Abstract
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.
MeSH terms
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Anaplastic Lymphoma Kinase
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Bone Marrow / pathology
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Cyclophosphamide / administration & dosage
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Dexamethasone / administration & dosage
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Diagnosis, Differential
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Fatal Outcome
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Humans
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Lymphocytes / pathology
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Lymphoma, Large-Cell, Anaplastic / diagnosis
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Lymphoma, Large-Cell, Anaplastic / drug therapy
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Lymphoma, Large-Cell, Anaplastic / enzymology*
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Lymphoma, Large-Cell, Anaplastic / pathology*
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Lymphoma, Primary Cutaneous Anaplastic Large Cell / diagnosis
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Lymphoma, Primary Cutaneous Anaplastic Large Cell / enzymology
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Lymphoma, Primary Cutaneous Anaplastic Large Cell / pathology
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Male
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Middle Aged
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Receptor Protein-Tyrosine Kinases / analysis*
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Salvage Therapy
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Skin / pathology*
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Skin Neoplasms / enzymology
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Skin Neoplasms / pathology
Substances
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Dexamethasone
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Cyclophosphamide
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ALK protein, human
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Anaplastic Lymphoma Kinase
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Receptor Protein-Tyrosine Kinases