Indian J Hematol Blood Transfus (July-Sept 2013) 29(3):171–172 DOI 10.1007/s12288-012-0171-x
CASE REPORT
Blastic Plasmacytoid Dendritic Cell Neoplasm: A Report of 2 Cases Raman Arora • Ritesh Sachdev • Tribhuwan Bind • Md Ateek Khan
Received: 23 July 2010 / Accepted: 11 June 2012 / Published online: 24 June 2012 Ó Indian Society of Haematology & Transfusion Medicine 2012
Abstract Blastic plasmacytoid dendritic cell neoplasm is a recently classified aggressive hematodermic neoplasm derived from plasmacytoid dendritic cells. We describe two cases of this neoplasm, one a 15 year old child and other a 65 year male. The diagnosis was made by evaluating the detailed clinical history, morphology and flow cytometric findings. The diagnosis is rendered difficult owing to common morphological and flow cytometric overlaps. The neoplasms were characterized by skin and soft tissue involvement and strong CD4/CD56 positivity. These neoplasms are highly aggressive and have many diagnostic overlaps. Strong suspicion and detection will help in early therapeutic interventions. Keywords Leukemia Dendritic Plasmacytoid Hematodermic Natural killer cell
Blastic plasmacytoid dendritic cell neoplasm (BPDC) is a rare form of aggressive hematologic neoplasm with a predilection for skin and marrow involvement. It was known previously by various names such as blastic NK cell lymphoma, CD4?/CD56? hematodermic neoplasm, agranular CD4? natural killer cell leukemia etc. Our first patient was a 15 year old girl child who presented with widespread papulo nodular skin lesions with one tumorigenic sacral skin lesion. Bone marrow aspirate R. Arora R. Sachdev T. Bind M. A. Khan OncQuest Laboratories Pvt Ltd, 3, Factory Road, New Delhi 110029, India R. Sachdev (&) A 803, Plot No 7A, Navrattan Apartments, Sector 23, Dwarka, New Delhi 110075, India e-mail: [email protected]
received showed a total leukocyte count (TLC) of 6,000 cells/lL with 40 % atypical blastoid cells. These cells had high N/C ratios with vesicular chromatin and plasmacytoid appearance in some cells (Fig. 1a). Immunophenotyping studies performed on BD FACS CANTO showed these atypical cells to be CD45 dim. The cells were highly positive (bright) for CD4, CD56, CD7 and CD33 (Fig. 1b). All other B, T and myeloid lineage markers were negative. The skin biopsy was done in this case. There were sheets of atypical cells. IHC for CD56 and CD4 was positive along with LCA with negative CD3. Other IHC markers were not performed due to limited panel. Flow cytometric data in conjunction with clinical data led to a diagnosis of BPDC. The patient was started on intensive acute leukemia chemotherapeutic regimen and is showing initial signs of improvement with resolution of some skin lesions. The second patient was a 65 year male who presented with a known past history of chest wall mass for which he had received radiotherapy. Bone marrow sample received showed TLC of 70,200/lL with 70 % atypical blastoid cells having a similar morphology to the first case. Flow cytometric analysis showed these atypical cells to express strong CD4, CD56, HLA DR, and CD38 with moderate expression of CD2 (Fig. 1c). Since MPO and monocytic markers were negative, AML was excluded as per WHO criteria. Cytoplasmic CD3 negativity excluded T cell leukemias. CD8 and CD16 negativity went against NK origin. TdT, however, was negative in both cases. An initial possibility of BPDC was kept and the patient started on chemotherapy. This neoplasm shares many diagnostic overlaps and poses many diagnostic dilemmas. Diagnosis rests primarily on frequently encountered skin lesions and characteristic immunoprofile (CD4 and CD56 positivity). Other frequently
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Indian J Hematol Blood Transfus (July-Sept 2013) 29(3):171–172
Fig. 1 a Bone marrow smear with atypical cells having high N/C ratios and plasmacytoid appearance. b Case 1 showing strong CD33, CD4 and CD56. c Case 2 showing atypical cells in the blastic area, positivity for CD2, CD4 and CD56
encountered positive markers include CD2, CD7, CD38, CD33, CD43, CD45 RA, CD123, CD117, CD68, TCL 1, BCL 11a, CLA, TdT and MxA [1]. Other common differentials include acute myeloid leukemia, extra nodal NK/T cell lymphoma, nasal type and certain T cell lymphoma particularly the gamma/delta type. Detailed immunophenotypic profiling along with clinical findings will help arrive at the diagnosis in most of the cases. Certain myelomonocytic neoplasms may involve proliferation of plasmacytoid dendritic cells, but these are mature and CD56 negative. Our cases did not fit the lineage specificity of myeloid/ B/T/NK cells. The aggressive clinical course, lack of
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typical lineage markers (MPO/CD14/64/11c/19/cy CD3), limitation of molecular testing and positivity for CD4/56/ 33/7 led us to type the lesion. The response to therapy has further strengthened the diagnosis.
