Ann Dermatol.  2010 May;22(2):186-190. 10.5021/ad.2010.22.2.186.

CD4-/CD56+/CD123+ Hematodermic Neoplasm Showing Early Liver Metastasis

• Choi KW
• Lee KY
• Lee YK
• Ku BS
• Kim HS
• Kim YH
• Kim KH

• KMID: 2265390
• DOI: http://doi.org/10.5021/ad.2010.22.2.186

Abstract

Hematodermic neoplasm (HN) is a clinically aggressive neoplasm with a high incidence of cutaneous involvement and a risk of leukemic dissemination. In the recent WHO-EORTC classification, the term blastic natural killer cell lymphoma has been replaced with CD4+/CD56+ HN because of its derivation from a plasmacytoid dendritic cell precursor. Cases of HN that completely lack CD4 or CD56 expression, therefore represents a diagnostic problem. A 68-year-old Korean male was diagnosed with CD4-/CD56+ HN and treated with hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) at initial treatment, and then switched to high dose methotrexate/cytarabine. His disease relapsed and resulted in death from bone and brain disease 6 months after complete clinical remission, despite diagnostic workups, including a radioisotope liver scan and ultrasound-guided fine needle aspiration biopsy. Further cytogenetic studies such as comparative genomic hybridization could elucidate the genetic mechanisms in the development and progression of lymphomas. We report an unusual case of 'CD4-/CD56+/CD123+ HN' showing early liver metastasis.

Keyword

CD4-/CD56+/CD123+ hematodermic neoplasm; Early liver metastasis

MeSH Terms

Aged
Biopsy
Biopsy, Fine-Needle
Brain Diseases
Comparative Genomic Hybridization
Cytogenetics
Dendritic Cells
Doxorubicin
Humans
Incidence
Killer Cells, Natural
Liver
Lymphoma
Male
Neoplasm Metastasis
Vincristine
Doxorubicin
Vincristine

Figure

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  Fig. 1 A 1.9×1.8 cm exophytic dome-shaped mass in normal skin on the presternal area.

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  Fig. 2 The tumor is composed of a diffuse monotonous infiltrate of medium-sized cells with fine chromatin and scanty cytoplasm resembling lymphoblastic or myeloblastic leukemia (A: H&E stain, ×200, B: H&E stain, ×400). Immunohistochemical staining for CD56 and CD123 is positive, but CD68 and CD4 is negative (C, D, E, F: ×200).

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  Fig. 3 A 10 cm sized huge mass was visualized on left lobe of liver with portal vein thrombosis and enlarged periportal lymph nodes by abdominal CT (A) and was observed as a SOL by 99mTc-liver scan (A-inset). Normal hepatocytes were replaced by CD56 positive tumor cells with atypical lymphocyte appearance (B, C: ×200).

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