Complete Remission with α-Interferon in a Poor Risk Patient with Blastic Plasmocytoid Dendritic Cell Neoplasm: A Case Report and Review of Literature

Case Report

Ann Hematol Oncol. 2015; 2(8): 1060.

Complete Remission with α-Interferon in a Poor Risk Patient with Blastic Plasmocytoid Dendritic Cell Neoplasm: A Case Report and Review of Literature

Mazziotta F¹*, Caracciolo F¹, Orciuolo E¹, Buda G¹, Benedetti E¹, Bonadio AG², Iovino L¹, Carulli G¹, Ciancia EM³, Azzarà A¹, Galimberti S¹ and Petrini M¹

¹Department of Oncology, Transplant and Advances in Medicine–Section of Haematology, University of Pisa, Italy

²First Division of Pathology, University of Pisa, Italy

³Second Division of Pathology, University of Pisa, Italy

*Corresponding author: Francesco Mazziotta, Department of Oncology, Transplant and Advances in Medicine–Section of Haematology, University of Pisa, Italy

Received: September 17, 2015; Accepted: November 15, 2015; Published: November 17, 2015

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and clinically aggressive haematologic malignancy caused by neoplastic transformation and clonal proliferation of plasmacytoid dendritic cell precursors. Current treatment approaches are not effective and the outcome remains poor in the majority of patients even after chemotherapy and bone marrow transplantation. In this report we consider the use of interferon alpha for old unfit patients not candidate for intensive strategies.

Keywords: Blastic plasmacytoid dendritic cell neoplasm; Interferon alpha; NK

Introduction

Blastic plasmacytoid dendritic cell neoplasm (BPDCN), due to its biological and clinical features, could be classified as an intermediate disease between acute myeloid leukemia (WHO 2008) [1] and non Hodgkin lymphomas (WHO-EORTC 2005) [2].

In the first BPDCN case published in 1994 [3], authors reported the lack of a lineage specific antigen and the co-expression of CD4 and CD56 suggesting a natural killer (NK) cell-lineage origin. This entity was firstly defined as blastic NK cell lymphoma (BNKL) [4]. Later, the discovery of CD123 (interleukin-3 receptor) and BDCA2 (blood dendritic cell antigen 2) expression allowed to identify these cells as plasmocytoid dendritic cells. Blastic cells appear to secrete α-Interferon (INF) [5-7] and thus are immunophenotipically and functionally identified as immature dendritic cells. For the above mentioned reasons, the tumor was renamed blastic plasmacytoid dendritic cell neoplasm [1].

Plasmocytoid dendritic cells (pDCs) develop in bone marrow, reside primarily in the lymphoid organs (nodes and thymus) in steady state and can be mobilized, in response to some stimula including G-CSF and FLT-3, into peripheral blood, where they are poorly represented (< 1% of the mononuclear cells).

In lymph nodes pDCs are detectable in T-cell rich areas [8,9] as round or oval cells with an eccentric nucleus and a well developed endoplasmic reticules (ER).

Fred Siegal and Yong-Jun Liu [10] demonstrated that these secretory cells produced large amounts of IFN and identified them with the IFN-producing cells previously described [11-13].

As components of the innate immune system, pDCs detect viral acid nucleic through toll-like receptor 7 (TLR7) and toll-like receptor 9 (TLR-9), inducing the IFN secretion. This protein is responsible for the resistance to viral infections and promotes NK, DC, T and B cell functions [14].

IFN signaling promotes the survival of ex vivo pDCs [15,16], but in vivo studies [17] have demonstrated that IFN mediates pDCs death through caspase activation, creating a proapoptotic loop.

Limited data are available to guide the management of BPDCN. Response to conventional chemotherapy is commonly poor and survival correspondingly short (mean survival of 12-14 months) [18].

Several Authors suggest the effectiveness of allogeneic hematopoietic stem cell transplantation (AHSCT) [19-21].

Case Presentation

A 74-year-old man was admitted on our chemotherapy ward because of BPDCN with initial skin involvement.

At diagnosis, in November 2013, the patient showed 2-3 cm purplish eruptive nodules, neither painful nor itchy, preferentially located on his trunk and legs (Figure 1).