scholarly journals Plasmacytoid dendritic cell nodules in bone marrow biopsies of chronic myelomonocytic leukemia

2010 ◽  
Vol 85 (11) ◽  
pp. 893-893 ◽  
Author(s):  
Kikkeri N. Naresh ◽  
Jiri Pavlu
Author(s):  
Ruth-Miriam Koerber ◽  
Stefanie A. E. Held ◽  
Maria Vonnahme ◽  
Georg Feldmann ◽  
Joerg Wenzel ◽  
...  

AbstractBlastic plasmacytoid dendritic-cell neoplasm (BPDCN) is an extremely rare disease that originates from dendritic cells and is associated with a poor overall survival (OS). Diagnostic and therapeutic standards are less well-established in comparison to other leukemic conditions and standards of care are lacking. Morphologic and molecular similarities to acute myeloid leukemia (AML), myelodysplastic syndrome (MDS) and chronic myelomonocytic leukemia (CMML) are hard to distinguish. We here report a BPDCN patient with a long, challenging diagnostic period. While bone marrow biopsies initially failed to prove the correct diagnosis, a cutaneous biopsy finally identified a CD45+/CD56+/CD4+/CD123+/CD33+/MPO− population suggestive of BPDCN which was confirmed by flow cytometry. Molecular analysis revealed an ASXL-1, TET2 and SRSF2-mutation, cytogenetic analysis showed a normal karyotype. Treatment with the recently approved CD123-cytotoxin Tagraxofusp showed initially a very good response. This case reflects diagnostic and therapeutic difficulties in BPDCN as very rare, easily misdiagnosed neoplasia and the need for precise diagnostic care.


Leukemia ◽  
2017 ◽  
Vol 31 (5) ◽  
pp. 1238-1240 ◽  
Author(s):  
L Brunetti ◽  
V Di Battista ◽  
A Venanzi ◽  
G Schiavoni ◽  
M P Martelli ◽  
...  

2014 ◽  
Vol 45 (12) ◽  
pp. 2471-2479 ◽  
Author(s):  
Birgit Federmann ◽  
Manuel Abele ◽  
David Santiago Rosero Cuesta ◽  
Wichard Vogel ◽  
Leonardo Boiocchi ◽  
...  

2009 ◽  
Vol 454 (4) ◽  
pp. 411-419 ◽  
Author(s):  
Marwan Qubaja ◽  
Béatrice Marmey ◽  
Agnès Le Tourneau ◽  
Stéphanie Haiat ◽  
Dominique Cazals-Hatem ◽  
...  

2013 ◽  
Vol 88 (6 suppl 1) ◽  
pp. 158-161 ◽  
Author(s):  
André Lencastre ◽  
Joana Cabete ◽  
Alexandre João ◽  
Pedro Farinha ◽  
Gilda Ferreira ◽  
...  

Blastic plasmacytoid dendritic cell neoplasm is a rare and aggressive hematodermic neoplasia with frequent cutaneous involvement and leukemic dissemination. We report the case of a 76-year-old man with a 2 month history of violaceous nodules and a tumor with stony consistency, located on the head, and mandibular, cervical and supraclavicular lymphadenopathies. Multiple thoracic and abdominal adenopathies were identified on computerized tomography. Flow cytometry analysis of the skin, lymph node and bone marrow biopsies demonstrated the presence of plasmocytoid dendritic cell neoplastic precursor cells (CD4+, CD45+, CD56+ and CD123+ phenotype). After initial clinical and laboratorial complete remission with chemotherapy, the patient died due to relapse of the disease associated with the appearance of a cervical mass with medullary compromise.


2020 ◽  
Author(s):  
Abhishek A. Mangaonkar ◽  
Kaaren K. Reichard ◽  
Moritz Binder ◽  
Giacomo Coltro ◽  
Terra L. Lasho ◽  
...  

AbstractSystemic immune tolerance is not well-characterized in chronic myelomonocytic leukemia (CMML). Due to the presence of clonal plasmacytoid dendritic cells (pDC) in CMML, and the established association of lymph node indoleamine 2,3-dioxygenase-1 (IDO1)-positive (+) DC populations (IDC) with systemic immune tolerance in other malignant contexts, we sought to determine the association of IDO1 expression and bone marrow (BM) DC populations with systemic T-cell compartment changes using primary CMML patient samples (BM, plasma, and peripheral blood mononuclear cells) via immunohistochemistry (IHC), liquid chromatography-mass spectrometry (LC-MS), and time-of-flight mass cytometry (CyTOF). Our results highlight that aggregate BM IDC (CD123 and/or CD11c positive) occur in 33% CMML patients at any disease time-point (IHC), correlate with accentuated tryptophan catabolism (LC-MS, increased kynurenine level, median 4.7 versus 3 microM, P=0.049*), systemic regulatory T-cell expansion (CyTOF, %parent cell type, 14.5 versus 4.9%, P=0.04*) and play a role in disease progression, as evidenced by a higher rate of transformation to acute myeloid leukemia (41 versus 13%, P=0.002**), when compared to CMML patients without BM IDC. Our data also highlight a perturbed immune system in CMML with specific systemic immune signatures, particularly type 1, IL-17 producing helper T, CD4 terminal effector and natural killer cell suppression.Key PointsAggregate IDO1+ dendritic cell populations occur in the CMML bone marrow microenvironment, and their presence correlates with disease progression.Systemic immune microenvironment signatures in CMML indicate an altered T- and natural killer (NK)-cell balance. Specifically, suppression of type 1 helper T (Th1), IL-17 producing helper T (Th17), CD4 terminal effector and NK cells.IDO1+ bone marrow dendritic cell populations in CMML are associated with a T-cell compartment shift towards a regulatory T cell phenotype.


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