Ann Dermatol.  2016 Jun;28(3):371-374. 10.5021/ad.2016.28.3.371.

CD30-Positive Anaplastic Lymphoma Kinase-Negative Systemic Anaplastic Large-Cell Lymphoma in a 9-Year-Old Boy

• Kim JE
• Oh EH
• Ro YS
• Ko JY

• KMID: 2164646
• DOI: http://doi.org/10.5021/ad.2016.28.3.371

Abstract

Anaplastic large-cell lymphoma (ALCL) is a CD30-positive T-cell/null-cell lymphoma that is clinically classified into either primary cutaneous ALCL or systemic ALCL (S-ALCL) sub-types. Because 90% of childhood S-ALCL cases are anaplastic lymphoma kinase (ALK)-positive, there is a lack of data on ALK-negative S-ALCL cases among pediatric patients. Herein, we report a rare case of ALK-negative S-ALCL in a 9-year-old Korean boy who initially presented with itchy erythematous maculopapules and an erosive nodule on the trunk area. We emphasize the need of high index of suspicion of an underlying malignant disease in the presence of refractory eczematous lesions.

Keyword

Anaplastic lymphoma kinase; Anaplastic large-cell lymphoma; Eczema

MeSH Terms

Child*
Eczema
Humans
Lymphoma*
Lymphoma, Large-Cell, Anaplastic*
Male*
Phosphotransferases
Phosphotransferases

Figure

• 

  Fig. 1 (A) Scattered erythematous maculopapules and an erosive nodule on the trunk (inset: close-up view). (B) Arrows indicate multiple hypermetabolic [18F] fluoro-2-deoxyglucose uptake observed on positron emission tomography.

• 

  Fig. 2 (A) A skin biopsy specimen from the erosive nodule showed epidermal erosion and diffuse dermal cellular infiltration (H&E, ×12.5). (B) The infiltration consisted of large atypical cells with eccentric nuclei admixed with inflammatory cells (H&E, ×200). Immunohistochemical staining of the specimen showed positivity for (C) CD30 with a membranous staining pattern on large cells (×100), as well as negativity for (D) anaplastic lymphoma kinase (×100).

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  Fig. 3 After chemotherapy, tiny papular lesions and a nodular erosive lesion were regressed with a subtle biopsy scar (inset: close-up view).

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