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CORRESPONDENCE
Year : 2017  |  Volume : 13  |  Issue : 6  |  Page : 1056-1058

Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous lesion


1 Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Pathology, King George's Medical University, Lucknow, Uttar Pradesh, India
3 Department of Pathology, Sahara Hospital, Lucknow, Uttar Pradesh, India

Date of Web Publication13-Dec-2017

Correspondence Address:
Dr. Sumaira Qayoom
Department of Pathology, King George's Medical University, Lucknow, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.220358

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 > Abstract 

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy, recognized as a distinct entity in the WHO 2008 classification of hematolymphoid neoplasm. Described for the first time in 1994 as CD4+ cutaneous lymphoma with high expression of CD56, BPDCN has been known previously with various names such as blastic natural killer (NK) leukemia/lymphoma, agranular CD4+ CD56+ hematodermic neoplasm, and agranular CD4+ NK cell leukemia. This disease usually presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood. Leukemia as the first presenting symptom without any cutaneous involvement is a rare finding and can masquerade as acute undifferentiated leukemia. We present here such a case of a 59-year-old male who presented as leukemia without any cutaneous lesion but subsequently developed a scalp nodule.

Keywords: Blastic plasmacytoid dendritic cell neoplasm, cutaneous lesion, leukemia


How to cite this article:
Awasthi NP, Qayoom S, Dabadghao S. Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous lesion. J Can Res Ther 2017;13:1056-8

How to cite this URL:
Awasthi NP, Qayoom S, Dabadghao S. Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous lesion. J Can Res Ther [serial online] 2017 [cited 2019 Dec 13];13:1056-8. Available from: http://www.cancerjournal.net/text.asp?2017/13/6/1056/220358


 > Introduction Top


Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy, recognized as a distinct entity in the WHO 2008 classification of hematolymphoid neoplasm.[1] It has been previously known by various other names such as agranular CD4+ CD56+ hematodermic neoplasm or blastic natural killer (NK)-cell lymphoma.[2] It usually presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood.[1] The characteristic immunophenotype of CD4+/CD56+/− cells expressing antigens associated with plasmacytoid dendritic cells such as CD123, TCL1, BDCA2/CD303, and cutaneous lymphocyte-associated and interferon-dependent molecule MxA, in the absence of any other lineage-specific marker, confirms the diagnosis of BPDCN.[2],[3] It has a highly aggressive clinical course with dismal prognosis, irrespective of the initial pattern of disease. Leukemia as the first manifestation, in the absence of cutaneous lesion, is an uncommon presentation of this rare disease. We report here a case of a 59-year-old male presenting as acute leukemia who later developed a nodule in scalp. The patient was treated with Berlin-Frankfurt-Münster (BFM)-95 protocol, and while on final maintenance, he succumbed to his illness.


 > Case Report Top


A 59-year-old male presented with low-grade fever, cough, and weakness of 1-month duration. His general condition was otherwise good. Physical examination revealed pallor. There was no palpable lymphadenopathy, hepatomegaly, splenomegaly, bone tenderness, or any skin lesion. A complete blood count showed hemoglobin of 99 g/L; total leukocyte count of 34.5 × 109/L; platelets of 75 × 109/L. The peripheral blood examination showed the presence of 85% atypical cells with blastoid morphology that were medium-sized, without any cytoplasmic granule/Auer rod and were negative for myeloperoxidase (MPO) [Figure 1]a and [Figure 1]b. Bone marrow aspiration (BMA) revealed a hypercellular smear with 90% blasts. Flow cytometric (FCM) immunophenotyping revealed the tumor cells to be moderate to dim positive for CD45 and expressing CD4, CD56, CD123, CD2, CD38; while being negative for CD34, TdT, MPO, cytCD3, CD19, CD10, CD22, CD7, CD8, surface CD3, CD13, CD33, CD14, CD117, and CD5 [Figure 2]. Radiological imaging studies revealed the presence of mild splenomegaly and bilateral axillary lymphadenopathy measuring 1.5 cm in largest dimension. A final diagnosis of BPDCN was put forth. The patient however deferred the treatment. About a month later, he came back with a small nodule on the scalp [Figure 1]c Fine-needle aspirate smears and FCM revealed infiltration by atypical cells having similar morphology [Figure 1]d and immunophenotype as that of marrow smears. Axillary lymph node biopsy showed partial effacement of nodal architecture with infiltration by tumor cells. The patient was treated with BFM-95 protocol and responded well, with the follow-up BMA being in morphological remission. While on final maintenance (after 2 years), he developed headache and vomiting. Investigations revealed a relapse with central nervous system (CNS) involvement. Treatment was augmented, but the patient succumbed to disease within a month of relapse.
Figure 1: Bone marrow aspiration smears showing presence of blastoid cells (a: May-Grunwald-Giemsa, ×40) which were negative for myeloperoxidase (b: Myeloperoxidase, ×40). Clinical image of the individual pointing the scalp nodule (c), Fine-needle aspirate smear from the scalp nodule showing the presence of atypical cells similar to bone marrow aspiration smears (d: H and E, ×40)

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Figure 2: Flow cytometry scatter plots showing clusters of cells gated with dim CD45 low side scatter region positive for CD4, CD123, CD56, CD38, and CD2; while being negative for other myeloid and lymphoid markers; suggesting a diagnosis of blastic plasmacytoid dendritic cell neoplasm

