ALK+ histiocytosis : a novel type of systemic histiocytic proliferative disorder of early infancy
Identifieur interne : 009852 ( Main/Merge ); précédent : 009851; suivant : 009853ALK+ histiocytosis : a novel type of systemic histiocytic proliferative disorder of early infancy
Auteurs : John K. C. Chan [Hong Kong] ; Laurence Lamant [France] ; Elizabeth Algar [Australie] ; Georges Delsol [France] ; William Y. W. Tsang [Hong Kong] ; King C. Lee [Hong Kong] ; Karin Tiedemann [Australie] ; Chung W. Chow [Australie]Source :
- Blood [ 0006-4971 ] ; 2008.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Enfant.
English descriptors
Abstract
We report 3 cases of a previously uncharacterized form of histiocytosis presenting in early infancy and showing ALK immunoreactivity. The patients presented with pallor, massive hepatosplenomegaly, anemia, and thrombocytopenia. Liver biopsy showed infiltration of the sinusoids by large histiocytes with markedly folded nuclei, fine chromatin, small nucleoli, and voluminous lightly eosinophilic cytoplasm that sometimes was vacuolated or contained phagocytosed blood cells. One patient developed cutaneous infiltrates that morphologically resembled juvenile xanthogranuloma. The histiocytes were immunoreactive for histiocytic markers (CD68, CD163, lysozyme), S100 protein, ALK (membranous and cytoplasmic pattern), and dendritic cell markers (fascin, factor Xllla), but not CD1a and langerin. One case successfully analyzed by molecular techniques revealed TPM3-ALK fusion. Thus the spectrum of diseases exhibiting ALK translocation should be expanded to include ALK+ histiocytosis. The disease in the 3 patients (2 having been given chemotherapy) resolved slowly over many months.
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Pascal:09-0005466Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">ALK<sup>+</sup>
histiocytosis : a novel type of systemic histiocytic proliferative disorder of early infancy</title>
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<author><name sortKey="Chow, Chung W" sort="Chow, Chung W" uniqKey="Chow C" first="Chung W." last="Chow">Chung W. Chow</name>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">ALK<sup>+</sup>
histiocytosis : a novel type of systemic histiocytic proliferative disorder of early infancy</title>
<author><name sortKey="Chan, John K C" sort="Chan, John K C" uniqKey="Chan J" first="John K. C." last="Chan">John K. C. Chan</name>
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<author><name sortKey="Lamant, Laurence" sort="Lamant, Laurence" uniqKey="Lamant L" first="Laurence" last="Lamant">Laurence Lamant</name>
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<s2>Toulouse</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>4 aut.</sZ>
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<author><name sortKey="Algar, Elizabeth" sort="Algar, Elizabeth" uniqKey="Algar E" first="Elizabeth" last="Algar">Elizabeth Algar</name>
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<author><name sortKey="Delsol, Georges" sort="Delsol, Georges" uniqKey="Delsol G" first="Georges" last="Delsol">Georges Delsol</name>
<affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Inserm, U563, Centre de Physiopathologie de Toulouse Purpan, and Université Toulouse III Paul Sabatier</s1>
<s2>Toulouse</s2>
<s3>FRA</s3>
<sZ>2 aut.</sZ>
<sZ>4 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><region type="region">Occitanie (région administrative)</region>
<region type="old region">Midi-Pyrénées</region>
<settlement type="city">Toulouse</settlement>
</placeName>
</affiliation>
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<author><name sortKey="Tsang, William Y W" sort="Tsang, William Y W" uniqKey="Tsang W" first="William Y. W." last="Tsang">William Y. W. Tsang</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Pathology, Queen Elizabeth Hospital</s1>
<s2>Kowloon</s2>
<s3>HKG</s3>
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<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
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<country>Hong Kong</country>
<wicri:noRegion>Kowloon</wicri:noRegion>
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<author><name sortKey="Lee, King C" sort="Lee, King C" uniqKey="Lee K" first="King C." last="Lee">King C. Lee</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Pathology, Queen Elizabeth Hospital</s1>
<s2>Kowloon</s2>
<s3>HKG</s3>
<sZ>1 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
</inist:fA14>
<country>Hong Kong</country>
<wicri:noRegion>Kowloon</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Tiedemann, Karin" sort="Tiedemann, Karin" uniqKey="Tiedemann K" first="Karin" last="Tiedemann">Karin Tiedemann</name>
<affiliation wicri:level="3"><inist:fA14 i1="04"><s1>Children's Cancer Center, Royal Children's Hospital</s1>
<s2>Melbourne</s2>
<s3>AUS</s3>
<sZ>3 aut.</sZ>
<sZ>7 aut.</sZ>
