Acute Leukemia with M3 Morphology Without Cytogenetic Abnormalities Related to Acute Promyelocytic Leukemia: Description of a Refractory Pediatric Case
Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML). APL is characterized by specific genetic abnormality t(15;17), which results in fusion between the promyelocytic leukemia (PML) gene and the retinoic acid receptor-α (RARα). We describe the case of a 4-year-old...
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| Veröffentlicht in: | Clinical leukemia 2009-08, Vol.3 (2), p.E27-E30 |
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| container_title | Clinical leukemia |
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| creator | Rodrigues, Morgani Kutner, José Mauro Ribeiro, Andreza Alice Feitosa Hidal, Luci Tabacow Stape, Adalberto Bacal, Nydia Hamerschlak, Nelson |
| description | Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML). APL is characterized by specific genetic abnormality t(15;17), which results in fusion between the promyelocytic leukemia (PML) gene and the retinoic acid receptor-α (RARα). We describe the case of a 4-year-old boy who was admitted to hospital with severe infection of the oropharynx due to a peritonsillar abscess, along with hepatomegaly and splenomegaly. The initial laboratory tests showed a condition compatible with AML. The cytologic morphology, cytochemistry, and immunophenotyping were compatible with the AML M3 variant but with normal karyotype, fluorescence in situ hybridization and polymerase chain reaction (PCR) negative for t(15;17), and PCR negative for t(11;17). There was resistance to the initial chemotherapy, but the patient experienced an excellent result from nonrelative umbilical cord transplantation. The case represents an atypical situation of AML with promyelocytic characteristics and normal cytogenetics showing a poor prognosis that responded only to bone marrow transplantation. |
| doi_str_mv | 10.3816/CLK.2009.n.012 |
| format | Article |
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APL is characterized by specific genetic abnormality t(15;17), which results in fusion between the promyelocytic leukemia (PML) gene and the retinoic acid receptor-α (RARα). We describe the case of a 4-year-old boy who was admitted to hospital with severe infection of the oropharynx due to a peritonsillar abscess, along with hepatomegaly and splenomegaly. The initial laboratory tests showed a condition compatible with AML. The cytologic morphology, cytochemistry, and immunophenotyping were compatible with the AML M3 variant but with normal karyotype, fluorescence in situ hybridization and polymerase chain reaction (PCR) negative for t(15;17), and PCR negative for t(11;17). There was resistance to the initial chemotherapy, but the patient experienced an excellent result from nonrelative umbilical cord transplantation. The case represents an atypical situation of AML with promyelocytic characteristics and normal cytogenetics showing a poor prognosis that responded only to bone marrow transplantation.</description><identifier>ISSN: 1931-6925</identifier><identifier>EISSN: 1938-0704</identifier><identifier>DOI: 10.3816/CLK.2009.n.012</identifier><language>eng</language><subject>Cord blood stem cell transplantation ; Myeloid leukemia ; Neoplasm drug resistance ; Promyelocytes ; Retinoic acid receptors</subject><ispartof>Clinical leukemia, 2009-08, Vol.3 (2), p.E27-E30</ispartof><rights>2009 Elsevier</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c189t-20726cf3828dc0aaaac997b82bd6b4b2eb41c65bb8c868d61214e50b9fe2ab6e3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids></links><search><creatorcontrib>Rodrigues, Morgani</creatorcontrib><creatorcontrib>Kutner, José Mauro</creatorcontrib><creatorcontrib>Ribeiro, Andreza Alice Feitosa</creatorcontrib><creatorcontrib>Hidal, Luci Tabacow</creatorcontrib><creatorcontrib>Stape, Adalberto</creatorcontrib><creatorcontrib>Bacal, Nydia</creatorcontrib><creatorcontrib>Hamerschlak, Nelson</creatorcontrib><title>Acute Leukemia with M3 Morphology Without Cytogenetic Abnormalities Related to Acute Promyelocytic Leukemia: Description of a Refractory Pediatric Case</title><title>Clinical leukemia</title><description>Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML). APL is characterized by specific genetic abnormality t(15;17), which results in fusion between the promyelocytic leukemia (PML) gene and the retinoic acid receptor-α (RARα). We describe the case of a 4-year-old boy who was admitted to hospital with severe infection of the oropharynx due to a peritonsillar abscess, along with hepatomegaly and splenomegaly. The initial laboratory tests showed a condition compatible with AML. The cytologic morphology, cytochemistry, and immunophenotyping were compatible with the AML M3 variant but with normal karyotype, fluorescence in situ hybridization and polymerase chain reaction (PCR) negative for t(15;17), and PCR negative for t(11;17). There was resistance to the initial chemotherapy, but the patient experienced an excellent result from nonrelative umbilical cord transplantation. The case represents an atypical situation of AML with promyelocytic characteristics and normal cytogenetics showing a poor prognosis that responded only to bone marrow transplantation.