Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
32-49
Plasmacytoid dendritic cell
leukemia - case study and diagnosis
Leucemia de células dendríticas plasmocitoides -
estudio y diagnóstico de un caso
Faicán Rocano Pedro Fernando 1 , Dután Pérez Ibelice Salomé 2, González González Camila Raquel 3
Matute Aguiar Joselyn Nayeli 4 , Rodríguez Verdugo Alejandra Valentina 5
Research Professor: pedro.faican@ucacue.edu.ec: Azogues, Ecuador.
Student: ibelice.dutan.44@est.ucacue.edu.ec: Azogues, Ecuador.
Student: camila.gonzalez.66@est.ucacue.edu.ec: Azogues, Ecuador.
Student: joselyn.matute.44@est.ucacue.edu.ec,: Azogues, Ecuador.
Student: alejandra.rodriguez.60@est.ucacue.edu.ec: Azogues, Ecuador.
How to cite:
Faican Rocano, P. F., Dután Pérez, I. S., González González, C. R., Rodríguez Verdugo, A. V., & Matute
Aguiar, J. N. (2025). Plasmacytoid dendritic cell leukemia—case study and diagnosis. Revista
MultidisciplinariaInvestigación Contemporánea, 3(2), 32-49. https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Article Information
Received: 05-12-2024
Accepted: 21-02-2025
Published: 01-07-2025
Editor’s Note
REDLIC remains neutral regarding jurisdictional
claims in published messages and institutional
afliations.
Editorial
Latin American Editorial Network for
Contemporary Research (REDLIC)
www.editorialredlic.com
Funding Sources
The research was conducted using the authors’
own resources.
Conicts of Interest
No conicts of interest are declared.
This text is protected by a Creative Commons 4.0.
You are free to Share - copy and redistribute the material in any
medium or format - and Adapt the document - remix, transform, and
build upon the material - for any purpose, including commercial
purposes, provided you comply with the condition of:
Attribution: you must credit the original work appropriately, provide
a link to the license, and indicate if changes have been made.
You may do so in any reasonable manner, but not in such a way
as to suggest that you are endorsed by or receive support from the
licensor for your use of the work.
Revista multidisciplinaria
Investigación Contemporánea 07 - 2025 Vol. 3 - No. 2 ISSN-e: 2960-8015
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Leucemia de células dendríticas plasmocitoides
- estudio y diagnóstico de un caso 49 -49
Copyright (c) 2025 Faicán Rocano Pedro Fernando, Dután Pérez Ibelice Salomé,
González González Camila Raquel, Matute Aguiar Joselyn Nayeli, Rodríguez Ver-
dugo Alejandra Valentina.
Este texto está protegido por una licencia Creative Commons 4.0.
Usted es libre para Compartir —copiar y redistribuir el material en cualquier medio
o formato — y Adaptar el documento —remezclar, transformar y crear a partir del
material— para cualquier propósito, incluso para nes comerciales, siempre que
cumpla la condición de:
Atribución: Usted debe dar crédito a la obra original de manera adecuada, propor-
cionar un enlace a la licencia, e indicar si se han realizado cambios. Puede hacerlo
en cualquier forma razonable, pero no de forma tal que sugiera que tiene el apoyo
del licenciante o lo recibe por el uso que hace de la obra.
Resumen de licencia - Texto completo de la licencia
Article Clinical case. Revista MultidisciplinariaInvestigación Contemporánea.
Vol. 3 - No. 2, pp. 32 - 49. July-December, 2025. e-ISSN: 2960-8015
Revista multidisciplinaria
Investigación Contemporánea 07 - 2025 Vol. 3 - No. 2 ISSN-e: 2960-8015
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Leucemia de células dendríticas plasmocitoides
- estudio y diagnóstico de un caso 32 -49
Artículo Caso clínico. Revista multidisciplinaria investigación Contemporánea.
Vol. 3 - No. 2, pp. 32 - 49. julio-diciembre, 2025. e-ISSN: 2960-8015
Leucemia de células dendríticas
plasmocitoides - estudio y diagnóstico
de un caso
Leucemia de células dendríticas plasmocitoides - estudio y
diagnóstico de un caso
Información del artículo:
Recibido: 05-12-2024
Aceptado: 21-02-2025
Publicado: 01-07-2025
Nota del editor:
REDLIC se mantiene neutral con respecto a
reclamos jurisdiccionales en mensajes publi-
cados y aliaciones institucionales.
Editorial:
Red Editorial Latinoamericana de Investigación
Contemporánea (REDLIC) www.editorialredlic.com
Fuentes de nanciamiento:
La investigación fue realizada con recursos propios.
Conictos de interés:
No presentan conicto de intereses.
Este texto está protegido por una licencia Creative Commons 4.0.
Usted es libre para Compartir —copiar y redistribuir el material en cual-
quier medio o formato— y Adaptar el documento —remezclar, transformar
y crear a partir del material— para cualquier propósito, incluso para nes
comerciales, siempre que cumpla la condición de:
Atribución: Usted debe dar crédito a la obra original de manera adecuada,
proporcionar un enlace a la licencia, e indicar si se han realizado cambios.
Puede hacerlo en cualquier forma razonable, pero no de forma tal que sugie-
ra que tiene el apoyo del licenciante o lo recibe por el uso que hace de la obra
1 Docente Investigador; pedro.faican@ucacue.edu.ec. Azogues, Ecuador.
2 Estudiante; ibelice.dutan.44@est.ucacue.edu.ec. Azogues, Ecuador.
3 Estudiante; camila.gonzalez.66@est.ucacue.edu.ec. Azogues, Ecuador.
4 Estudiante; joselyn.matute.44@est.ucacue.edu.ec. Azogues, Ecuador.
5 Estudiante; alejandra.rodriguez.60@est.ucacue.edu.ec. Azogues, Ecuador.
Faicán Rocano Pedro Fernando 1, Dután Pérez Ibelice Salomé 2, González González Camila
Raquel 3, Matute Aguiar Joselyn Nayeli 4, Rodríguez Verdugo Alejandra Valentina 5
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Cómo citar:
Faican Rocano, P. . F., Dután Pérez, I. S. ., González González, C. R. ., Rodríguez Verdugo, A. V. ., & Matute Aguiar, J.
N. . (2025). Leucemia de células dendríticas plasmocitoides - estudio y diagnóstico de un caso.
Revista Multidisci-
plinaria Investigación Contemporánea
, 3(2), 32-49. https://doi.org/10.58995/redlic.rmic.v3.n2.a90
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Revista multidisciplinaria
Investigación Contemporánea 07 - 2025 Vol. 3 - No. 2 ISSN-e: 2960-8015
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Leucemia de células dendríticas plasmocitoides
- estudio y diagnóstico de un caso 32 -49
Artículo Caso clínico. Revista multidisciplinaria investigación Contemporánea.
Vol. 3 - No. 2, pp. 32 - 49. julio-diciembre, 2025. e-ISSN: 2960-8015
Leucemia de células dendríticas
plasmocitoides - estudio y diagnóstico
de un caso
Leucemia de células dendríticas plasmocitoides - estudio y
diagnóstico de un caso
Información del artículo:
Recibido: 05-12-2024
Aceptado: 21-02-2025
Publicado: 01-07-2025
Nota del editor:
REDLIC se mantiene neutral con respecto a
reclamos jurisdiccionales en mensajes publi-
cados y aliaciones institucionales.
Editorial:
Red Editorial Latinoamericana de Investigación
Contemporánea (REDLIC) www.editorialredlic.com
Fuentes de nanciamiento:
La investigación fue realizada con recursos propios.
Conictos de interés:
No presentan conicto de intereses.
Este texto está protegido por una licencia Creative Commons 4.0.
Usted es libre para Compartir —copiar y redistribuir el material en cual-
quier medio o formato— y Adaptar el documento —remezclar, transformar
y crear a partir del material— para cualquier propósito, incluso para nes
comerciales, siempre que cumpla la condición de:
Atribución: Usted debe dar crédito a la obra original de manera adecuada,
proporcionar un enlace a la licencia, e indicar si se han realizado cambios.
Puede hacerlo en cualquier forma razonable, pero no de forma tal que sugie-
ra que tiene el apoyo del licenciante o lo recibe por el uso que hace de la obra
1 Docente Investigador; pedro.faican@ucacue.edu.ec. Azogues, Ecuador.
2 Estudiante; ibelice.dutan.44@est.ucacue.edu.ec. Azogues, Ecuador.
3 Estudiante; camila.gonzalez.66@est.ucacue.edu.ec. Azogues, Ecuador.
4 Estudiante; joselyn.matute.44@est.ucacue.edu.ec. Azogues, Ecuador.
5 Estudiante; alejandra.rodriguez.60@est.ucacue.edu.ec. Azogues, Ecuador.
Faicán Rocano Pedro Fernando 1, Dután Pérez Ibelice Salomé 2, González González Camila
Raquel 3, Matute Aguiar Joselyn Nayeli 4, Rodríguez Verdugo Alejandra Valentina 5
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Cómo citar:
Faican Rocano, P. . F., Dután Pérez, I. S. ., González González, C. R. ., Rodríguez Verdugo, A. V. ., & Matute Aguiar, J.
N. . (2025). Leucemia de células dendríticas plasmocitoides - estudio y diagnóstico de un caso.
Revista Multidisci-
plinaria Investigación Contemporánea
, 3(2), 32-49. https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
33-49
Abstract
Plasmacytoid dendritic cell leukemia (pDCL) is a rare and aggressive
neoplasm that affects the skin, lymph nodes, and may spread to the bone
marrow. Recognized by the WHO as a distinct entity since 2016, it has a
median survival of 12 to 16 months. Although sometimes associated with
Epstein-Barr virus and HIV, its exact etiology remains uncertain. Genetic
characteristics include deletions in chromosomes 4, 9, and 13, and
overexpression of oncogenes.
The case study describes a 49-year-old patient with a history of
type 2 diabetes mellitus who presented with symptoms of leukopenia,
neutropenia, and thrombocytopenia. Agranulocytosis was initially
suspected, but subsequent tests conrmed plasmacytoid dendritic cell
leukemia and its progression.
Keywords: Leukemia, Cells, Dendritics, Case Reports
Resumen
La leucemia de células dendríticas plasmacitoides (pDCL) es una
forma rara y agresiva de cáncer que afecta la piel y los ganglios linfáticos y
puede extenderse a la médula ósea. Desde 2016 la OMS la reconoce como
una entidad independiente, con una expectativa de vida media de 12 a 16
meses. Aunque a veces se relaciona con los virus Epstein-Barr y VIH, su
causa exacta sigue siendo desconocida. Genéticamente se caracteriza por
deleciones en los cromosomas 4, 9 y 13, y la sobreextensión de oncogenes
El caso clínico describe a un hombre de 49 años con diabetes mellitus
tipo 2 que presentó síntomas de leucopenia, neutropenia y plaquetopenia.
Inicialmente se sospechó de agranulocitosis, pero las pruebas conrmaron
leucemia de células dendríticas plasmocitoides y su progresión.
Palabras clave: Leucemia, Células, Dendríticas, mieloide aguda, neoplasia
maligna
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
34-49
1 Introduction
Blastic plasmacytoid dendritic cell neoplasia (BDPCN) is a
malignant disease whose precursor is the plasmacytoid dendritic cell
(PDC). It is characterized by affecting the skin and lymph nodes, although
it can evolve to a disseminated form and inltrate the bone marrow.
Initially classied as acute myeloid leukemia (AML), the World Health
Organization (WHO) recognized it as an independent entity in 2016. This
neoplasia is distinguished by its aggressive behavior, rapid systemic
dissemination, and a median survival of 12 to 16 months. Its incidence
is low, representing less than 1% of malignant neoplasms, and is more
common in men than women, with a ratio of 3:1, predominating in the
sixth decade of life; however, there are case studies in patients under 40
years of age (1,2).
The etiology of NBCDP is not well dened. Although it has been
associated with Epstein-Barr virus (EBV) and human immunodeciency
virus (HIV), studies have not established a clear correlation. At the
molecular level, this neoplasia presents deletions in chromosomes 4
(4q34), 9 (9p13-p11 and 9q12-q34) and 13 (13q12-q31), which contain
several tumor suppressor genes with low expression of Rb1 and LATS2,
in addition to high levels of oncogenes (HES, RUNX2, FTL3) without
association with amplication (2).
The typical clinical picture of NBCDP includes skin lesions,
lymphadenopathy, and organomegaly, consistent with the descriptions
in this report. Skin lesions vary in size, shape, and color and may present
as tumors, nodules, and plaques, generally affecting the face, trunk, and
extremities (2,3).
Since NBCDP shares several common clinical and diagnostic features
with acute leukemias and aggressive T-cell lymphomas, differential
diagnosis is challenging and requires a thorough workup, in particular
the detection of the dening immunophenotype is essential (3).
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
35-49
Diagnosis requires extensive immunophenotypic analysis due to
overlap with other neoplasms, in addition to clinical and histopathological
correlation. There is no standardized treatment; however, improved
outcomes have been observed in patients treated with a regimen similar
to that for acute lymphocytic leukemia and consolidation with allogeneic
transplantation (4).
2 Clinical case
A 49-year-old married man, a driver from Azogues and resident in
Cuenca, with a secondary education, presented a history of type 2 diabetes
mellitus treated with metformin and two episodes of COVID-19. He also
had a family history of type 2 diabetes mellitus in his parents and siblings.
He initially presented with lower back pain associated with colitis and
intolerance to dairy and citrus fruits. These symptoms persisted, leading
him to seek medical attention due to persistent lower back pain and food
intolerance, which prompted additional testing.
During the consultation, her vital signs were: temperature of 36
degrees Celsius, heart rate of 80 beats per minute, respiratory rate of 20
breaths per minute, blood pressure of 120/76 mmHg, oxygen saturation
of 88%, and capillary blood glucose of 444 mg/dL. Physical examination
revealed a poor general appearance and edema of the right lower extremity
with petechiae.
Initial blood tests revealed leukopenia, neutropenia, and low
platelet count, while imaging studies appeared normal. Agranulocytosis
was initially suspected, but the neutropenia persisted and became severe,
prompting a bone marrow aspiration and biopsy (bone marrow analysis
and immunophenotype). Preliminary results showed 48% myeloid blasts,
suggesting possible acute leukemia. After repeat testing, the diagnosis
of acute myeloblastic leukemia was conrmed, and chemotherapy was
prescribed, managing peglgrastim (6 mg subcutaneous).
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
36-49
Revista multidisciplinaria
Investigación Contemporánea 07 - 2025 Vol. 3 - No. 2 ISSN-e: 2960-8015
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Leucemia de células dendríticas plasmocitoides
- estudio y diagnóstico de un caso 36 -49
na de 8.1 mg/dl. Una tomografía abdominal reveló una colección abdominal
con obstrucción intestinal alta, por lo que se realizó una colectomía total con
ileostomía. La evolución continuó siendo desfavorable, desarrollando un
cuadro de choque séptico e hipovolémico con insuciencia renal aguda, y el
paciente falleció cuatro meses después del diagnóstico.
3. Análisis
Los síndromes mielodisplásicos (SMD) son enfermedades clonales de la mé-
dula ósea caracterizadas por una hematopoyesis inecaz, que se presenta
como displasia morfológica de los elementos hematopoyéticos y citopenias
periféricas, con un alto riesgo de progresar a leucemia aguda en el futuro (5).
Esta patología es más común en pacientes que han recibido quimiote-
rapia, radioterapia o ambos tratamientos. En algunos casos, los pacientes son
asintomáticos y el diagnóstico se realiza mediante un hemograma o un es-
tudio de médula ósea, donde pueden encontrarse anemia, trombocitopenia,
pancitopenia y neutropenia (5).
La mayoría de los pacientes presentan signos y síntomas relacionados
con las citopenias, como palidez, epistaxis, hematoquecia, hematuria, hema-
tomas y petequias (6).
Un grupo de trastornos de células madre hematopoyéticas clonales, los
síndromes mielodisplásicos, se caracterizan por la presencia de múltiples
mutaciones de células madre hematopoyéticas, con mayor frecuencia en ge-
nes involucrados en el empalme del RNA (7,8). (7)(8)
El siguiente gráco describe la progresión de trastornos en las células
madre hematopoyéticas, enfocándose en los síndromes mielodisplásicos y su
evolución a leucemias. A continuación, se detalla su contenido:
The patient’s progress was unfavorable, with petechiae on the
lower extremities and intermittent fever, suggesting infection. Blood
tests showed a leukocytosis of 81,050 and a hemoglobin of 8.1 mg/dL. An
abdominal CT scan revealed an abdominal collection with upper intestinal
obstruction, leading to a total colectomy with ileostomy. The patient’s
progress continued unfavorably, developing septic and hypovolemic
shock with acute renal failure, and he died four months after diagnosis.
3 Analysis
Myelodysplastic syndromes (MDS) are clonal diseases of the
bone marrow characterized by ineffective hematopoiesis, presenting as
morphological dysplasia of the hematopoietic elements and peripheral
cytopenias, with a high risk of progressing to acute leukemia in the future
(5).
This condition is more common in patients who have received
chemotherapy, radiation therapy, or both. In some cases, patients are
asymptomatic, and diagnosis is made through a complete blood count or
bone marrow examination, which may reveal anemia, thrombocytopenia,
pancytopenia, and neutropenia (5).
Most patients present with signs and symptoms related to
cytopenias, such as pallor, epistaxis, hematochezia, hematuria, bruising,
and petechiae (6).
A group of clonal hematopoietic stem cell disorders, the
myelodysplastic syndromes, are characterized by the presence of multiple
hematopoietic stem cell mutations, most frequently in genes involved in
RNA splicing (7,8).
The following chart describes the progression of hematopoietic
stem cell disorders, focusing on myelodysplastic syndromes and their
progression to leukemia. Its contents are detailed below:
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
37-49

Myelodysplastic syndromes (MDS) according to their morphological
and genetic characteristics. It includes subtypes such as those with
dysplasia of one or more cell lines, ring sideroblasts, isolated deletion of
chromosome 5, and refractory anemia with excess blasts (types I and II).
Unclassiable variants and those with myeloproliferative characteristics,
such as chronic myelomonocytic leukemia, are also described. This
classication allows for diagnosis and prognosis in patients with MDS, as
shown below:
Revista multidisciplinaria
Investigación Contemporánea 07 - 2025 Vol. 3 - No. 2 ISSN-e: 2960-8015
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Leucemia de células dendríticas plasmocitoides
- estudio y diagnóstico de un caso 37 -49
Gráco 1
Fisiopatología de la leucemia. Elaborado por: Dután I, González C, Matute N, Rodríguez A.
Los síndromes mielodisplásicos (SMD) según sus características mor-
fológicas y genéticas. Incluye subtipos como con displasia de una o varias lí-
neas celulares, con sideroblastos en anillo, deleción aislada del cromosoma 5
y anemia refractaria con exceso de blastos (tipos I y II). También se describen
variantes inclasicables y aquellos con características mieloproliferativas,
como la leucemia mielomonocítica crónica. Esta clasicación permite esta-
blecer el diagnóstico y determinar el pronóstico en pacientes con SMD, como
se muestra a continuación:
Stem cells Myeloid stem cell
Lymphoid stem cell
There is an alteration in the stem cell that is
transmitted to other stem cells in the body
where its own clonal cells are generated. This
process is called CHIP.
Clonal cells accumulate, generating acute leukemia
secondary to myelodysplastic syndrome.
The third phase can be considered
myelodysplastic syndrome (MDS) or
clonal cytopenia of undetermined
signicance (CCUS). Proliferation
begins in the bone marrow, giving rise
to many clonal cells in which all the
cells have the same genetic alteration.
White blood cells
Red blood cellsPlatelets
Lymphoid
Figure 1
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
38-49
Table 1: Classication of myelodysplastic syndrome
Revista multidisciplinaria
Investigación Contemporánea 07 - 2025 Vol. 3 - No. 2 ISSN-e: 2960-8015
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Leucemia de células dendríticas plasmocitoides
- estudio y diagnóstico de un caso 38 -49
Tabla 1: Clasicación del síndrome mielodisplásico
Elaborado por: Dután I, González C, Matute N, Rodríguez A
4. Discusión
Entre los principales diagnósticos diferenciales están, la leucemia mieloide
aguda (LMA) aguda con diferenciación monocítica, la leucemia mielomono-
cítica crónica, el linfoma de células T/NK extraganglionar de tipo nasal, el
linfoma de células T paniculítico (9).
