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Cutaneous Lymphoproliferative Disorders

What’s New in the Revised 4th Edition of the World Health Organization (WHO) Classification of Lymphoid Neoplasms

Sundram, Uma, MD, PhD

Advances in Anatomic Pathology: March 2019 - Volume 26 - Issue 2 - p 93–113
doi: 10.1097/PAP.0000000000000208
Review Articles

Cutaneous lymphoproliferative disorders remain a challenging aspect of dermatopathology, in part due to the rarity of the entities and extreme variability in clinical outcomes. Although many of the entities remain unchanged, the approach to some of them has changed in the new 2016 classification scheme of the World Health Organization. Chief among these are Epstein-Barr virus–associated lymphoproliferative disorders such as Epstein-Barr virus–associated mucocutaneous ulcer and hydroa vacciniforme-like lymphoproliferative disorder, primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma, primary cutaneous acral CD8+ T-cell lymphoma, primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder, and breast implant–associated anaplastic large cell lymphoma. In addition, translocations and gene rearrangements such as those involving the 6p25.3 locus have started to inform diagnosis and classification of anaplastic large cell lymphoma and lymphomatoid papulosis. In this review, we will examine what is new in the diagnostic toolbox of cutaneous lymphoproliferative disorders.

Department of Anatomic Pathology, Oakland University William Beaumont School of Medicine and Beaumont Health Systems, Royal Oak, MI

The author has no funding or conflicts of interest to disclose.

Reprints: Uma Sundram, MD, PhD, Department of Anatomic Pathology, Beaumont Health Systems-Royal Oak, 3601 West 13 Mile Road, Royal Oak, MI 48073 (e-mail:

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