</description><subject>Cord blood stem cell transplantation</subject><subject>Myeloid leukemia</subject><subject>Neoplasm drug resistance</subject><subject>Promyelocytes</subject><subject>Retinoic acid receptors</subject><issn>1931-6925</issn><issn>1938-0704</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2009</creationdate><recordtype>article</recordtype><recordid>eNp1kE9P3DAQxaOqSN1Crz37xC2p7Wwcm9sq_YfYFQhR9WjZzmTXkMRb2wHlk_B18bLlyFxmNHrvaeaXZV8JLkpO2LdmfVVQjEUxFpjQD9mCiJLnuMbLj68zyZmg1afscwj3GFclF_Uie16ZKQJaw_QAg1XoycYd2pRo4_x-53q3ndHftHJTRM0c3RZGiNaglR6dH1Rvo4WAbqFXEVoUHTrG3Xg3zNA7Mx_Eb-EX6DsE4-0-Wjci1yGVnJ1XJjo_oxtorYo-6RsV4Cw76VQf4Mv_fpr9-fnjrvmdr69_XTardW4IFzGnuKbMdCWnvDVYpTJC1JpT3TK91BT0khhWac0NZ7xlhJIlVFiLDqjSDMrT7PyYu_fu3wQhysEGA32vRnBTkJSURLC6SsLiKDTeheChk3tvB-VnSbA88JeJvzzwl6NM_JOBHw2Qzn-04GUwFkaT3vRgomydfc_6AlAaj0w</recordid><startdate>20090801</startdate><enddate>20090801</enddate><creator>Rodrigues, Morgani</creator><creator>Kutner, José Mauro</creator><creator>Ribeiro, Andreza Alice Feitosa</creator><creator>Hidal, Luci Tabacow</creator><creator>Stape, Adalberto</creator><creator>Bacal, Nydia</creator><creator>Hamerschlak, Nelson</creator><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope></search><sort><creationdate>20090801</creationdate><title>Acute Leukemia with M3 Morphology Without Cytogenetic Abnormalities Related to Acute Promyelocytic Leukemia: Description of a Refractory Pediatric Case</title><author>Rodrigues, Morgani ; Kutner, José Mauro ; Ribeiro, Andreza Alice Feitosa ; Hidal, Luci Tabacow ; Stape, Adalberto ; Bacal, Nydia ; Hamerschlak, Nelson</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c189t-20726cf3828dc0aaaac997b82bd6b4b2eb41c65bb8c868d61214e50b9fe2ab6e3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2009</creationdate><topic>Cord blood stem cell transplantation</topic><topic>Myeloid leukemia</topic><topic>Neoplasm drug resistance</topic><topic>Promyelocytes</topic><topic>Retinoic acid receptors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Rodrigues, Morgani</creatorcontrib><creatorcontrib>Kutner, José Mauro</creatorcontrib><creatorcontrib>Ribeiro, Andreza Alice Feitosa</creatorcontrib><creatorcontrib>Hidal, Luci Tabacow</creatorcontrib><creatorcontrib>Stape, Adalberto</creatorcontrib><creatorcontrib>Bacal, Nydia</creatorcontrib><creatorcontrib>Hamerschlak, Nelson</creatorcontrib><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><jtitle>Clinical leukemia</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Rodrigues, Morgani</au><au>Kutner, José Mauro</au><au>Ribeiro, Andreza Alice Feitosa</au><au>Hidal, Luci Tabacow</au><au>Stape, Adalberto</au><au>Bacal, Nydia</au><au>Hamerschlak, Nelson</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Acute Leukemia with M3 Morphology Without Cytogenetic Abnormalities Related to Acute Promyelocytic Leukemia: Description of a Refractory Pediatric Case</atitle><jtitle>Clinical leukemia</jtitle><date>2009-08-01</date><risdate>2009</risdate><volume>3</volume><issue>2</issue><spage>E27</spage><epage>E30</epage><pages>E27-E30</pages><issn>1931-6925</issn><eissn>1938-0704</eissn><abstract>Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML). APL is characterized by specific genetic abnormality t(15;17), which results in fusion between the promyelocytic leukemia (PML) gene and the retinoic acid receptor-α (RARα). We describe the case of a 4-year-old boy who was admitted to hospital with severe infection of the oropharynx due to a peritonsillar abscess, along with hepatomegaly and splenomegaly. The initial laboratory tests showed a condition compatible with AML. The cytologic morphology, cytochemistry, and immunophenotyping were compatible with the AML M3 variant but with normal karyotype, fluorescence in situ hybridization and polymerase chain reaction (PCR) negative for t(15;17), and PCR negative for t(11;17). There was resistance to the initial chemotherapy, but the patient experienced an excellent result from nonrelative umbilical cord transplantation. The case represents an atypical situation of AML with promyelocytic characteristics and normal cytogenetics showing a poor prognosis that responded only to bone marrow transplantation.</abstract><doi>10.3816/CLK.2009.n.012</doi></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 1931-6925 |
| ispartof | Clinical leukemia, 2009-08, Vol.3 (2), p.E27-E30 |
| issn | 1931-6925 1938-0704 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_21319675 |
| source | Alma/SFX Local Collection |
| subjects | Cord blood stem cell transplantation Myeloid leukemia Neoplasm drug resistance Promyelocytes Retinoic acid receptors |
| title | Acute Leukemia with M3 Morphology Without Cytogenetic Abnormalities Related to Acute Promyelocytic Leukemia: Description of a Refractory Pediatric Case |
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