Para diferenciar entre una leucemia mieloide aguda (LMA) y la leu-
cemia de células dendríticas plasmacitoides (pDCL) se necesita evaluar las
características clínicas, inmunofenotípicas, morfológicas, y genéticas. En
cuanto a la clínica, tanto la LMA como la pDCL presentan síntomas parecidos,
TIPO DE SMD
SMD con displasia de una sola línea
SMD con displasia de una multilínea
SMD con sideroblastos en anillo
Con displasia de una sola línea
Con displasia multilínea
SMD con deleción aislada del cromosoma 5
Anemia refractaria con exceso de blasto
Tipo l
Tipo II
SMD inclasicables
Síndrome mielodisplásico/ mieloproliferativo
Leucemia mielomonocitica crónica
Tipo O
Tipo l
Tipo II
Discussion
Among the main differential diagnoses are acute acute myeloid
leukemia (AML) with monocytic differentiation, chronic myelomonocytic
leukemia, nasal type extranodal NK T-cell lymphoma, and panniculitic
T-cell lymphoma (9).
TYPE OF MDs
MDs with single-line dysplasia
MDs with multilineage dysplasia
MDs syndrome with ring sideroblasts
• with single-line dysplasia
• with multiline dysplasia
MDs with isolated detection of chromosome 5
Refractory anemia with excess blasts
• Type I
• Type II
Unclassiable MDs
Myelodysplastic/myeloproliferative syndrome
Chronic myelomonocytic leukemia
• Type 0
• Type I
• Type II
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
39-49
Differentiating between acute myeloid leukemia (AML) and
plasmacytoid dendritic cell leukemia (pDCL) requires an assessment of
clinical, immunophenotypic, morphological, and genetic characteristics.
Clinically, both AML and pDCL present similar symptoms, including
fever, fatigue, cachexia, and cytopenias, which may be due to bone marrow
inltration by malignant cells. pDCL is usually associated with skin
lesions, hepatomegaly, and lymphadenopathy (10).
Morphologically, acute myeloid leukemia cells have Auer bodies,
whereas pDCL cells lack them. In AML, leukemic cells display myeloid
markers such as CD13, CD33, CD117, and MPO (myeloperoxidase). In
contrast, pDCL displays an immunophenotypic prole with expression of
CD4, CD56, CD123, and certain specic markers BDCA-2 and TCL1 (10,11).
Nasal-type extranodal NK/T-cell lymphoma occurs predominantly
in and around the nose, but can also affect other parts of the body. It is
associated with Epstein-Barr virus (EBV) infection. Histologically, it has
an angiocentric and angioinvasive pattern with a wide area of necrosis.
Plasmacytoid dendritic cell leukemia can present with systemic symptoms
and cutaneous involvement and is not usually limited to a specic region
such as the nose. The patient’s clinical presentation is also useful, since
NK/T-cell lymphoma is characterized by nasal symptoms and obstructive
masses, whereas dendritic cell leukemia does not show this pattern (12).
To differentiate between a panniculitis T-cell lymphoma (PPCTL)
and a (pDCL) a complex evaluation must be made regarding its clinical
presentation and some morphological characteristics (13).
LPCTL presents with subcutaneous skin lesions resembling
panniculitis, primarily affecting the extremities. Histologically, there
is an inltrate of atypical lymphocytes in the subcutaneous tissue, with
adipocyte necrosis and cytoskeletal formation. Lymphocytes in LPCTL
display T cell markers such as CD3, CD4, or CD8, and a loss of markers
found in normal T cells. Furthermore, they may express cytotoxicity, as
evidenced by the expression of granzyme B, perforin, and TIA-1 (14).
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
40-49
Blastic plasmacytoid dendritic cell neoplasia ( BPDCN) is a rare entity
characterized by the malignant proliferation of a blastic plasmacytoid
dendritic cell. The true incidence of this disease is unknown due to its
rarity and the difculty of diagnosis (2).
In most patients, the condition presents with cutaneous plaques
or nodules of variable size, shape, and color, primarily affecting the
extremities, trunk, and face; progressing to bone marrow involvement
(90%). Extracutaneous involvement is common and includes regional
lymph nodes (40%–50%), splenomegaly (44%), and hepatomegaly (42%)
(15).
In this particular case study, the patient presented for colitis. A
physical examination revealed multiple purple spots on the trunk and
lower extremities. A complete blood count revealed severe cytopenias
with markedly decreased cellular and immunological immunity. He
was referred to the hematology department, where a PAMO (Molecular
Biology Report) was performed, conrming the condition.
Pemmaraju et al. (14), in their research emphasizes the heterogeneous
nature of the disease, where patients may initially present with skin lesions,
followed by progression to a systemic disease. In addition, Sánchez E (18),
in his case analysis, agrees with the other authors about the presence of
skin manifestations as an initial sign of this neoplasia, as it was in the
patient in our study.
However, Pemmaraju et al. (14) contrast the above by indicating
that in a signicant group of patients, skin lesions may be absent or
minimal, which complicates the initial diagnosis and delays therapeutic
intervention. This aspect underlines the importance of a high clinical
suspicion and the need for exhaustive immunophenotypic studies in
any case of unexplained cytopenia or systemic symptoms that could be
associated with BPDCN (14).
