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Primary cutaneous anaplastic large cell lymphoma
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Primary cutaneous anaplastic large cell lymphoma

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Contributors: Vivian Wong MD, PhD, Susan Burgin MD
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Synopsis

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare form of cutaneous T-cell lymphoma characterized clinically by a solitary nodule or tumor that ulcerates, and histopathologically by CD30+ T-cell lymphoproliferation.

This condition typically presents during late adulthood with a median age of 60 at the time of diagnosis. Males are more commonly affected. It demonstrates a chronic, indolent, intermittently recurring clinical course. C-ALCL and lymphomatoid papulosis (LYP) constitute a spectrum of primary cutaneous CD30+ lymphoproliferative disorders, with C-ALCL at one end, the even more indolent LYP at the other, and borderline cases in between.

Overall, prognosis is excellent with a 5-year survival rate above 90% for patients with involvement limited to the skin. Multifocal involvement occurs in around one-fifth of cases. Extensive limb disease, with multifocal lesions on one limb or contiguous body regions, may be associated with a poorer prognosis and thus may require more aggressive treatment. Disease on the head and neck may have a worse prognosis as well. Approximately 10% of individuals will experience extracutaneous involvement (most often to the regional lymph node).

Related topic: Non-Hodgkin lymphoma

Codes

ICD10CM:
C86.6 – Primary cutaneous CD30-positive T-cell proliferations

SNOMEDCT:
397352006 – Primary cutaneous anaplastic large cell lymphoma

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Mycosis fungoides (MF) with large cell transformation – Transformed MF may present with large CD30+ T-cells. While histopathological and immunochemical findings may be similar, clinical features and disease outcome are helpful for distinguishing the two entities. Transformed MF typically involves the trunk with multifocal lesions, is more common in patients older than age 60, and exhibits a more aggressive course with early progression and no spontaneous regression.
  • Systemic anaplastic large cell lymphoma with cutaneous involvement
  • Lymphomatoid papulosis
  • B-cell lymphoma
  • Leukemia cutis
  • Pseudolymphoma
  • Lichen planus
  • Sarcoidosis

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated: 10/19/2017
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Primary cutaneous anaplastic large cell lymphoma
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Primary cutaneous anaplastic large cell lymphoma : Arm, Leg, Smooth nodule, Smooth plaque, Single skin lesion
Copyright © 2018 VisualDx®. All rights reserved.