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 > Discussion Top


Described for the first time in 1994 as CD4+ cutaneous lymphoma with high expression of CD56, BPDCN has been known previously with various names such as blastic NK leukemia/lymphoma, agranular CD4+ CD56+ hematodermic neoplasm, and agranular CD4+ NK cell leukemia.[3],[4] The WHO 2008 classification recognized it as a distinct entity under “Acute myeloid leukemia (AML) and related precursor neoplasms.” This neoplasm is derived from the CD4/CD56 expressing precursors of plasmacytoid dendritic cells also known as professional Type 1 interferon-producing cells or plasmacytoid monocytes.[1],[5] Its overall incidence is extremely low, accounting for 0.44% of all hematologic malignancies and 0.7% of cutaneous lymphomas.[6],[7] BPDCN affects males predominantly (male/female ratio of 3.3), most patients being elderly, with 61 years as the median age of diagnosis.[3],[8] This disease usually presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood. Rare cases of lymph nodes, lung, liver, spleen, tonsils, eyes, and CNS involvement have also been reported.[2],[9] It is exceedingly rare to see BPDCN with a leukemic presentation at the time of diagnosis, without a cutaneous lesion, with only thirty cases reported in literature till date.[10] The present case being only the second from this country. The leukemic form of disease represents <1% of cases of acute leukemia.[11] Although it has a characteristic immunophenotype, aberrant myeloid and lymphoid antigenic expression is frequently observed.[2],[3] Because of the rarity of these cases, any standardized therapeutic approach has not been described. Some cases have shown better response with acute lymphocytic leukemia-like regimen while some have shown good response with AML-like regimen. Whatever be the initial response, the disease has very high propensity to relapse, especially with CNS involvement, as CNS appears to be a sanctuary site during the systemic therapies.[12] This warrants for a prophylactic intrathecal therapy as well. The present case similarly showed a good initial response and the patient was well for almost 2 years. However, subsequently, he relapsed with a CNS involvement and succumbed to the disease within a month.


 > Conclusion Top


The present case is an addition to the handful of those cases of BPDCN which presents with leukemia, without any skin involvement. The disease has a poor outcome, which very frequently relapses after the initial response.

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Conflicts of interest

There are no conflicts of interest.

 
 > References Top

1.
Facchetti F, Jones D, Petrella T. Blastic plasmacytoid dendritic cell neoplasm. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri AS, Stein H, et al., editors. WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2008. p. 145-7.  Back to cited text no. 1
    
2.
Rauh MJ, Rahman F, Good D, Silverman J, Brennan MK, Dimov N, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation, lacking cutaneous involvement: Case series and literature review. Leuk Res 2012;36:81-6.  Back to cited text no. 2
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3.
Garnache-Ottou F, Feuillard J, Ferrand C, Biichle S, Trimoreau F, Seilles E, et al. Extended diagnostic criteria for plasmacytoid dendritic cell leukaemia. Br J Haematol 2009;145:624-36.  Back to cited text no. 3
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4.
Adachi M, Maeda K, Takekawa M, Hinoda Y, Imai K, Sugiyama S, et al. High expression of CD56 (N-CAM) in a patient with cutaneous CD4-positive lymphoma. Am J Hematol 1994;47:278-82.  Back to cited text no. 4
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Pilichowska ME, Fleming MD, Pinkus JL, Pinkus GS. CD4+/CD56+hematodermic neoplasm (“Blastic natural killer cell lymphoma”): Neoplastic cells express the immature dendritic cell marker BDCA-2 and produce interferon. Am J Clin Pathol 2007;128:445-53.  Back to cited text no. 5
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6.
Bueno C, Almeida J, Lucio P, Marco J, Garcia R, de Pablos JM, et al. Incidence and characteristics of CD4(+)/HLA DRhi dendritic cell malignancies. Haematologica 2004;89:58-69.  Back to cited text no. 6
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Ng AP, Lade S, Rutherford T, McCormack C, Prince HM, Westerman DA. Primary cutaneous CD4+/CD56+hematodermic neoplasm (blastic NK-cell lymphoma): A report of five cases. Haematologica 2006;91:143-4.  Back to cited text no. 7
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Facchetti F, Ungari M, Marocolo D, Lonardi S, Vermi W. Blastic plasmacytoid dendritic cell neoplasm. Hematol Meet Rep 2009;3:1-3.  Back to cited text no. 8
    
9.
Wang H, Cao J, Hong X. Blastic plasmacytoid dendritic cell neoplasm without cutaneous lesion at presentation: Case report and literature review. Acta Haematol 2012;127:124-7.  Back to cited text no. 9
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Qayoom S, Durga G, George S, Rahman K. Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity. Indian J Pathol Microbiol 2015;58:377-80.  Back to cited text no. 10
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11.
Jacob MC, Chaperot L, Mossuz P, Feuillard J, Valensi F, Leroux D, et al. CD4+CD56+lineage negative malignancies: A new entity developed from malignant early plasmacytoid dendritic cells. Haematologica 2003;88:941-55.  Back to cited text no. 11
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12.
Petrella T, Bagot M, Willemze R, Beylot-Barry M, Vergier B, Delaunay M, et al. Blastic NK-cell lymphomas (Agranular CD4+CD56+hematodermic neoplasms): A review. Am J Clin Pathol 2005;123:662-75.  Back to cited text no. 12
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