A case of primary cutaneous anaplastic large cell lymphoma with variant anaplastic lymphoma kinase translocation.

Abstract	Anaplastic lymphoma kinase (ALK) is frequently observed in systemic anaplastic large cell lymphoma (ALCL), mostly in childhood or adolescence, but only rarely in primary cutaneous cases. We report a case of primary cutaneous ALCL (pcALCL) with cytoplasmic ALK expression. A 54-year-old woman with an ulcerative tumour on her forehead was admitted to our hospital. Histologically, there was an infiltrate consisting of atypical large lymphocytes and small lymphocytes in the dermis and fat tissue. Southern blot analysis showed monoclonal T-cell receptor Cbeta1 gene rearrangement. Atypical large lymphocytes were positive for CD30, CD4 and CD25, and negative for CD3 and CD79a. They were also positive for ALK only in the cytoplasm, and neurophosmin (NPM)-ALK fusion transcript was not detected by reverse transcription-polymerase chain reaction. This suggested that the translocation partner of the ALK gene in this case was different from NPM (variant translocation). The tumour on the forehead resolved in 1 month after biopsy. Nodular lesions recurred on the right knee, and were histologically identical with the forehead lesion. Our case suggests the existence of a subgroup with variant ALK translocation in pcALCL; examining NPM-ALK translocation in each case with ALK expression should be useful to characterize the disease further.
Authors	K Sasaki, M Sugaya, H Fujita, K Takeuchi, H Torii, A Asahina, K Tamaki
Journal	The British journal of dermatology (Br J Dermatol) Vol. 150 Issue 6 Pg. 1202-7 (Jun 2004) ISSN: 0007-0963 [Print] England
PMID	15214912 (Publication Type: Case Reports, Journal Article)
Chemical References	CD4 Antigens Ki-1 Antigen Receptors, Interleukin-2 ALK protein, human Anaplastic Lymphoma Kinase Protein-Tyrosine Kinases Receptor Protein-Tyrosine Kinases
Topics	Anaplastic Lymphoma Kinase CD4 Antigens (analysis) Cytoplasm (enzymology) Facial Neoplasms (enzymology, genetics, immunology) Female Humans Immunophenotyping Ki-1 Antigen (analysis) Knee Leg Dermatoses (enzymology, genetics, immunology) Lymphoma, Large B-Cell, Diffuse (enzymology, genetics, immunology) Middle Aged Protein-Tyrosine Kinases (analysis, genetics) Receptor Protein-Tyrosine Kinases Receptors, Interleukin-2 (analysis) Remission, Spontaneous Skin Neoplasms (enzymology, genetics, immunology) Translocation, Genetic

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