Abstract A 7-year-old girl was admitted to Xiangya Hospital due to systemic lymphadenectasis for 2 months and skin ecchymosis for 3 days. Nine months ago, the girl experienced painless nodules in the left lower extremity with no apparent causes. Three months later, dermatorrhagia and ecchymosis occurred in many regions such as the periocular areas, conjunctiva, oral mucosa, perineal area, and groin, with a "raccoon sign" in both eyes;superficial lymphadenectasis and hepatosplenomegaly were also observed in many regions. The pathological sections for the skin nodules showed malignant tumors in lymphohematopoietic cells, and in combination with clinical manifestations, immunohistochemistry, and positive results for CD4, CD56, and CD123 by bone marrow flow cytometry, the girl was diagnosed with blastic plasmacytoid dendritic cell neoplasm. Then high-risk ALL regimen was applied as the chemotherapy for this girl. At present, the girl has been followed up for 3 months;ecchymosis has disappeared, and the enlarged lymph nodes have shrunk. No abnormal cells have been found in bone marrow morphological examination, and bone marrow flow cytometry has shown that primitive precursor cells account for 1.5% and express CD33, CD34, CD123, and CD117.
Au WY, Weisenburger DD, Intragumtornchai T, et al. Clinical differences between nasal and extranasal natural killer/T-cell lymphoma:a study of 136 cases from the International Peripheral T-Cell Lymphoma Project[J]. Blood, 2009, 113(17):3931-3937.
Yu G, Wang W, Han Y, et al. Blastic plasmacytoid dendritic cell neoplasm presenting with a cutaneous tumor alone as the first symptom of onset:a case report and review of literature[J]. Oncol Lett, 2015, 9(2):819-821.
[4]
Pongpruttipan T, Kummalue T, Bedavanjia A, et al. Aberrant antigenic expression in extranodal NK/T-cell lymphoma:a multiparameter study from Thailand[J]. Diagn Pathol, 2011, 6:79.
[5]
Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome:a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)[J]. Blood, 2007, 110(6):1713-1722.
[6]
Cota C, Vale E, Viana I, et al. Cutaneous manifestations of blasticplasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients[J]. Am J Surg Pathol, 2010, 34(1):75-87.
[7]
Lokare A, Nikolousis E, Phillips N, et al. Reduced intensity allogeneicstem cell transplant for treatment of blastic plasmacytoid dendritic cell neoplasm[J]. Hematol Rep, 2014, 6(1):5119.
[8]
Sakashita K, Saito S, Yanagisawa R, et al. Usefulness of allogeneic hematopoietic stem cell transplantation in first complete remission for pediatric blastic plasmacytoid dendritic cell neoplasm with skin involvement:a case report and review of literature[J]. Pediater Blood Cancer, 2013, 60(11):140-142.
[9]
Jegalian AG, Buxbaum NP, Facchetti F, et al. Blastic plasmacytoid dendritic cell neoplasm in children:diagnostic features and clinical implications[J]. Haematologica, 2010, 95(11):1873-1879.
[10]
Julia F, Petrella T, Barry MB, et al. Blastic plasmacytoid dendritic cell neoplasm:clinical features in 90 patients[J]. Br J Dermatol, 2013, 169(3):579-586.
[11]
Unteregger M, Valentin A, Cerwenka-Zinke W, et al. Unrelated SCT induces long-term remission in patients with blastic plasmacytoid dendritic cell neoplasm[J]. Bone Marrow Transplant, 2013, 48(6):799-802.
[12]
Wang H1, Cao J, Hong X, et al. Blastic plasmacytoid dendritic cell neoplasm without cutaneous lesion at presentation:case report and literature review[J]. Acta Haematol, 2010, 127(2):124-127.
[13]
Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of heamatopioetics and lymphoid tissues[M]. France:IARC Press, 2008:145-147.
[14]
Julia F, Dalle S, Duru G, et al. Blastic plasmtoid dendritic cell neoplasms clinico-immunoistochemical correlations in a series of 91 patients[J]. Am J Surg Pathol, 2014, 38(5):673-680.
[15]
Adachi M, Maeda K, Takekawa M, et al. High expression of CD56 (N-CAM) in a patient with cutaneous CD4-positive lymphoma[J]. Am J Hematol, 1994, 47(4):278-282.
[16]
Wiesner T, Obenauf AC, Cota C, et al. Alterations of the cellcycle inhibitors p27(KIP1) and p16(INK4a) are frequent in blastic plasmacytoid dendritic cell neoplasms[J]. Invest Dermatol, 2010, 130(4):1152-1157.
[17]
Atalay F, Demircl GT, Bayrangurler D, et al. Blastic plasmacytoid dendritic cell neoplasm:skin and bone marrow infiltration of three cases and the review of the literature[J]. Indian Hematol Blood, 2015, 31(2):302-306.
[18]
Hwang K, Park CJ, Jang S, et al. Immunohistochemical analysis of CD123, CD56, and CD4 for the diagnosis of minimal bone marrow involvement by blasticplasmacytoid dendritic cellneoplasm[J]. Histopathology, 2013, 62(5):764-770.
[19]
Dharmani PA, Mittal NM, Subramanian PG, et al. Blastic plasmacytoid dendritic cell meoplasm:Report of two pediatric cases[J]. Indian J Pathol Microbiol, 2015, 58(1):72-76.
[20]
Clovic N, Vidovic A, Tomin D, et al. Acute myelomonocytic leukemia presenting as CD4+/CD56+ blastic plasmacytoid dendritic cell neoplasm[J]. Med Pract Rev, 2013, 4(2):9-13.
[21]
Danielle MP, Kaaren K, Tracy I, et al. Immunophenotypic analysis of myeloperoxidase-negative leukemia cutis and blastic plasmacytoid dendritic cell neoplasm[J]. Am J Clin Pathol, 2012, 137(3):367-376.
[22]
Hansford JR, Kotecha RS, Jevon G, et al. Efficacy of acute myeloid leukemia therapy without stem-cell transplantationin a child with blastic plasma cytoid dendritic cell neoplasm[J]. Haematologica, 2013, 98(3):e30.
[23]
Boers JE, KluinPM, VerdonckLF, et al. Allogeneic stemcell transplantation for blastic plasmacytoid dendritic cell neoplasm[J]. J Clin Oncol, 2012, 30(8):102-103.
[24]
Pagano L, Valentini CG, PlusoniA, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation:anltalian multicenter study[J]. Haematologica, 2013, 98(2):239-245.
[25]
Dalle S, Beylot-Barry M, Bagot M, et al. Blastic plasmacytoid dendritic cell neoplasm:is transplantation the treatment of choice[J]. Br J Dermatol, 2010, 162(1):74-79.
