Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive neoplasm classified under "acute myeloid leukemia (AML) and related precursor neoplasm" by current WHO classification. Elderly male are commonly affected with cutaneous lesion being the hallmark of disease presentat...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2015-01-01
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| Series: | Indian Journal of Pathology and Microbiology |
| Subjects: | |
| Online Access: | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2015;volume=58;issue=3;spage=377;epage=380;aulast=Qayoom |
| _version_ | 1818254621437591552 |
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| author | Sumaira Qayoom Garima Durga Seena George Khaliqur Rahman |
| author_facet | Sumaira Qayoom Garima Durga Seena George Khaliqur Rahman |
| author_sort | Sumaira Qayoom |
| collection | DOAJ |
| description | Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive neoplasm classified under "acute myeloid leukemia (AML) and related precursor neoplasm" by current WHO classification. Elderly male are commonly affected with cutaneous lesion being the hallmark of disease presentation. The disease progresses rapidly and sooner or later involves bone marrow and peripheral blood. Cases presenting primarily as leukemia without cutaneous involvement is a rarity with about 29 cases reported in literature till date. Characteristic immunophenotype of CD4 + /CD56 +/− cells expressing antigens associated with plasmacytoid dendritic cells like CD123, TCL1, BDCA2/CD303, cutaneous lymphocyte-associated and interferon dependent molecule MxA, in absence of any other lineage specific marker confirms the diagnosis. The disease has a poor survival and no standardized therapeutic strategy in the current scenario. A case of 25-year-male presenting with leukemic BPDCN without cutaneous involvement is presented here, who was treated with AML like protocol followed by hematopoietic stem cell transplantation, but succumbed to the disease within 8 months of diagnosis. The present case is being first to be reported from India. |
| first_indexed | 2024-12-12T16:58:53Z |
| format | Article |
| id | doaj.art-51f93de9606548b2a8c7e3202535cd45 |
| institution | Directory Open Access Journal |
| issn | 0377-4929 |
| language | English |
| last_indexed | 2024-12-12T16:58:53Z |
| publishDate | 2015-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Pathology and Microbiology |
| spelling | doaj.art-51f93de9606548b2a8c7e3202535cd452022-12-22T00:18:10ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292015-01-0158337738010.4103/0377-4929.162912Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entitySumaira QayoomGarima DurgaSeena GeorgeKhaliqur RahmanBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive neoplasm classified under "acute myeloid leukemia (AML) and related precursor neoplasm" by current WHO classification. Elderly male are commonly affected with cutaneous lesion being the hallmark of disease presentation. The disease progresses rapidly and sooner or later involves bone marrow and peripheral blood. Cases presenting primarily as leukemia without cutaneous involvement is a rarity with about 29 cases reported in literature till date. Characteristic immunophenotype of CD4 + /CD56 +/− cells expressing antigens associated with plasmacytoid dendritic cells like CD123, TCL1, BDCA2/CD303, cutaneous lymphocyte-associated and interferon dependent molecule MxA, in absence of any other lineage specific marker confirms the diagnosis. The disease has a poor survival and no standardized therapeutic strategy in the current scenario. A case of 25-year-male presenting with leukemic BPDCN without cutaneous involvement is presented here, who was treated with AML like protocol followed by hematopoietic stem cell transplantation, but succumbed to the disease within 8 months of diagnosis. The present case is being first to be reported from India.http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2015;volume=58;issue=3;spage=377;epage=380;aulast=QayoomBlastic plasmacytoid dendritic cell neoplasmflow cytometryplasmacytoid dendritic cell leukemia |
| spellingShingle | Sumaira Qayoom Garima Durga Seena George Khaliqur Rahman Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity Indian Journal of Pathology and Microbiology Blastic plasmacytoid dendritic cell neoplasm flow cytometry plasmacytoid dendritic cell leukemia |
| title | Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity |
| title_full | Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity |
| title_fullStr | Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity |
| title_full_unstemmed | Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity |
| title_short | Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity |
| title_sort | blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient unusual presentation of a rare entity |
| topic | Blastic plasmacytoid dendritic cell neoplasm flow cytometry plasmacytoid dendritic cell leukemia |
| url | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2015;volume=58;issue=3;spage=377;epage=380;aulast=Qayoom |
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