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Case Report: Mature Plasmacytoid Dendritic Cell Proliferation Associated With a Lymphoid Neoplasm

Fei Fei1, Michaela Liedtke2, Oscar Silva1

  • 1Department of Pathology, Stanford University School of Medicine, Stanford, CA, United States.

Frontiers in Oncology
|July 25, 2022
PubMed

Insights

Mature plasmacytoid dendritic cell proliferations (MPDCPs) are non-malignant clonal growths. This study reports the first MPDCP associated with T-lymphoblastic leukemia (T-ALL), a lymphoid neoplasm, expanding understanding of MPDCP origins.

Area of Science:

  • Hematology
  • Immunology
  • Oncology

Background:

  • Mature plasmacytoid dendritic cell proliferations (MPDCPs) are clonal, non-malignant growths typically associated with myeloid neoplasms.
  • Previous reports have linked MPDCPs to conditions like CMML, AML, MDS, and MPNs.

Observation:

  • This study details the first documented case of an MPDCP occurring in conjunction with T-lymphoblastic leukemia (T-ALL), a lymphoid neoplasm.
  • The MPDCP comprised approximately 50% of the bone marrow, presenting in nodular aggregates with specific immunophenotypic markers (CD123, CD4, CD303) and aberrant expression of T-lymphoblastic markers (CD7, CD5, CD10, CD13).

Findings:

  • Mutational analysis revealed mutations in JAK3, NOTCH1, NRAS, KRAS, DNMT3A, and SH2B3 in the MPDCP.
  • Bone marrow plasmacytoid dendritic cell (pDC) frequency was often decreased in T-ALL patients compared to controls.

Implications:

  • This case represents the first association of MPDCP with a lymphoid neoplasm, challenging previous associations solely with myeloid disorders.
  • The findings suggest that MPDCPs may originate from multipotent hematopoietic progenitors with lymphoid and dendritic cell potential.
  • Further research into MPDCPs in lymphoid neoplasms is warranted to understand their pathogenesis and clinical significance.
Keywords:
BPDCNMPDCPT-ALLleukemialymphoidmaturepDC proliferation

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