title: Isolated cutaneous anaplastic large cell lymphoma progressing to severe systemic disease with myocardial involvement and central nervous system infiltration.
abstract: Anaplastic large cell lymphoma (ALCL) is a rare tumor comprising around 10-15% of childhood lymphomas. We describe the case of a female who initially presented with localized skin disease associated with an insect bite. However, she subsequently relapsed with widespread systemic ALK-positive ALCL that included lymphoma deposits in the myocardium, a very rare manifestation. Her disease responded well to chemotherapy but she later developed a fatal relapse in the CNS. We also present data on an immune response to ALK, demonstrating a fluctuation in the levels of circulating antibodies to ALK corresponding to the different phases of her illness.
authors.affiliation: Department of Paediatric Oncology, Royal Liverpool Children's NHS Trust, Liverpool, UK.
authors.forename: Yi Fan
authors.initials: YF
authors.lastname: Rannan-Eliya
authors.forename: Karen
authors.initials: K
authors.lastname: Pulford
authors.forename: Rob
authors.initials: R
authors.lastname: Johnson
authors.forename: Ian
authors.initials: I
authors.lastname: Peart
authors.forename: George
authors.initials: G
authors.lastname: Kokai
authors.forename: Colin
authors.initials: C
authors.lastname: Baillie
authors.forename: Kamel
authors.initials: K
authors.lastname: Ait-Tahar
authors.forename: Barry
authors.initials: B
authors.lastname: Pizer
mesh_terms: D000293:Adolescent; D000077548:Anaplastic Lymphoma Kinase; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016543:Central Nervous System Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D018450:Disease Progression; D017548:Echocardiography, Transesophageal; D017809:Fatal Outcome; D005260:Female; D006338:Heart Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D006801:Humans; D017728:Lymphoma, Large-Cell, Anaplastic / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D009206:Myocardium; D015514:Oncogene Proteins, Fusion / Q000096:biosynthesis / Q000235:genetics / Q000276:immunology; D011505:Protein-Tyrosine Kinases / Q000096:biosynthesis / Q000235:genetics / Q000276:immunology; D020794:Receptor Protein-Tyrosine Kinases; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery
chemical_list: D015514:Oncogene Proteins, Fusion; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases