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Case 1. Macroscopic view of the skin tumors. Multiple infiltrating erythemas on the trunk and arms (1a & 1b).
Source publication
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive tumor derived from the precursor of plasmacytoid dendritic cells. We describe two cases of BPDCN. In case 1, the patient presented with multiple erythema on the trunk and arms. Histopathology of a skin biopsy specimen and immunohistochemistry demonstrated that the tumor...
Context in source publication
Context 1
... 86-year-old, previously healthy Japanese man was admitted because of a 6-month history of cutaneous lesions located on the trunk and arms. Physical examination showed multiple areas of infiltrating erythema without other symp- toms ( Fig. 1a & 1b). Superficial lymph nodes were not palpa- ble on physical examination. Histological examination of a biopsy specimen taken from plaque on the trunk revealed the presence of a dermal lymphomatous infiltrate with an alveolar growth pattern, which extended into the subcutis. The epider- mis was not involved, and a grenz zone was present ...
Citations
... Literature on radiotherapy in BPDCN is scarce, with only few reports detailing radiotherapy regimens, regardless of curative or palliative settings. In our literature review, we identified 19 publications with which reported on local radiotherapy as the only or part of the first-line treatment in 47 patients with cutaneous BPDCN lesions (Table 2; [11,13,14,[28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43]). With the exception of five patients aged 23, 32, 36, 38 and 43 years, the age range was 59-90 years, corresponding to the median age of onset of 65 years. ...
... All other publications also describe favorable responses to radiotherapy, with an at least transient partial or complete response of irradiated cutaneous nodules. Of the few publications giving detail on radiation protocols, one reported a cumulative dose of 27.0 in single fractions of 3.0 Gy [14], to which a complete response could initially be observed. However, relapse occurred 2 months after radiation treatment. ...
... In a palliative setting, such as in our case, the benefit of a higher radiation dose should be weighed against the comparatively long treatment time of up to over 3 weeks [12][13][14]30], as longer treatment time and trips to the hospital can cause strain for patients. Moreover, in our case, systemic disease occurred before skin lesions, which developed after systemic therapy had already been initiated. ...
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that can manifest with skin nodules and erythematous plaques. In most cases BPDCN progresses rapidly, causing multiple skin lesions and also affecting internal organs and bone marrow, warranting initiation of systemic therapies or hematopoietic stem cell transplantation (HCT). Although not curative, radiotherapy for isolated lesions might be indicated in case of (imminent) ulceration and large or symptomatic lesions. To this end, doses of 27.0–51.0 Gy have been reported. Here, we present the case of an 80-year-old male with BPDCN with multiple large, nodular, and ulcerating lesions of the thorax, abdomen, and face. Low-dose radiotherapy of 2 × 4.0 Gy was administered to several lesions, which resolved completely within 1 week with only light residual hyperpigmentation of the skin in affected areas and reliably prevented further ulceration. Radiotoxicity was not reported. Therefore, low-dose radiotherapy can be an effective and low-key treatment in selected cases of BPDCN, especially in a palliative setting, with a favorable toxicity profile.
... Furthermore, additional immunostaining of cutaneous lymphocyte-associated antigen (CLA), CD43, and terminal deoxynucleotidyl transferase (TdT) often facilitates BPDCN diagnosis. 4,[6][7][8] Previously, some prognostic factors for BPDCN, including age, tumor involvement at the time of diagnosis, immunophenotype, and genetic abnormality, have also been reported. 3,5,7,9 Of note, early diagnosis is essential to determine the state of disease and devise an appropriate treatment plan. ...
... Although BPDCN cells are often lymphoblast-like, they are occasionally myeloid-like. 8,17 Cytologically, neoplastic cells had round to oval nuclei, finely dispersed chromatin, prominent nucleoli, scant pale-blue agranular cytoplasm, occasional cytoplasmic microvacuoles, and pseudopodiashaped cytoplasmic extensions. 4 In this study, minimal BM involvement was identified as small clusters of neoplastic cells expressing CD123 and/or CD303, which was not easily recognized in BM clots stained by H&E (case 1, 4 & 5). ...
