Fatal ALK-negative systemic anaplastic large cell lymphoma presenting with disseminated cutaneous dome-shaped papules and nodules.
Anaplastic large cell lymphoma (ALCL) is classified as a CD30 positive non-Hodgkin lymphoma. Systemic ALCL (S-ALCL) is further subdivided into two subgroups based on anaplastic lymphoma kinase (ALK) expression. In systemic ALCL, positive ALK expression correlates with a favorable prognosis, whereas negative ALK expression correlates with poorer overall survival. By definition, primary cutaneous ALCL (cut-ALCL) is limited to the skin and is uniformly ALK-negative. Cut-ALCL closely resembles LyP with regards to its benign clinical course and CD30 positivity. We describe a unique case of ALK-negative (ALK-) S-ALCL presenting with cutaneous disseminated dome-shaped papules.
Oregon Health and Science University, Portland, Oregon, USA.
Jayne Eleanor
JE
Bird
Justin John
JJ
Leitenberger
Alvin
A
Solomon
Andrew
A
Blauvelt
Sam
S
Hopkins
D000077548:Anaplastic Lymphoma Kinase; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D008214:Lymphocytes / Q000473:pathology; D017728:Lymphoma, Large-Cell, Anaplastic / Q000175:diagnosis / Q000188:drug therapy / Q000201:enzymology* / Q000473:pathology*; D054446:Lymphoma, Primary Cutaneous Anaplastic Large Cell / Q000175:diagnosis / Q000201:enzymology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D020794:Receptor Protein-Tyrosine Kinases / Q000032:analysis*; D016879:Salvage Therapy; D012867:Skin / Q000473:pathology*; D012878:Skin Neoplasms / Q000201:enzymology / Q000473:pathology
D003907:Dexamethasone; D003520:Cyclophosphamide; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases