<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1409-0015</journal-id>
<journal-title><![CDATA[Medicina Legal de Costa Rica]]></journal-title>
<abbrev-journal-title><![CDATA[Med. leg. Costa Rica]]></abbrev-journal-title>
<issn>1409-0015</issn>
<publisher>
<publisher-name><![CDATA[Asociación Costarricense de Medicina Forense]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1409-00152017000100342</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Hemoglobinuria paroxística nocturna]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[García Rojas]]></surname>
<given-names><![CDATA[Víctor Andrés]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sánchez Vargas]]></surname>
<given-names><![CDATA[Xinia Alexandra]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Mora Hernández]]></surname>
<given-names><![CDATA[Gustavo Adolfo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,CCSS Hospital México Sistema de Estudios de Posgrado Universidad de Costa Rica]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>03</month>
<year>2017</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>03</month>
<year>2017</year>
</pub-date>
<volume>34</volume>
<numero>1</numero>
<fpage>342</fpage>
<lpage>352</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.sa.cr/scielo.php?script=sci_arttext&amp;pid=S1409-00152017000100342&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.sa.cr/scielo.php?script=sci_abstract&amp;pid=S1409-00152017000100342&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.sa.cr/scielo.php?script=sci_pdf&amp;pid=S1409-00152017000100342&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen La hemoglobinuria paroxística nocturna es una anemia hemolítica crónica, adquirida, poco común, que afecta con igual frecuencia ambos sexos. Se manifiesta a cualquier edad y con mayor incidencia en países del sudeste asiático. Es el resultado de la expansión clonal no maligna de células progenitoras hematopoyéticas. Se caracteriza por anemia hemolítica intravascular, tendencia a la trombosis y un componente variable de insuficiencia medular. Se asocia a otras patologías hematológicas como anemia aplásica y síndrome mielodisplásico. La citometría de flujo es el método de elección para diagnóstico. El eculizumab y el trasplante de médula ósea alogénico son las únicas terapias efectivas.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract: Paroxysmal nocturnal hemoglobinuria is a rare acquired chronic hemolytic anemia, which affects both sexes with equal frequency. It occurs at any age and more frequently in Southeast Asian countries. It is the result of non malignant clonal expansion of hematopoietic progenitor cells. It is characterized by intravascular hemolytic anemia, recurrent thrombosis and a variable component of bone marrow failure. It is associated with other hematologic disorders such as aplastic anemia and myelodysplastic syndrome. Flow cytometry is the method of choice for diagnosis. Eculizumab and allogeneic bone marrow transplantation is the only effective therapies.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Hemoglobinuria paroxística nocturna]]></kwd>
<kwd lng="es"><![CDATA[anemia hemolítica microangiopática]]></kwd>
<kwd lng="en"><![CDATA[Paroxysmal nocturnal hemoglobinuria]]></kwd>
<kwd lng="en"><![CDATA[Microangiopathic hemolytic anemia]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Arruda]]></surname>
<given-names><![CDATA[M.]]></given-names>
</name>
<name>
<surname><![CDATA[Rodrigues]]></surname>
<given-names><![CDATA[C.]]></given-names>
</name>
<name>
<surname><![CDATA[Yamamoto]]></surname>
<given-names><![CDATA[M.]]></given-names>
</name>
<name>
<surname><![CDATA[Figueiredo]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hemoglobinúria paroxística noturna: da fisiopatologiaa o tratamento]]></article-title>
<source><![CDATA[Rev Assoc Med Bras]]></source>
<year>2010</year>
<volume>56</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>214-21</page-range></nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brodsky]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Narrative review: Paroxysmal nocturnal hemoglobinuria: the physiology of complementrelated hemolytic anemia]]></article-title>
<source><![CDATA[Ann. Intern. Med]]></source>
<year>2008</year>
<volume>148</volume>
<page-range>587-95</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brodsky]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Paroxysmal nocturnal hemoglobinuria]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2014</year>
<volume>124</volume>
<page-range>2804</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brodsky]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[How I treat paroxysmal nocturnal hemoglobinuria]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2009</year>
<volume>113</volume>
<page-range>6522-7</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hernández]]></surname>
<given-names><![CDATA[P.]]></given-names>
</name>
<name>
<surname><![CDATA[Almeida]]></surname>
<given-names><![CDATA[J.]]></given-names>
</name>
<name>
<surname><![CDATA[Orfao]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hemoglobinuria paroxística nocturna]]></article-title>
<source><![CDATA[Med Clin (Barc)]]></source>
<year>2008</year>
<volume>131</volume>