Blastic plasmacytoid dendritic cell (pDC) neoplasm (BPDCN) is a relatively rare hematological malignancy with significantly complex clinicopathological features that are still unclear. This study aimed to analyze the clinicopathological data of BPDCN and evaluate immunohistochemical detection of minimal bone marrow (BM) involvement. In this study, we examined skin and BM lesions from 6 patients with BPDCN. Neoplastic cells tested positive for CD303 (polyclonal, 100%; monoclonal, 40%) in the skin lesions and for CD303 (polyclonal, 100%; monoclonal, 67%) in the BM clots. Although immunostaining of CD4, CD56, CD123, CD303, and TCLl detected minimal BM involvement in 3 patients, morphological identification was challenging in the BM clots stained with hematoxylin-eosin. In conclusion, our results demonstrate the significance of observing BM smears to detect neoplastic cells and that immunohistochemical examination, including CD303 antibodies, is useful to detect minimal BM involvement. This study is the first to report the expression of thymic stromal lymphopoietin (TSLP) and its receptor in BPDCN cells. Therefore, the TSLP/TSLP receptor axis may be associated with the proliferation of BPDCN, and consequently, the survival of patients.
... Only 19 patients with LS-BPDCN have been reported in the literature since the latest classification of BPDCN in 2008, [3][4][5][6][7][8][9][10][11][12] (their data are summarized in Table I). Median age of the reported patients at presentation was 46 years (range, 5-83), which is younger than the median age documented for all patients with BPDCN (67.8 years). 2 None of the reports mentioned time to diagnosis from initial evaluation by a clinician. ...
... The clinical hallmarks of BPDCN are predominant cutaneous involvement (nodules and / or plaques in color from pink to purple and hard-elastic consistency), with subsequent or simultaneous extension to bone marrow and peripheral blood (in advanced progression to leukemia or in case of relapse) [4]. However, in a minority of cases, it may appear from the start as a fulminant leukemia in the absence of cutaneous manifestations. ...
... The differential diagnosis includes primary anaplastic large-cell lymphoma (ALCL) and primary cutaneous large B-cell Lymphoma (DLBCL). ALCL frequently involves both lymph nodes and extranodal tissues, including skin, bones and soft tissues [4]. The hallmark of primary cutaneous ALCL cells is the expression of CD30, but in BPDCN cells there is no expression of CD30. ...
... In the current case, CD30 was negative and there was no involvement of lymph nodes and extranodal tissues. DLBLC primary affects lower legs, but 10-15% of other cases arise to other sites [4]. Negativity for B-cell lineage markers (CD20 and CD79a) distinguishes BPDCN from primary cutaneous DBLCL. ...
Blastic plasmacytoid dendritic cell neoplasm is a rare, highly aggressive hematopoietic malignancy that is characterized by the clonal proliferation of immature or precursors of plasmacytoid dendritic cells. We report the case of 70-year-old woman affected by blastic plasmacytoid dendritic cell neoplasm who died despite 6 months of chemotherapy. [J Interdiscipl Histopathol 2013; 1(2.000): 98-101]
... Confusion can also arise as the CD4 antigen may be expressed in 82% by leukemic monocytes together with CD56 antigen which can be positive in up to 71% of monocytic blast cells. In such cases beside CD4 and CD56 specific dendritic cell markers CD123 and Tcl-1, CD303 and CD2AP should be used to make differential diagnosis between these two entities (Herling and Jones, 2007;Cronin et al., 2012;Petrella et al., 2002;Herling et al., 2003;Tsunoda et al., 2012;Chang et al., 2010). The expression, at least three of those markers are necessary for the diagnosis of BPDCN. ...
... The disease typically involves the skin at presentation, later spreading to the bone marrow, lymph nodes and blood (Herling and Jones, 2007;Cronin et al., 2012). The cutaneous nodules normally express CD4+/CD56+, and are usually negative for CD3, CD20, MPO, CD13, and CD33 (Herling and Jones, 2007;Cronin et al., 2012;Khoury et al., 2002;Tsunoda et al., 2012;Chang et al., 2010). Though a disease of the elderly, CD4+/CD56+ BPDCN can also occur in younger patients and even children. ...
