Turkish Journal of Hematology Volume: 37 Issue: 1 / 2020

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BRIEF REPORTDOI: 10.4274/tjh.galenos.2019.2019.0195Turk J Hematol 2020;37:48-52Blastic Plasmacytoid Dendritic Cell Neoplasia: A Single CenterExperienceBlastik Plazmasitoid Dendritik Hücreli Neoplazi: Tek Merkez DeneyimiAhu Senem Demiröz 1 , Cuyan Demirkesen 1 , Ayşe Salihoğlu 2 , Nükhet Tüzüner 11İstanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Department of Pathology, İstanbul, Turkey2İstanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Department of İnternal Medicine, Hematology, İstanbul, TurkeyAbstractBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a raremalignancy with skin tropism. The entity was recently defined and thediagnosis is generally made by skin biopsies. It is necessary to applyappropriate immunohistochemistry to recognize this rare entity. Thereis no consensus on therapy and the survival rates are low. The aim ofthis study is to describe the clinical and histopathological features ofBPDCN. We retrospectively reviewed 8 BPDCN cases of the CerrahpaşaMedical Faculty diagnosed between 2005 and 2019. We documentedthe clinical findings, histopathologic diagnoses, and outcomes. Themean age of the patients was 58.7 years (range=11-86 years), and7 patients were male. The patients presented with erythematous orpurple papules, plaques, and papulonodular or nodular cutaneouslesions. Two had lymphadenomegaly at presentation. In microscopicevaluations, tumor cells infiltrated the entire dermis with a clear-cutsubepidermal Grenz zone in all cases. CD4, CD56, and CD123 were themost frequently expressed immunohistochemical markers. The medianfollow-up of 7 cases was 14 months, ranging from 6 to 48 months.Three patients died of the disease, while 4 patients were still alive. Outof 7 patients, 5 received chemotherapy. We found that the outcomesof some patients were different from others but we did not link anydistinct clinical or histopathological characteristics to these differentoutcomes.Keywords: Acute leukemia, Other leukemia, NeoplasiaÖzBlastik plazmasitoid dendritic hücreli neoplazi (BPDCN) nadir görülen,malign bir tümördür. Bu antite son yıllarda tanımlanmış olup, tanıgenellikle deri biyopsisinde uygun immunhistokimya panelininuygulanması ile konulur. Tedavi konusunda tam bir fikir birliği yokturve beklenen yaşam süresi kısadır. Bu çalışmanın amacı BPDCN’nin klinikve histopatolojik özelliklerinin tanımlanmasına katkıda bulunmaktır.Cerrahpaşa Tıp Fakültesi’nde 2005 ile 2014 yılları arası tanı almış olan8 olgu geriye dönük olarak incelenmiştir. Kliniğe başvuru bulguları,histopatolojik tanı ve klinik gidiş incelenmiştir. Olguların ortalamayaşı 58,7 (11-86) olup, 7 olgu erkektir. Başvuru şikayeti eritematözya da mor renkli papül, plak, papülonodül ya da nodüler derilezyonlarıdır. İki olguda başvuru sırasında lenfadenomegali mevcuttur.Mikroskobik olarak tümör hücreleri subepidermal grenz zon bırakaraktüm dermisi infiltre etmektedir. CD4, CD56 ve CD123 en sık ekspreseolan immünhistokimyasal işaretleyicilerdir. Yedi olguda median takipsüresi 14 ay (6-48 ay) olup 3 olgu hastalığa bağlı kaybedilmiş, 4 olguhayattadır. Yedi olgunun 5 tanesi kemoterapi almıştır. Olguların birkısmında diğerlerine göre farklı klinik gidiş gözlenmiştir, ancak bu durumincelenen klinik ve histokimyasal bulgularla ilişkilendirilememiştir.Anahtar Sözcükler: Akut lösemi, Diğer lösemi, NeoplaziIntroductionBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is aclinically aggressive tumor derived from the precursors ofplasmacytoid dendritic cells (PDCs) with a high frequencyof cutaneous and bone marrow involvement and leukemicdissemination [1]. The tumor is considered among the “acutemyeloid leukemia and related neoplasms” since 2008 [2] andrecently it was reclassified as a separate entity in the latestWorld Health Organization classification scheme. The tumor cellstypically express CD4, CD56, CD43, CD45RA, and plasmacytoiddendritic cell antigens (CD123, CD303, TCL1A, CD2AP, SPIB, andtype 1 interferon-dependent molecule MX1) [3]. The molecularprofile showed that this entity is much more related to myeloidneoplasms [4].In this study, we present 8 patients with BPDCN from a singlecenter to emphasize the clinical and histopathological featuresof this rare entity.©Copyright 2020 by Turkish Society of HematologyTurkish Journal of Hematology, Published by Galenos Publishing HouseAddress for Correspondence/Yazışma Adresi: Ahu Senem Demiröz, M.D., İstanbul University-Cerrahpasa,Cerrahpasa Faculty of Medicine, Department of Pathology, İstanbul, TurkeyPhone : +90 505 441 81 73E-mail : ahusenem@yahoo.com ORCID: orcid.org/0000-0002-8370-1130Received/Geliş tarihi: May 17, 2019Accepted/Kabul tarihi: November 19, 201948
Turk J Hematol 2020;37:48-52Demiröz AS, et al: Blastic Plasmacytoid Dendritic Cell Neoplasia: A Single Center ExperienceMaterials and MethodsEight cases of BPDCN were retrieved from the archives ofthe Department of Pathology of the Cerrahpaşa MedicalFaculty. Data regarding the clinical features and follow-upwere obtained from the patients’ records and their attendingphysicians. We retrospectively reviewed hematoxylin and eosinand immunohistochemical (IHC) stained slides of skin and bonemarrow biopsies. All IHC stainings were performed on a VENTANABenchMark automated staining system using 4-µm paraffintissue sections. The primary antibodies used in this study wereCD4 (ready to use, Novocastra), CD56 (1:250, Cell Marque), CD123(1:50, Novocastra), MPO (1:600, DAKO), CD68 (1:400, Novocastra),TdT (1:400, Thermo Scientific), CD7 (1:40, Thermo Scientific), CD20(1:250, Thermo Scientific), and CD3 (1:300, Novocastra).ResultsThe clinical features of the 8 cases are summarized in Table 1.The median follow-up of 7 cases was 14 months (ranging from2 to 48 months). Three patients died of the disease, while 4were still alive. Of 7 patients, 5 received chemotherapy. Threeof them (patients 4, 6, and 7), given the hyper-CVAD regimen,were in remission during the follow-up period. One (patient 5)was given the hyper-CVAD regimen fortified with methotrexateand cytarabine, but he died 8 months after chemotherapy dueto systemic involvement. One (the child, patient 8) was givena BFM-ALL high-risk regimen; he was in remission 11 monthsafter the initial diagnosis.Tumor cells infiltrated the entire dermis with a subepidermalGrenz zone in all cases. In one case, the infiltration reached thesubcutaneous fat tissue. The infiltration pattern was diffuse in5 of the cases, patchy in 1 case, and both diffuse and patchyin 2 cases. Tumor cells were medium-sized with fine chromatinresembling lymphoblasts. Cytoplasm was variably abundantand lacked granules. Mitotic activity was scored as 0-3 in onehigh-power field (HPF) in 7 cases. A patient with subcutaneousinfiltration (14.2%, patient 3) had 2-5 mitotic findings per HPF.Necrosis, vascular invasion, and angiotropism were not detectedin any of the cases.The IHC features are summarized in Table 2.DiscussionBPDCN is a recently described entity with an aggressive course.There are only a few series published in the literature. Thelargest such series comprised 91 patients [5]. This disease is stillan obscure entity with many unknowns.It is typically seen in middle-aged or elderly men, althoughpediatric cases have also been rarely reported. The age rangein our series was 11-86 years with a mean age of 58.7 years. Of8 patients, one was a child (14.2%). The male:female ratio was2.5-3:1 [2,6,7,8].Table 1. Patient characteristics, clinical data, and outcomes.Case Age/Sex Cutaneous lesion Size(cm)1 86/M Papulonodular and tumorallesions on the trunk and scalp2 73/F Purple erythematous papulesand plaques on the chest, back,and extremities3 71/M Multiple purplish or skincoloredpapules/nodules4 59/M Purple infiltrated noduleson the abdomen and back,palpable papules on the leg5 73/M Multiple purple-red nodulesand plaques on the face, back,and chest6 57/M Maculopapular lesions onchest, back, and shoulder7 40/M Indurated nodular lesions onthe extremities and chest8 11/M Red macular bruise-like lesionon the legExtracutaneouslesionBonemarrowinfiltrationTreatmentFollowup(months)5 Absent Absent NAa 2 DOD0.5-1.5 Absent NAb NA Lost tofollowup0.5-2 Absent Absent NAa 4 DOD10 Absent Present Hyper-CVADregimen2-15 Absent Absent Hyper-CVAD regimenwith methotrexateand cytarabineOutcomeNA48 NED8 DOD0.5-1 Cervical LAM Absent Hyper-CVAD regimen 36 NED3.5 Cervical andsubmandibular LAMDOD: Dead of disease; NA: Not applicable; NED: No evidence of disease; LAM: Lymphadenomegaly.a These patients could not receive chemotherapy and died immediately after the initial diagnosis.b The patient refused bone marrow biopsy.Absent Hyper-CVAD regimen 12 NED3.5 Absent Present BFM-ALL high-riskregimen11 NED49
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