Related Experiment Video
Updated: Aug 8, 2026
Tumor Engraftment in a Xenograft Mouse Model of Human Mantle Cell Lymphoma
Published on: March 30, 2018
[Agranular CD4+ CD56+ hematodermic neoplasm: a new case report]
M Chamaillard1, M Beylot-Barry, J-F Viallard
1Service de Dermatologie, Hôpital Haut Lévêque, CHU de Bordeaux, avenue de Magellan, 33600 Pessac.
Insights
Agranular CD4+ CD56+ hematodermic neoplasm, a rare cancer of plasmacytoid dendritic cells, presents unique skin and blood characteristics. This distinct entity has a poor prognosis despite various treatments.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Agranular CD4+ CD56+ hematodermic neoplasm is a rare hematologic malignancy.
- This neoplasm is now understood to originate from plasmacytoid dendritic cells.
Observation:
- An 83-year-old patient presented with purple skin lesions infiltrating the dermis.
- Biopsy revealed malignant cells (CD4+, CD56+, CD43+) with dermal infiltration, but no bone marrow or blood involvement.
- The patient achieved remission with chemotherapy but relapsed four months later.
Findings:
- Agranular CD4+ CD56+ hematodermic neoplasm is distinct from cutaneous lymphomas.
- Plasmacytoid monocytes expressing CD123 (IL-3 receptor) are identified as malignant precursors.
- The disease exhibits skin tropism, potential bone marrow involvement, and a poor prognosis.
Implications:
- This neoplasm represents a distinct clinicopathologic entity.
- Current treatments show limited efficacy, highlighting the need for novel therapeutic strategies.
- Targeting CD123 with antibody-based therapies may offer a promising treatment avenue.
Background:
"Agranular CD4+ CD56+ hematodermic neoplasm" are rare hematologic neoplasms which were recently shown to correspond to the plasmocytoid dendritic cells.
Case Report:
A 83-year-old presented isolated skin lesions purple, infiltrating the dermis. The biopsy has shown a dense dermal infiltration with malignant cells CD4+ CD56+ CD43+. There were no bone marrow involvement and no circulating blood cells. A chemotherapy permitted a clinical remission after six courses. Unfortunately, skin and blood relapses appear four months later. After a short success of chemotherapy by DHAP, the patient died three month later.
Discussion:
"Agranular CD4+ CD56+ hematodermic neoplasm" is a distinct entity from the cutaneous primary lymphomas. Recently plasmocytoid monocyte cells have been identified as the precursor of the malignant population with the high expression of CD123, IL3 receptor. It is a distinct clinicopathologic entity by its clinical presentation with skin tropism, bone marrow involvement with or without leukemic phase and poor prognosis independent of the kind of treatment and its particular phenotype CD4+ CD56+ CD43+. It would be interesting to use antibodies linked to CD123 in therapeutic because any treatment have efficacity in this disease.
