[ATIC-ALK-positive anaplastic large cell lymphoma: a case report and review of the literature].
We report a 10-year-old girl with ATIC-anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL). She presented with inguinal, axillary, and paraaortic lymph node swellings that showed spontaneous regression over a 3-month period, followed by recurrence after an interval of 8 months. Radiological and clinical findings indicated Ann Arbor stage IIIA. Pathological findings showed that staining of ALK was restricted to the cytoplasm of ALCL cells. ATIC-ALK chimeric transcripts were detected by reverse transcriptase polymerase chain reaction. The patient was assigned to the standard risk group proposed by the international multicenter study for pediatric ALCL, ALCL99. The patient responded well to the treatment and remained in complete remission for more than 26 months. To date, 7 genes have been identified as a fusion partner of ALK, with the highest frequency in nucleophosmin (NPM). Little is known about the clinical implications of subtypes of ALCL harboring each of the 7 fusion genes, especially those of variant fusion genes other than NPM-ALK. In this paper, we review 9 patients with ATIC-ALK-positive ALCL in the literature in addition to discussing our patient. In eight of these 10 cases, disease occurred within the first three decades. Five of 6 cases that were followed continuously remained in complete remission.
Department of Pediatrics, Nishi-Kobe Medical Center.
Kousaku
K
Matsubara
Takayuki
T
Tanaka
Tomohiko
T
Taki
Atsuko
A
Nakagawa
Hiroyuki
H
Nigami
Akihiro
A
Tamura
Takashi
T
Fukaya
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D002648:Child; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D017728:Lymphoma, Large-Cell, Anaplastic / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy*; D015514:Oncogene Proteins, Fusion / Q000032:analysis* / Q000235:genetics; D012074:Remission Induction; D016896:Treatment Outcome
C429803:ATIC-ALK fusion protein, human; D015514:Oncogene Proteins, Fusion