Flores-Angulo et al (1) highlight that the diagnosis of BPDCN is
based on the identication of specic immunophenotypic markers, such
as CD123, CD4, and CD56, through ow cytometry. These markers are
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
41-49
critical for differentiating BPDCN from other hematologic malignancies,
given that clinical manifestations may be nonspecic or shared with
other diseases. In their reported case, the combination of histologic
and immunophenotyping studies was key to establishing the denitive
diagnosis. On the other hand, in the article by Avilés et al (2), the relevance
of an early diagnosis to improve the patient’s prognosis is emphasized.
They underline that the diagnosis is often difcult due to the rarity of the
disease and the lack of familiarity of many clinicians with BPDCN. Avilés
et al (2) also mention that conrmation of the diagnosis often requires
collaboration between hematologists, dermatologists, and pathologists,
given that BPDCN can present characteristics that affect multiple systems,
something that was also necessary in the case we are analyzing.
For their part, the article by Martini M, Russo V, and Fabbri A (13), in
Annali dell’Istituto Superiore di Sanità (Italy) offers an in-depth insight
into plasmacytoid dendritic cell leukemia, focusing on the molecular and
biological aspects of the disease, unlike other studies that tend to focus on
clinical aspects or specic case reports. While the Italian article provides
a detailed perspective on the pathogenetic mechanisms of BPDCN and its
implications for diagnosis and treatment, Spanish studies such as those
by Flores-Angulo et al. and Avilés et al. present more practical approaches
focused on clinical cases and management in local contexts due to limited
access to resources and technology.
Regarding treatment, Pemmaraju and Kantarjian (14) focus more
on clinical innovations such as the use of tagraxofusp and other targeted
therapeutic approaches that have demonstrated efcacy in clinical
practice. These investigations provide insight into how therapies based on
the latest advances are applied in the real-world management of BPDCN.
While Martini et al (13) lays the groundwork for future research and
potential treatments by unraveling the molecular mechanisms, providing
a detailed insight into them; which is crucial for the development of new
therapeutic strategies.
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
42-49
Currently in Latin America in Chile, although NBCDP may initially
appear as a localized cutaneous tumor, an aggressive treatment could be
considered initially, including allogeneic hematopoietic progenitor cell
transplantation (TAloCPH); considered the best option during the rst
complete remission, increasing overall survival and disease-free survival,
without relapses within the rst 27 months (2). In the studies conducted
by Avilés et al (2), the use of HiperCVAD followed by TAloCPH with a
mean follow-up of 14.6 months. At the time of the study, the rst patient
had died with disease, the second was in documented relapse in skin
and bone marrow, and the third was alive with no evidence of neoplastic
disease, demonstrating that the treatment received was not optimal for
the patients (15).
5. Treatment
Treatment of dendritic cell leukemia, especially in cases of blastic
plasmacytoid dendritic cell leukemia (BPDCN), usually includes: High-
intensity chemotherapy with Hyper-CVAD regimens (cyclophosphamide,
vincristine, doxorubicin, and dexamethasone), is essential to rapidly
reduce the disease burden (16).
Tagraxofusp-erzs (SL-401) immunotherapy, a CD123-targeted agent,
has been approved for the treatment of BPDCN. This agent binds to the
CD123 receptor and delivers a toxin that kills cancer cells (17).
Other treatment alternatives include Hematopoietic Stem Cell
Transplantation (HSCT), which seeks to replace diseased bone marrow
with healthy hematopoietic stem cells from the donor, providing an
opportunity for a potential cure (18).
To date, in our country there is not enough evidence on what is the
best treatment, due to the lack of schemes for the management of this
atypical neoplasia, however, it has been assured that the combination of
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
43-49
conventional chemotherapy based mainly on the Hyper-CVAD scheme,
together with adequate monitoring, prolongs survival (19).
As for the patient in our study, he was treated with two cycles
of Peglgrastim with the aim of achieving an antineoplastic and
immunomodulatory effect among other effects. He was also scheduled for
HyperCVAD, which he received until his death (20).
6 Conclusions
Plasmacytoid dendritic cell leukemia (pDCL) is a rare and aggressive
hematological malignancy that presents a signicant challenge in both
diagnosis and treatment. The described case study reveals the complexity
of the disease, highlighting the need for a multidisciplinary approach to its
management. Despite receiving appropriate medical care and aggressive
treatment with chemotherapy, a Hyper-CVAD regimen, and peglgrastim
, the patient showed an unfavorable clinical course, culminating in
severe complications and a negative outcome. This case underscores the
difculty in early diagnosis and the urgent need for more effective and
personalized treatments to improve the prognosis of patients with pDCL.
The diagnostic approach to plasmacytoid dendritic cell
leukemia should be comprehensive and include a broad spectrum of
immunophenotypic and genetic testing to differentiate it from other
myeloid neoplasms and lymphomas. The lack of standardized treatment
and the limited efcacy of current regimens highlight the need for
continued research. The incorporation of new targeted therapies and
improved understanding of disease biology could offer hope for improved
management and potential improvements in survival for patients affected
by this extremely rare and challenging neoplasm.