</inist:fA14>
<country>Australie</country>
<placeName><settlement type="city">Melbourne</settlement>
<region type="état">Victoria (État)</region>
</placeName>
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</author>
<author><name sortKey="Chow, Chung W" sort="Chow, Chung W" uniqKey="Chow C" first="Chung W." last="Chow">Chung W. Chow</name>
<affiliation wicri:level="4"><inist:fA14 i1="03"><s1>Department of Pediatrics, University of Melbourne</s1>
<s3>AUS</s3>
<sZ>3 aut.</sZ>
<sZ>8 aut.</sZ>
</inist:fA14>
<country>Australie</country>
<placeName><settlement type="city">Melbourne</settlement>
<region type="état">Victoria (État)</region>
</placeName>
<orgName type="university">Université de Melbourne</orgName>
</affiliation>
<affiliation wicri:level="3"><inist:fA14 i1="05"><s1>Department of Anatomical Pathology, Royal Children's Hospital</s1>
<s2>Melbourne</s2>
<s3>AUS</s3>
<sZ>8 aut.</sZ>
</inist:fA14>
<country>Australie</country>
<placeName><settlement type="city">Melbourne</settlement>
<region type="état">Victoria (État)</region>
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<series><title level="j" type="main">Blood</title>
<title level="j" type="abbreviated">Blood</title>
<idno type="ISSN">0006-4971</idno>
<imprint><date when="2008">2008</date>
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</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Autoimmune disease</term>
<term>Child</term>
<term>Chromosome translocation</term>
<term>Histiocytosis</term>
<term>Infant</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Histiocytose</term>
<term>Maladie autoimmune</term>
<term>Translocation chromosomique</term>
<term>Enfant</term>
<term>Nourrisson</term>
<term>Gène ALK</term>
</keywords>
<keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Enfant</term>
</keywords>
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<front><div type="abstract" xml:lang="en">We report 3 cases of a previously uncharacterized form of histiocytosis presenting in early infancy and showing ALK immunoreactivity. The patients presented with pallor, massive hepatosplenomegaly, anemia, and thrombocytopenia. Liver biopsy showed infiltration of the sinusoids by large histiocytes with markedly folded nuclei, fine chromatin, small nucleoli, and voluminous lightly eosinophilic cytoplasm that sometimes was vacuolated or contained phagocytosed blood cells. One patient developed cutaneous infiltrates that morphologically resembled juvenile xanthogranuloma. The histiocytes were immunoreactive for histiocytic markers (CD68, CD163, lysozyme), S100 protein, ALK (membranous and cytoplasmic pattern), and dendritic cell markers (fascin, factor Xllla), but not CD1a and langerin. One case successfully analyzed by molecular techniques revealed TPM3-ALK fusion. Thus the spectrum of diseases exhibiting ALK translocation should be expanded to include ALK<sup>+</sup>
histiocytosis. The disease in the 3 patients (2 having been given chemotherapy) resolved slowly over many months.</div>
</front>
</TEI>
<affiliations><list><country><li>Australie</li>
<li>France</li>
<li>Hong Kong</li>
</country>
<region><li>Midi-Pyrénées</li>
<li>Occitanie (région administrative)</li>
<li>Victoria (État)</li>
</region>
<settlement><li>Melbourne</li>
<li>Toulouse</li>
</settlement>
<orgName><li>Université de Melbourne</li>
</orgName>
</list>
<tree><country name="Hong Kong"><noRegion><name sortKey="Chan, John K C" sort="Chan, John K C" uniqKey="Chan J" first="John K. C." last="Chan">John K. C. Chan</name>
</noRegion>
<name sortKey="Lee, King C" sort="Lee, King C" uniqKey="Lee K" first="King C." last="Lee">King C. Lee</name>
<name sortKey="Tsang, William Y W" sort="Tsang, William Y W" uniqKey="Tsang W" first="William Y. W." last="Tsang">William Y. W. Tsang</name>
</country>
<country name="France"><region name="Occitanie (région administrative)"><name sortKey="Lamant, Laurence" sort="Lamant, Laurence" uniqKey="Lamant L" first="Laurence" last="Lamant">Laurence Lamant</name>
</region>
<name sortKey="Delsol, Georges" sort="Delsol, Georges" uniqKey="Delsol G" first="Georges" last="Delsol">Georges Delsol</name>
</country>
<country name="Australie"><region name="Victoria (État)"><name sortKey="Algar, Elizabeth" sort="Algar, Elizabeth" uniqKey="Algar E" first="Elizabeth" last="Algar">Elizabeth Algar</name>
</region>
<name sortKey="Algar, Elizabeth" sort="Algar, Elizabeth" uniqKey="Algar E" first="Elizabeth" last="Algar">Elizabeth Algar</name>
<name sortKey="Chow, Chung W" sort="Chow, Chung W" uniqKey="Chow C" first="Chung W." last="Chow">Chung W. Chow</name>
<name sortKey="Chow, Chung W" sort="Chow, Chung W" uniqKey="Chow C" first="Chung W." last="Chow">Chung W. Chow</name>
<name sortKey="Tiedemann, Karin" sort="Tiedemann, Karin" uniqKey="Tiedemann K" first="Karin" last="Tiedemann">Karin Tiedemann</name>
</country>
</tree>
</affiliations>
</record>
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