM is high&#44; especially among men&#44;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">4</span></a> routine measurement of bone mineral density and morphometric study are recommended in these patients&#46; Systemic mastocytosis can appear in an indolent form&#44; with no cutaneous or organ manifestations and&#44; thus&#44; the diagnosis is not suspected&#46; We report the case of a young healthy man who had had several vertebral compression fractures in the absence of traumatic injury&#46; He had risk factors for OP&#44; and secondary causes had been ruled out&#46; The diagnosis was idiopathic osteoporosis&#44; although the diagnosis of SM was not discovered for years as there were no compatible symptoms&#46; For this reason&#44; we consider that SM should be included in the diagnostic study of OP in adults in whom an identifiable cause or risk factors are not detected&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">At the present time&#44; the most adequate treatment according to histomorphometric studies seems to be antiresorptive therapy&#59; however&#44; although the bone mass increases&#44; certain patients are still at risk for fractures&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusions</span><p id="par0035" class="elsevierStylePara elsevierViewall">Systemic mastocytosis is more common than we actually detect in clinical practice&#46; Even in patients with cutaneous mastocytosis&#44; the average time between the onset of symptoms and the diagnosis is 1 year&#46; In the absence of clinical signs&#44; SM should be suspected in men with OP and in patients with osteoblastic or osteolytic bone lesions of unknown origin&#46; In these cases&#44; repeatedly high serum tryptase levels make it possible to identify patients who should undergo a bone marrow study&#44; to provide early support in the definitive diagnosis&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Ethical Disclosures</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Protection of human and animal subjects</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Confidentiality of data</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Right to privacy and informed consent</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conflicts of Interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare they have no conflicts of interest&#46;</p></span></span>"
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            2 => "Mastocitosis sist&#233;mica"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Systemic mastocytosis &#40;SM&#41; is a clonal disease of mast cell progenitors from the bone marrow&#46; The clinical picture varies from asymptomatic forms &#40;indolent&#41; to a highly aggressive form with a very short &#40;mast cell leukemia&#41; survival&#46; Between 28 and 34&#37; of patients with SM are related to bone condition at the time of diagnosis and 16&#37; have symptomatic fractures&#46; The presentation of SM as clinical vertebral fractures in young men is rare&#46; Here&#44; we describe a case of established osteoporosis as the only manifestation of SM&#46;</p></span>"
      ]
      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La mastocitosis sist&#233;mica &#40;MS&#41; es una enfermedad clonal de los progenitores mastoc&#237;ticos de la m&#233;dula &#243;sea&#46; El cuadro cl&#237;nico var&#237;a desde una forma asintom&#225;tica &#40;indolente&#41; hasta una forma altamente agresiva con una supervivencia muy corta &#40;leucemia de mastocitos&#41;&#46; Un 28-34&#37; de los pacientes con MS tiene s&#237;ntomas relacionados con la afecci&#243;n &#243;sea en el momento del diagn&#243;stico y el 16&#37; fracturas&#46; La presentaci&#243;n de MS como fracturas vertebrales cl&#237;nicas en varones j&#243;venes no es frecuente&#46; A continuaci&#243;n describimos un caso de osteoporosis establecida como &#250;nica manifestaci&#243;n de MS&#46;</p></span>"
      ]
    ]
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      0 => array:2 [
        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Carrasco Cubero C&#44; Chamizo Carmona E&#46; Fracturas vertebrales m&#250;ltiples en var&#243;n joven como primera manifestaci&#243;n de una mastocitosis sist&#233;mica&#46; Reumatol Clin&#46; 2017&#59;13&#58;107&#8211;109&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleItalic">Magnetic resonance image of the thoracolumbar spine&#58;</span> a decrease can be seen in the thoracic and lumbar vertebral body height&#46; Thoracic 7 vertebral body is notably diminished in height and presents an alteration in signal intensity&#46; There are similar changes&#44; although less marked&#44; in the upper borders of thoracic 11 and lumbar 2&#46; <span class="elsevierStyleItalic">Lateral radiograph of the thoracic spine&#58;</span> vertebroplasty in thoracic 7&#46;</p>"
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                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Diagnostic criteria &#40;laboratory&#41;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Major&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Presence of mast cell &#40;MC&#41; clusters &#40;&#62;15 mast cells&#41; in the biopsy of bone marrow or other tissues&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Minor&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">&#8226;<span class="elsevierStyleHsp" style=""></span>&#62;25&#37; MC with abnormal morphology in the bone marrow extension<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Expression of CD25 or CD2 antigen detected by flow cytometry<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Serum tryptase &#62;20<span class="elsevierStyleHsp" style=""></span>ng&#47;mL &#40;not valid if there is an associated myeloid hematologic disease&#41;<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Presence of a c-Kit activating mutation in MC of bone marrow or other tissues&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Mastocytosis is considered to be systemic if a mayor and a minor criterion are met&#44; or 3 minor criteria coexist</td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td" title="table-entry  " colspan="2" align="left" valign="top"><span class="elsevierStyleItalic">Severity criteria &#40;clinical&#41;</span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>B manifestations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Indicative of a high tumor burden and expansion of the genetic defect to several myeloid cell lines<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Bone marrow&#47;extracutaneous mastocytosis &#62;30&#37; with serum tryptase &#60;200<span class="elsevierStyleHsp" style=""></span>ng&#47;mL<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Hypercellular bone marrow with loss of lipid material&#44; subtle signs of dysmyelopoiesis in the absence of cytopenia&#44; or the World Health Organization criteria for myelodysplastic or myeloproliferative syndrome&#46;<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Organomegaly&#58; palpable hepatomegaly&#47;splenomegaly or lymphadenopathy &#62;2<span class="elsevierStyleHsp" style=""></span>cm according to images&#44; with no evidence of functional involvement&#46;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>C manifestations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Indicative of organic functional changes owing to the infiltration<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Cytopenia&#58; neutrophils &#60;1000&#47;&#956;L&#44; platelets &#60;100&#44;000&#47;&#956;L&#44; hemoglobin &#60;10<span class="elsevierStyleHsp" style=""></span>g&#47;dL<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Hepatomegaly with ascites and an alteration in liver function<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Palpable splenomegaly with hypersplenism<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Malabsorption syndrome with hypoalbuminemia and weight loss<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Severe osteolytic or osteoporotic lesions with pathological fractures<br>&#8226;<span class="elsevierStyleHsp" style=""></span>Enlargement of diverse organs with critical risk due to mast cell infiltration&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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                0 => array:2 [
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                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
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                          ]
                        ]
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                    ]
                  ]
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                    0 => array:1 [
                      "Revista" => array:5 [
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            1 => array:3 [
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                  "host" => array:1 [
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                      "Revista" => array:6 [
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                            1 => "W&#46; van der Goot"
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Article information
ISSN: 21735743
Original language: English
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