Blastic dendritic plasmacytoid cell neoplasm is a rare aggressive tumor of plasmacytoid dendritic cells. We report a 38-year-old female with nodular skin lesions on both legs at presentation which on immunohistochemical staining were CD4+ and CD56+ responding to blastic plasmacytoid dendritic cell neoplasm. Five months later the bone marrow analysis showed infiltration with 80% blasts which on flowcytometry had the immunophenotype suggesting acute myelomonocytic leukemia. Additional staining of skin lesions with CD123 was negative excluding blastic dendritic plasmacytoid cell neoplasm and suggesting the diagnosis of leukemia cutis. The patient achieved a complete remission after first course of standard induction chemotherapy, but relapsed 2 months later with leukemic manifestations and skin lesions. She died 13 months after initial diagnosis. This case illustrates the significance of immunophenotyping in differential diagnosis of blastic plasmacytoid dendritic cell neoplasm and myeloid leukemia cutis.
We present a case of 76-year-old man with a medical history of laryngeal cancer, rectal cancer, and rheumatoid arthritis. Two weeks before the first visit, a reddish tumor appeared on his left abdominal skin, and he was referred to our department. Indurated erythema was observed with a major axis of 5 cm on the left abdominal skin, and a hemispherical dark reddish tumor was noted with a major axis of 2 cm on the erythematous lesion. Histopathological examination of skin biopsy revealed large, well-defined atypical cells with nucleoli proliferating throughout the dermis. Immunostaining revealed positive findings for CD4, CD56, CD123, and TCL1, and we diagnosed the patient with a blastic plasmacytoid dendritic cell neoplasm. CHOP chemotherapy was performed. It was initially effective for the skin lesions, but gradually became refractory to treatment. Over time, many reddish tumors and indurated erythema appeared on the trunk and limbs. Radiation therapy performed for the skin lesions was effective in local control. Although this disease is extremely rare, it is a fatal disease that transfers to the leukemic phase at a high rate. Most cases develop from skin lesions, so dermatologists should be able to recognize this entity. In general, radiation therapy for blastic plasmacytoid dendritic cell tumors is effective in local control of skin lesions, although it does not contribute to prolonged prognosis.
Case 1 : An 85-year-old woman presented with a tumor on her left knee that had appeared 5 months before, followed by many papules and nodules on her face and limbs. Histopathology and immunohistochemistry of the skin biopsy sample led to the diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Case 2 : A 91-year-old man presented with excessive purplish erythema on his trunk that had appeared 7 months before. Case 3 : A 67-year-old man presented with many papules and nodules on his limbs and trunk that had appeared a month before. These cases suggest that there are a variety of clinical features of BPDCN. We reviewed previous reports of BPDCN from the last 10 years. The clinical features of BPDCN can be divided into two types, namely, features associated with nodules and tumors and features associated with erythema and purpura. Cases with multiple nodules and tumors tend to be associated with poor prognosis.[Skin Cancer (Japan) 2018 ; 33 : 185-191]
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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy characterized by frequent skin involvement that most commonly affects older patients. BPDCN is known to have a poor prognosis. Our objective was to assess if outcome and disease prognosis were independently influenced by age when evaluated with clinical presentation, sex, and treatment regimens. We conducted a systematic review to identify BPDCN cases, to compare pediatric BPDCN cases with adult cases. A total of 125 publications were identified detailing 356 cases. Including 1 pediatric case from our institution, 74 were children, and 283 were adults aged 19 or over. Age was shown to be an independent prognostic factor predictive of more favorable outcomes across measures including initial response to therapy, likelihood of relapse, and overall survival at follow-up. The distribution of affected organs at diagnosis was similar across children and adults and type of clinical presentation did not disproportionately influence 1 age group's prognosis over the other. Acute lymphoblastic leukemia-type chemotherapy regimens were shown to be superior to other chemotherapy regimens (acute myeloid leukemia, lymphoma, acute lymphoblastic leukemia/lymphoma, other, or none) in inducing complete remission. Allogeneic stem cell transplantation was shown to increase mean survival time. Future research may be directed toward elucidating the further morphologic, cytogenetic, and cytochemical differences between younger and older BPDCN patients.
