Abstract:Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive clinical course and poor prognosis. Diagnosis is based on detection of CD4+ CD56+, CD123high, TCL-1+, and blood dendritic cell antigen-2/CD303+ blasts, together with the absence of lineage specific antigens on tumour cells. In this report we present a case of BPDCN presenting with extramedullary and bone marrow involvement, extensively studied by flow cytometry and immunohistochemistry, who achieved complete r… Show more
“…Although histopathology showing lymphoblasts or small immunoblots supports the presence of a neoplastic reaction, it requires IHC staining to verify the diagnosis and distinguish this malignancy from its differential diagnoses [ 17 , 18 ]. The differential diagnoses of BPDCN include mature T-cell lymphoma, myeloid sarcoma, acute myeloid leukemia, and T-cell lymphoblastic lymphoma [ 19 ].…”
Background
Blastic plasmacytoid dendritic cell neoplasm represents a rare type of hematologic malignancy that often manifests itself through various skin lesions. It commonly affects the elderly male population. Lymph nodes, peripheral blood, and bone marrow involvement are the typical findings that justify its aggressive nature and dismal prognosis. On histopathological assessment, malignant cells share some similarities with blastic cells from the myeloid lineage that make immunohistochemistry staining mandatory for blastic plasmacytoid dendritic cell neoplasm diagnosis.
Case presentation
A 35-year-old Asian man presented with cervical lymphadenopathy followed by an erythematous lesion on his left upper back. At first, the lesion was misdiagnosed as an infectious disease and made the patient receive two ineffective courses of azithromycin and clarithromycin. Six months later, besides persistent skin manifestations, he felt a cervical mass, which was misdiagnosed as follicular center cell lymphoma. Tumor recurrence following the chemoradiation questioned the diagnosis, and further pathologic assessments confirmed blastic plasmacytoid dendritic cell neoplasm. The second recurrence occurred 3 months after chemotherapy. Eventually, he received a bone marrow transplant after complete remission. However, the patient expired 3 months after transplant owing to the third recurrence and gastrointestinal graft versus host disease complications.
Conclusions
Early clinical suspicion and true pathologic diagnosis play a crucial role in patients’ prognosis. Moreover, allogenic bone marrow transplant should be performed with more caution in aggressive forms of blastic plasmacytoid dendritic cell neoplasm because of transplant side effects and high risk of cancer recurrence.
“…Although histopathology showing lymphoblasts or small immunoblots supports the presence of a neoplastic reaction, it requires IHC staining to verify the diagnosis and distinguish this malignancy from its differential diagnoses [ 17 , 18 ]. The differential diagnoses of BPDCN include mature T-cell lymphoma, myeloid sarcoma, acute myeloid leukemia, and T-cell lymphoblastic lymphoma [ 19 ].…”
Background
Blastic plasmacytoid dendritic cell neoplasm represents a rare type of hematologic malignancy that often manifests itself through various skin lesions. It commonly affects the elderly male population. Lymph nodes, peripheral blood, and bone marrow involvement are the typical findings that justify its aggressive nature and dismal prognosis. On histopathological assessment, malignant cells share some similarities with blastic cells from the myeloid lineage that make immunohistochemistry staining mandatory for blastic plasmacytoid dendritic cell neoplasm diagnosis.
Case presentation
A 35-year-old Asian man presented with cervical lymphadenopathy followed by an erythematous lesion on his left upper back. At first, the lesion was misdiagnosed as an infectious disease and made the patient receive two ineffective courses of azithromycin and clarithromycin. Six months later, besides persistent skin manifestations, he felt a cervical mass, which was misdiagnosed as follicular center cell lymphoma. Tumor recurrence following the chemoradiation questioned the diagnosis, and further pathologic assessments confirmed blastic plasmacytoid dendritic cell neoplasm. The second recurrence occurred 3 months after chemotherapy. Eventually, he received a bone marrow transplant after complete remission. However, the patient expired 3 months after transplant owing to the third recurrence and gastrointestinal graft versus host disease complications.
Conclusions
Early clinical suspicion and true pathologic diagnosis play a crucial role in patients’ prognosis. Moreover, allogenic bone marrow transplant should be performed with more caution in aggressive forms of blastic plasmacytoid dendritic cell neoplasm because of transplant side effects and high risk of cancer recurrence.
“…9 Chromosomal losses and deletions in 5q, 12p13, 13q21, 6q23-ter, and 9 have also been observed in BPDCN cells. 10,11 Inactivation of the tumour suppressors retinoblastoma-associated protein (RB1), tumour protein 53 (TP53) and cyclindependent kinase inhibitor 2A (CDKN2A), and activation of the oncogenes NRAS proto-oncogene, GTPase (NRAS), and KRAS proto-oncogene, GTPase (KRAS) has also been observed in many patients with BPDCN. 12 The primary therapy that most patients with BPDCN receive is an acute lymphocytic leukaemia (ALL)-based or acute myeloid leukaemia (AML)-based chemotherapy regimen, after which, patients tend to rapidly achieve complete remission.…”
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy that derives from plasmacytoid dendritic cells. The cancer is characterized by aggressive development and dismal prognosis, and due to limited prospective study, there is currently no established standard treatment. In this study, the case of a 77-year old female patient with BPDCN, who presented with a cutaneous lesion on the right of her face, is described. The lesion developed into a serious ulcer due to rapid disease progress, thus, a surgical excision was performed. As the patient refused to receive radiotherapy or haematopoietic stem cell transplantation following surgery, a new palliative combination chemotherapy was administered, comprising gemcitabine, nedaplatin and bleomycin. The therapy gave satisfactory results in terms of short-term treatment response and was well-tolerated. Published literature regarding BPDCN is also reviewed.
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