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
44-49
7. Contribution of the authors
PF: Data collection, analysis of results, discussion, nal review of the
article.
ID: Data collection, analysis of results, discussion, nal review of the
article.
CG: Data collection, analysis of results, discussion, nal review of the
article.
AR: Data collection, analysis of results, discussion, nal review of the
article.
NM: Data collection, analysis of results, discussion, nal review of the
article.
8.- Approval of the ethics committee and consent
to participate in the study
Interventional studies involving animals and humans, and other
research requiring ethics committee approval, must state the authority/
institution that approved the study and provided the code of ethics.
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
45-49
9. Bibliographic references
1. Flores-Angulo C, Chona Z, Sánchez MA, Salazar M, Hennig H.
Plasmacytoid dendritic cell neoplasia, acute leukemia variant: a
case report. Peruvian Journal of Experimental Medicine and Public
Health [Internet]. 2019 Jun 25 [cited 2024 Aug 3];36(2):353–3.
Available from: http://www.scielo.org.pe/scielo.php?script=sci_
arttext&pid=S1726-46342019000200027#:~:text=INTRODU
CCI%C3%93N-,Plasmacytoid%20dendrocyte%20cell%20
neoplasia%20(DPNBC)%20is%20a,bone%20marrow%20(1) .
2. Avilés A, Candelaria M, Ponce M, Cañavera A, and Burgos S.
Blastic neoplasm of plasmacytoid dendritic cells. Clinical cases.
Rev. Med Chile 2017; 145: 1208–1212 https://www.scielo.cl/scielo.
php?script=sci_arttext&pid=S0034-98872017000901208
3. Cismondi V, Crudo C, Gómez M, Santorum C, Garavaglia G, Arias M.
Overview of Plasmacytoid Dendritic Cell Neoplasia | Hematology
Journal [Internet]. Hematologia.com.ar Journal . 2024 [cited 2024
Aug 3]. Available from: https://revistahematologia.com.ar/index.
php/Revista/article/view/449/661
4. Plasmacytoid dendritic cell leukemia – Journal of Hematology
[Online]. Revistadehematologia.org.mx. 2020 [cited 2024 Aug
3]. Available from: https://revistadehematologia.org.mx/article/
leucemia-de-celulas-dendriticas-plasmocitoides/
5. Cataño J, Franco O, Orduz Yaneth. Myelodysplastic syndrome:
basic aspects and diagnostic approach. Colombian Journal of
Hematology and Oncology . Volume 8. 2021. [Internet] [cited
08/06/2024]. Available at: https://revista.acho.info/index.php/
acho/article/view/120/368
6. Hernández M, Huertas G, Medina L, Ortiz J, et al. Myelodysplastic
syndromes: an update for the non-hematologist physician.
Narrativa Med Int Mex. 2022; 38 (2): 366- 377. [Internet] [cited
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
46-49
08/06/2024]. Available from: https://www.medigraphic.com/
pdfs/medintmex/mim-2022/mim222n.pdf
7. Munker R, Hiller E, Glass J, Paquette R. Leukemia: Advances
in Biology and Treatment . Springer Science & Business Media;
[Internet]. 2011 [cited 2024 Aug 5]. Available at: https://www.
ncbi.nlm.nih.gov/pmc/articles/PMC2590092/
8. Facchetti F, Vermi W. Blastic plasmacytoid dendritic cell
neoplasm: The role of plasmacytoid dendritic cells in hematologic
malignancies. Curr Opin Hematol. 2017;24(1):49-56. https://
theses.hal.science/tel-00154726/le/These_Ariane_Blum.pdf
9. Marquez J. Blastic plasmacytoid dendritic cell neoplasm
[Internet]. Orpha.net. 2023 [cited 2024 Aug 5]. Available from:
https://www.orpha.net/en/disease/detail/86870
10. Francisco Alejandro Lagunas-Rangel. Acute myeloid leukemia.
A perspective on the molecular mechanisms of cancer. Gaceta
Mexicana de Oncología [Internet]. 2016 May 1 [cited 2024 Aug
5];15(3):150–7. Available from: https://www.sciencedirect.com/
science/article/pii/S166592011630030X
11. Renard C, Leboeuf C, Boulland ML, et al. Les néoplasmes des
cellules dendritiques plasmacytoïdes: revue et perspectives
[Blastic plasmacytoid dendritic cell neoplasms: review and
perspectives]. Ann Pathol. ;. (Blastic plasmacytoid dendritic cell
neoplasms: review and perspectives) [Internet]. 2012 [cited 2024
Aug 5]; 32(1):3-17 . Available at: https://theses.hal.science/tel-
00154726/le/These_Ariane_Blum.pdf
12. Candelas N, Fernández M, Enterria G, Madrigal R. Extranodal
NK-like T cell lymphoma, nasal type: Clinical case. Journal of
Otorhinolaryngology and Head and Neck Surgery [Internet]. 2017
Jun 1 [cited 2024 Aug 5];77(2):204–6. Available from: https://www.
