BPDCN
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare and aggressive hematologic malignancy that originates from plasmacytoid dendritic cells. It is characterized by the proliferation of malignant cells that can infiltrate the skin, bone marrow, lymph nodes, and other organs.
Clinical Presentation[edit | edit source]
BPDCN typically presents with skin lesions, which may appear as bruise-like patches, nodules, or plaques. These lesions are often purple or red and can be found on the face, trunk, and extremities. Patients may also experience systemic symptoms such as fever, fatigue, and weight loss. In advanced stages, BPDCN can involve the bone marrow, leading to cytopenias and other hematologic abnormalities.
Diagnosis[edit | edit source]
The diagnosis of BPDCN is confirmed through a combination of clinical, histopathological, and immunophenotypic findings. Skin biopsies and bone marrow aspirates are commonly used to obtain tissue samples. Immunohistochemistry is essential for diagnosis, with malignant cells typically expressing markers such as CD4, CD56, and CD123. The presence of these markers helps distinguish BPDCN from other hematologic malignancies.
Treatment[edit | edit source]
Treatment options for BPDCN include chemotherapy, targeted therapy, and hematopoietic stem cell transplantation. Initial treatment often involves multi-agent chemotherapy regimens similar to those used for acute lymphoblastic leukemia or acute myeloid leukemia. Targeted therapies, such as tagraxofusp-erzs, have shown promise in treating BPDCN by targeting CD123. Hematopoietic stem cell transplantation may be considered for eligible patients to achieve long-term remission.
Prognosis[edit | edit source]
The prognosis for BPDCN is generally poor due to its aggressive nature and high relapse rate. However, early diagnosis and appropriate treatment can improve outcomes. The overall survival rate varies, with some patients achieving long-term remission following stem cell transplantation.
Related Pages[edit | edit source]
- Plasmacytoid dendritic cell
- Acute lymphoblastic leukemia
- Acute myeloid leukemia
- Hematopoietic stem cell transplantation
- Immunohistochemistry
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