{"title":"揭开暴风雨的面纱皮肤浆细胞树突状细胞增殖是急性髓性白血病的先兆","authors":"Brandon Zelman, Victor Barragan, Samreen Fathima, Rachit Gupta, Faaris Hanif, Kamran Mirza, Jodi Speiser","doi":"10.1097/DAD.0000000000002822","DOIUrl":null,"url":null,"abstract":"<p><strong>Abstract: </strong>Plasmacytoid dendritic cell neoplasms are rare neoplasms originating from plasmacytoid dendritic cells (pDCs). They are subclassified into 2 types: blastic plasmacytoid dendritic cell neoplasm and mature plasmacytoid dendritic cell proliferation. Neoplastic expansion of pDCs has also been found to be associated with myeloid neoplasia. We present the diagnostically challenging case of a 62-year-old woman who presented to the emergency department with numerous hemorrhagic nodules and papules on the face and extensor surfaces near the elbows and neutropenic fevers. The patient had a history notable for lupus erythematosus and a recently performed excisional lymph node biopsy involved by a \"plasmacytoid dendritic cell proliferation.\" A punch biopsy was performed, which showed a robust dermal infiltrate of atypical intermediate-sized mononuclear cells. The infiltrate was positive for CD4, CD43, and CD123. CD3 and CD8 highlighted background T cells. The infiltrate was negative for CD10, CD34, CD56, CD68, CD117, myeloperoxidase, lysozyme, TdT, and TCL-1. The findings favored a diagnosis of cutaneous involvement of the plasmacytoid dendritic cell proliferation. Given the association with acute leukemias, a subsequent bone marrow biopsy was recommended. The bone marrow biopsy was performed, which showed increased blasts (68% on a 500 differential cell count). Furthermore, immunohistochemical stains were performed, which highlighted the blasts to be positive for CD34 and BEST (alpha-naphthyl butyrate esterase) cytochemical stain. This diagnosis was consistent with bone marrow involvement of acute myelomonocytic leukemia. Given the overlapping presenting symptoms (skin lesions, adenopathy, marrow involvement) of pDC neoplasms and myeloid neoplasia and the possibility of presenting concurrently, increased awareness is of pivotal importance to help prevent potential misdiagnosis, missed diagnosis, and prompt investigation of possible associated neoplasms.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1000,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Unveiling the Tempest: Dermal Plasmacytoid Dendritic Cell Proliferation as the Harbinger of Acute Myeloid Leukemia.\",\"authors\":\"Brandon Zelman, Victor Barragan, Samreen Fathima, Rachit Gupta, Faaris Hanif, Kamran Mirza, Jodi Speiser\",\"doi\":\"10.1097/DAD.0000000000002822\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Abstract: </strong>Plasmacytoid dendritic cell neoplasms are rare neoplasms originating from plasmacytoid dendritic cells (pDCs). They are subclassified into 2 types: blastic plasmacytoid dendritic cell neoplasm and mature plasmacytoid dendritic cell proliferation. Neoplastic expansion of pDCs has also been found to be associated with myeloid neoplasia. We present the diagnostically challenging case of a 62-year-old woman who presented to the emergency department with numerous hemorrhagic nodules and papules on the face and extensor surfaces near the elbows and neutropenic fevers. The patient had a history notable for lupus erythematosus and a recently performed excisional lymph node biopsy involved by a \\\"plasmacytoid dendritic cell proliferation.\\\" A punch biopsy was performed, which showed a robust dermal infiltrate of atypical intermediate-sized mononuclear cells. The infiltrate was positive for CD4, CD43, and CD123. CD3 and CD8 highlighted background T cells. The infiltrate was negative for CD10, CD34, CD56, CD68, CD117, myeloperoxidase, lysozyme, TdT, and TCL-1. The findings favored a diagnosis of cutaneous involvement of the plasmacytoid dendritic cell proliferation. Given the association with acute leukemias, a subsequent bone marrow biopsy was recommended. The bone marrow biopsy was performed, which showed increased blasts (68% on a 500 differential cell count). Furthermore, immunohistochemical stains were performed, which highlighted the blasts to be positive for CD34 and BEST (alpha-naphthyl butyrate esterase) cytochemical stain. This diagnosis was consistent with bone marrow involvement of acute myelomonocytic leukemia. Given the overlapping presenting symptoms (skin lesions, adenopathy, marrow involvement) of pDC neoplasms and myeloid neoplasia and the possibility of presenting concurrently, increased awareness is of pivotal importance to help prevent potential misdiagnosis, missed diagnosis, and prompt investigation of possible associated neoplasms.</p>\",\"PeriodicalId\":50967,\"journal\":{\"name\":\"American Journal of Dermatopathology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.1000,\"publicationDate\":\"2024-11-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"American Journal of Dermatopathology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1097/DAD.0000000000002822\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/8/14 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"DERMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"American Journal of Dermatopathology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1097/DAD.0000000000002822","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/8/14 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"DERMATOLOGY","Score":null,"Total":0}
Unveiling the Tempest: Dermal Plasmacytoid Dendritic Cell Proliferation as the Harbinger of Acute Myeloid Leukemia.
Abstract: Plasmacytoid dendritic cell neoplasms are rare neoplasms originating from plasmacytoid dendritic cells (pDCs). They are subclassified into 2 types: blastic plasmacytoid dendritic cell neoplasm and mature plasmacytoid dendritic cell proliferation. Neoplastic expansion of pDCs has also been found to be associated with myeloid neoplasia. We present the diagnostically challenging case of a 62-year-old woman who presented to the emergency department with numerous hemorrhagic nodules and papules on the face and extensor surfaces near the elbows and neutropenic fevers. The patient had a history notable for lupus erythematosus and a recently performed excisional lymph node biopsy involved by a "plasmacytoid dendritic cell proliferation." A punch biopsy was performed, which showed a robust dermal infiltrate of atypical intermediate-sized mononuclear cells. The infiltrate was positive for CD4, CD43, and CD123. CD3 and CD8 highlighted background T cells. The infiltrate was negative for CD10, CD34, CD56, CD68, CD117, myeloperoxidase, lysozyme, TdT, and TCL-1. The findings favored a diagnosis of cutaneous involvement of the plasmacytoid dendritic cell proliferation. Given the association with acute leukemias, a subsequent bone marrow biopsy was recommended. The bone marrow biopsy was performed, which showed increased blasts (68% on a 500 differential cell count). Furthermore, immunohistochemical stains were performed, which highlighted the blasts to be positive for CD34 and BEST (alpha-naphthyl butyrate esterase) cytochemical stain. This diagnosis was consistent with bone marrow involvement of acute myelomonocytic leukemia. Given the overlapping presenting symptoms (skin lesions, adenopathy, marrow involvement) of pDC neoplasms and myeloid neoplasia and the possibility of presenting concurrently, increased awareness is of pivotal importance to help prevent potential misdiagnosis, missed diagnosis, and prompt investigation of possible associated neoplasms.
期刊介绍:
The American Journal of Dermatopathology offers outstanding coverage of the latest diagnostic approaches and laboratory techniques, as well as insights into contemporary social, legal, and ethical concerns. Each issue features review articles on clinical, technical, and basic science advances and illuminating, detailed case reports.
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