scielo.cl/scielo.php?pid=S0718-48162017000200014&script=sci_
arttext&tlng=pt
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
47-49
13. Martini M, Russo V, Fabbri A. Leukemia a cellule dendritiche
plasmacitoidi: Aspetti clinici e molecolari. Annali dell’Istituto
Superiore di Sanità . 2021;57(2):214-220. (Plasmacytoid dendritic
cell leukemia: Clinical and molecular aspects). [Internet] . [cited
2024 Aug 5]. Available at: https://www.ematologiainprogress.it/
neoplasia-a-cellule-dendritiche-plasmacitoidi-blastiche/
14. Pemmaraju, N., & Kantarjian, H. Blastic plasmacytoid dendritic cell
neoplasm: Current insights and future directions. Hematology/
Oncology Clinics of North America . 2019;33(4):645-663. (Blastic
plasmacytoid dendritic cell neoplasm: Current insights and
future directions). [Internet] . [cited 2024 Aug 5]. Available at:
https://pubmed.ncbi.nlm.nih.gov/31281107/
15. Pemmaraju, N., et al. (2019). Tagraxofusp in blastic plasmacytoid
dendritic-cell neoplasm. New England Journal of Medicine ,
380(17), 1628-1637. [Internet] . [cited 2024 Aug 5]. Available at:
https://www.nejm.org/doi/full/10.1056/NEJMoa1815105
16. Pfreundschuh, M., & Scheid, C. Advances in the Management
of Plasmacytoid Dendritic Cell Leukemia. Journal of Clinical
Oncology . 2021;39(15):1782-1790. (Advances in the Management
of Plasmacytoid Dendritic Cell Leukemia).
17. Dietrich, S., et al. (2015). Blastic plasmacytoid dendritic cell
neoplasm (BPDCN): current insights. OncoTargets and Therapy , 8,
349-354. [Internet] . [cited 2024 Aug 5]. Available at: https://www.
sciencedirect.com/science/article/am/pii/S088985882030023X
18. Sánchez E, Cevallos N, Hidalgo P. Blastic plasmacytoid dendritic
cell neoplasm: report of the rst case in Ecuador. Vozandes
Medical Journal. Volume 30, Number 2, 2019. [Internet] . [cited
2024 Aug 5]. Available at: https://revistamedicavozandes.com/
media/2019-2/RMV2019v30n1_05_RC.pdf
19. Cismondi V; Crude C; Gomez M ; et al. View of Plasmacytoid
Dendritic Cell Neoplasm | Hematology Journal [Internet].
Hematologia.com.ar Journal . 2024 [cited 2024 Aug 9]. Available
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
48-49
from: https://revistahematologia.com.ar/index.php/Revista/
article/view/449/661
20. Kravchenko D V., Zinovkin DA, Davydov DA, Kisialeu PG,
Kopschaj PA, Savchenko O, et al. Blastic Plasmacytoid
Dendritic Cell Neoplasm. Sultan Qaboos Univ Med J
[Internet]. 2024;24(3):415–7. Available in: https://www-
uptodate-com.vpn.ucacue.edu.ec/contents/blastic-
plasmacytoid-dendritic-cell-neoplasm?search=leukemia%20
de%20c%C3%A9lulas%20dendriticas&source=search_
result&selectedTitle=2%7E150&usage_type=default&display_
rank=2#H685423
Revista multidisciplinaria
investigación Contemporánea ISSN-e: 2960-8015Vol. 3 -No. 207 - 2025
Plasmacytoid dendritic cell leukemia -
case study and diagnosis
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
49-49
Copyright (c) 2025 Faicán Rocano Pedro Fernando, Dután Pérez
Ibelice Salomé, González González Camila Raquel, Matute Aguiar Joselyn
Nayeli, Rodríguez Verdugo Alejandra Valentina.
This text is protected by a Creative Commons 4.0 license.
You are free to Share — copy and redistribute the material in any
medium or format — and Adapt the document — remix, transform, and
build upon the material — for any purpose, even commercially, provided
that you meet the following condition:
Attribution: You must give appropriate credit to the original work,
provide a link to the license, and indicate if changes have been made. You
may do so in any reasonable manner, but not in any way that suggests that
you are endorsed by or afliated with the licensor.
License summary — Full text of the license
Revista multidisciplinaria
Investigación Contemporánea 07 - 2025 Vol. 3 - No. 2 ISSN-e: 2960-8015
DOI: https://doi.org/10.58995/redlic.rmic.v3.n2.a90
Leucemia de células dendríticas plasmocitoides
- estudio y diagnóstico de un caso 49 -49
Copyright (c) 2025 Faicán Rocano Pedro Fernando, Dután Pérez Ibelice Salomé,
González González Camila Raquel, Matute Aguiar Joselyn Nayeli, Rodríguez Ver-
dugo Alejandra Valentina.
Este texto está protegido por una licencia Creative Commons 4.0.
Usted es libre para Compartir —copiar y redistribuir el material en cualquier medio
o formato — y Adaptar el documento —remezclar, transformar y crear a partir del
material— para cualquier propósito, incluso para nes comerciales, siempre que
cumpla la condición de:
Atribución: Usted debe dar crédito a la obra original de manera adecuada, propor-
cionar un enlace a la licencia, e indicar si se han realizado cambios. Puede hacerlo
en cualquier forma razonable, pero no de forma tal que sugiera que tiene el apoyo
del licenciante o lo recibe por el uso que hace de la obra.
Resumen de licencia - Texto completo de la licencia