<page-range>617-30</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hernández]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Almeida]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Acevedo]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Sánchez]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Alberca]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Vidriales]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Detailed immunophenotypic characterization of different major and minor subsets of peripheral blood cells in patients with paroxysmal nocturnal hemoglobinuria]]></article-title>
<source><![CDATA[Transfusion]]></source>
<year>2008</year>
<volume>48</volume>
<page-range>1403-14</page-range></nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hill]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Kelly]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Hillmen]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Thrombosis in paroxysmal nocturnal hemoglobinuria]]></article-title>
<source><![CDATA[Blood]]></source>
<year>2013</year>
<volume>121</volume>
<numero>25</numero>
<issue>25</issue>
<page-range>4985-96</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hillmen]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Muus]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Röth]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Long-term safety and efficacy of sustain edeculizumab treatment in patients with paroxismal nocturnal haemoglobinuria]]></article-title>
<source><![CDATA[Br J Haematol]]></source>
<year>2013</year>
<volume>162</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>62-73</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hillmen]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Young]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Schubert]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Brodsky]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Socié]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Muus]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria]]></article-title>
<source><![CDATA[N Engl J Med]]></source>
<year>2006</year>
<volume>355</volume>
<page-range>1233-43</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hoekstra]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Paroxysmal nocturnal hemoglobinuria in Budd-Chiari Syndrome: Findings from a cohort study]]></article-title>
<source><![CDATA[J Hepatol]]></source>
<year>2009</year>
<month>,</month>
<volume>51</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>696-706</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Moyo]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Mukhina]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Garrett]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Brodsky]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Natural history of paroxismal nocturnal haemoglobinuria using modern diagnostic assays]]></article-title>
<source><![CDATA[Br J Haematol]]></source>
<year>2004</year>
<volume>126</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>133-8</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Rother]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Bell]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Hillmen]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Gladwin]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease]]></article-title>
<source><![CDATA[JAMA]]></source>
<year>2005</year>
<volume>293</volume>
<numero>13</numero>
<issue>13</issue>
<page-range>1653-62</page-range></nlm-citation>
</ref>
<ref id="B13">
<label>13</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sánchez]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hemoglobinuria paroxística nocturna. Novedades patogénicas y terapéuticas]]></article-title>
<source><![CDATA[An. Med. Interna (Madrid)]]></source>
<year>2001</year>
<volume>18</volume>
<numero>8</numero>
<issue>8</issue>
<page-range>435-9</page-range></nlm-citation>
</ref>
<ref id="B14">
<label>14</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Urbano]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Diagnóstico y tratamiento de la hemoglobinuria paroxística nocturna]]></article-title>
<source><![CDATA[MedClin (Barc)]]></source>
<year>2011</year>
<volume>136</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>121-7</page-range></nlm-citation>
</ref>
<ref id="B15">
<label>15</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Van Bijnen]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Østerud]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Barteling]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Verbeek]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Willemsen]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Van Heerde]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Muus]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Alterations in markers of coagulation and fibrinolysis in patients with Paroxysmal Nocturnal Hemoglobinuria before and during treatment with eculizumab]]></article-title>
<source><![CDATA[Thromb Res]]></source>
<year>2015</year>
<month>,</month>
<volume>136</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>274-81</page-range></nlm-citation>
</ref>
<ref id="B16">
<label>16</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Vega]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Estudio ultraestructural de las plaquetas de pacientes con hemoglobinuria paroxística nocturna]]></article-title>
<source><![CDATA[Rev Invest Clin]]></source>
<year>2006</year>
<volume>58</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>475-86</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
