Blastic plasmacytoid dendritic neoplasia

Rare haematological neoplasia, which can be classified as acute myeloid leukaemia (WHO classification). The original cell is assumed to be the so-called plasmocytoid dendritic cell. There are competing views on this cell regarding its lineage affiliation to the lymphatic or myeloid series. The current WHO classification defines neoplasia as blastic plasmocytoid neoplasia of dendritic cells. Thus, it is no longer listed under lymphomas, but is assigned to acute myeloid leukemias.

It occurs in adolescents and young adults (10-20 years) as well as in older age groups (50-60 years).

face, trunk, extremities

More than 90% of patients present with skin lesions at an early stage, so that the skin changes can be monitor signs of neoplasia. The skin lesions are very diverse. Often disseminated, solid, red, surface smooth papules, plaques or nodules are found. More rarely are localized plaques or nodules. Lymphadenopathies occur early on together with the skin changes, followed by leukocytosis and lymphocytosis.

Nodular proliferates of medium-sized cells that resemble myeloblasts or lymphoblasts. No epidermotropy (see clinic - surface smooth papules and nodules as in B-cell lymphomas). The cells show the following expression pattern: CD3-/CD4+/CD8-/CD56+ (see CD classification below).

Therapy: the first-line therapy is the chemotherapy used for aggressive lymphomas or acute leukemias. Stem cell transplantation is intended for relapses.

The average survival time is 12 months for chemotherapy alone and 30 months for stem cell transplantation after the first relapse. The median survival time is 12-14 months.

The cutaneous CD 4 positive CD 56 positive malignant tumor proliferation was originally called: CD 4/CD 56 hematodermic neoplasm. Recent studies have shown that this disease develops from plasmacytoid dendritic cells. In this respect, the tumour is now called "Blastic plamocytoid dendridic cell neoplasm". This is an aggressive disease with a bad prognosis that clinically behaves like an acute leukemia.

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