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Lymphomatoid Papulosis with DUSP22-IRF4 Gene Rearrangement

MedGen UID:
1612398
•Concept ID:
C4528215
•
Neoplastic Process
Synonyms: Lymphomatoid Papulosis with Chromosome 6p25.3 Rearrangement; LyP with Chromosome 6p25 Rearrangement; LyP with Chromosome 6p25.3 Rearrangement; LyP with DUSP22-IRF4 Gene Rearrangement

Definition

A variant of lymphomatoid papulosis associated with chromosomal rearrangements involving the DUSP22-IRF4 locus at 6p25.3. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLymphomatoid Papulosis with DUSP22-IRF4 Gene Rearrangement

Recent clinical studies

Etiology

DUSP22-IRF4 Rearranged CD30-Positive Primary Cutaneous Lymphoproliferative Disorder With Gamma/Delta Phenotype.
Fattah YH, Crasto D, Liu SS, Linhares Y, Kerdel F, Hanly A, Karai LJ
Am J Dermatopathol 2023 Dec 1;45(12):831-834. doi: 10.1097/DAD.0000000000002573. PMID: 37883980
Primary cutaneous anaplastic large-cell lymphoma with 6p25.3 rearrangement exhibits a biphasic histopathologic pattern: Two case reports and literature review.
Xue YN, Wang Z, Sun JF, Chen H
J Cutan Pathol 2021 Dec;48(12):1463-1470. Epub 2021 Jul 21 doi: 10.1111/cup.14089. PMID: 34164830

Diagnosis

DUSP22-IRF4 Rearranged CD30-Positive Primary Cutaneous Lymphoproliferative Disorder With Gamma/Delta Phenotype.
Fattah YH, Crasto D, Liu SS, Linhares Y, Kerdel F, Hanly A, Karai LJ
Am J Dermatopathol 2023 Dec 1;45(12):831-834. doi: 10.1097/DAD.0000000000002573. PMID: 37883980
Chromosomal rearrangements of 6p25.3 define a new subtype of lymphomatoid papulosis.
Karai LJ, Kadin ME, Hsi ED, Sluzevich JC, Ketterling RP, Knudson RA, Feldman AL
Am J Surg Pathol 2013 Aug;37(8):1173-81. doi: 10.1097/PAS.0b013e318282d01e. PMID: 23648461
Mucosal CD30-positive T-cell lymphoproliferations of the head and neck show a clinicopathologic spectrum similar to cutaneous CD30-positive T-cell lymphoproliferative disorders.
Sciallis AP, Law ME, Inwards DJ, McClure RF, Macon WR, Kurtin PJ, Dogan A, Feldman AL
Mod Pathol 2012 Jul;25(7):983-92. Epub 2012 Mar 2 doi: 10.1038/modpathol.2012.38. PMID: 22388754

Prognosis

Primary cutaneous anaplastic large-cell lymphoma with 6p25.3 rearrangement exhibits a biphasic histopathologic pattern: Two case reports and literature review.
Xue YN, Wang Z, Sun JF, Chen H
J Cutan Pathol 2021 Dec;48(12):1463-1470. Epub 2021 Jul 21 doi: 10.1111/cup.14089. PMID: 34164830
Chromosomal rearrangements of 6p25.3 define a new subtype of lymphomatoid papulosis.
Karai LJ, Kadin ME, Hsi ED, Sluzevich JC, Ketterling RP, Knudson RA, Feldman AL
Am J Surg Pathol 2013 Aug;37(8):1173-81. doi: 10.1097/PAS.0b013e318282d01e. PMID: 23648461
Mucosal CD30-positive T-cell lymphoproliferations of the head and neck show a clinicopathologic spectrum similar to cutaneous CD30-positive T-cell lymphoproliferative disorders.
Sciallis AP, Law ME, Inwards DJ, McClure RF, Macon WR, Kurtin PJ, Dogan A, Feldman AL
Mod Pathol 2012 Jul;25(7):983-92. Epub 2012 Mar 2 doi: 10.1038/modpathol.2012.38. PMID: 22388754

Clinical prediction guides

Primary cutaneous anaplastic large-cell lymphoma with 6p25.3 rearrangement exhibits a biphasic histopathologic pattern: Two case reports and literature review.
Xue YN, Wang Z, Sun JF, Chen H
J Cutan Pathol 2021 Dec;48(12):1463-1470. Epub 2021 Jul 21 doi: 10.1111/cup.14089. PMID: 34164830
Chromosomal rearrangements of 6p25.3 define a new subtype of lymphomatoid papulosis.
Karai LJ, Kadin ME, Hsi ED, Sluzevich JC, Ketterling RP, Knudson RA, Feldman AL
Am J Surg Pathol 2013 Aug;37(8):1173-81. doi: 10.1097/PAS.0b013e318282d01e. PMID: 23648461

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