References 1. Facchetti F, Ungari M, Marocolo D, Lonardi S, Vermi W (2009) Blastic plasmacytoid dendritic cell neoplasm. Hematol Meet Rep 3(3):1–3
CASE REPORT
Blastic Plasmacytoid Dendritic Cell Neoplasm: A Report of 2 Cases Raman Arora • Ritesh Sachdev • Tribhuwan Bind • Md Ateek Khan
Received: 23 July 2010 / Accepted: 11 June 2012 / Published online: 24 June 2012 Ó Indian Society of Haematology & Transfusion Medicine 2012
Abstract Blastic plasmacytoid dendritic cell neoplasm is a recently classified aggressive hematodermic neoplasm derived from plasmacytoid dendritic cells. We describe two cases of this neoplasm, one a 15 year old child and other a 65 year male. The diagnosis was made by evaluating the detailed clinical history, morphology and flow cytometric findings. The diagnosis is rendered difficult owing to common morphological and flow cytometric overlaps. The neoplasms were characterized by skin and soft tissue involvement and strong CD4/CD56 positivity. These neoplasms are highly aggressive and have many diagnostic overlaps. Strong suspicion and detection will help in early therapeutic interventions. Keywords Leukemia Dendritic Plasmacytoid Hematodermic Natural killer cell
Blastic plasmacytoid dendritic cell neoplasm (BPDC) is a rare form of aggressive hematologic neoplasm with a predilection for skin and marrow involvement. It was known previously by various names such as blastic NK cell lymphoma, CD4?/CD56? hematodermic neoplasm, agranular CD4? natural killer cell leukemia etc. Our first patient was a 15 year old girl child who presented with widespread papulo nodular skin lesions with one tumorigenic sacral skin lesion. Bone marrow aspirate R. Arora R. Sachdev T. Bind M. A. Khan OncQuest Laboratories Pvt Ltd, 3, Factory Road, New Delhi 110029, India R. Sachdev (&) A 803, Plot No 7A, Navrattan Apartments, Sector 23, Dwarka, New Delhi 110075, India e-mail: [email protected]
received showed a total leukocyte count (TLC) of 6,000 cells/lL with 40 % atypical blastoid cells. These cells had high N/C ratios with vesicular chromatin and plasmacytoid appearance in some cells (Fig. 1a). Immunophenotyping studies performed on BD FACS CANTO showed these atypical cells to be CD45 dim. The cells were highly positive (bright) for CD4, CD56, CD7 and CD33 (Fig. 1b). All other B, T and myeloid lineage markers were negative. The skin biopsy was done in this case. There were sheets of atypical cells. IHC for CD56 and CD4 was positive along with LCA with negative CD3. Other IHC markers were not performed due to limited panel. Flow cytometric data in conjunction with clinical data led to a diagnosis of BPDC. The patient was started on intensive acute leukemia chemotherapeutic regimen and is showing initial signs of improvement with resolution of some skin lesions. The second patient was a 65 year male who presented with a known past history of chest wall mass for which he had received radiotherapy. Bone marrow sample received showed TLC of 70,200/lL with 70 % atypical blastoid cells having a similar morphology to the first case. Flow cytometric analysis showed these atypical cells to express strong CD4, CD56, HLA DR, and CD38 with moderate expression of CD2 (Fig. 1c). Since MPO and monocytic markers were negative, AML was excluded as per WHO criteria. Cytoplasmic CD3 negativity excluded T cell leukemias. CD8 and CD16 negativity went against NK origin. TdT, however, was negative in both cases. An initial possibility of BPDC was kept and the patient started on chemotherapy. This neoplasm shares many diagnostic overlaps and poses many diagnostic dilemmas. Diagnosis rests primarily on frequently encountered skin lesions and characteristic immunoprofile (CD4 and CD56 positivity). Other frequently
123
172
Indian J Hematol Blood Transfus (July-Sept 2013) 29(3):171–172
Fig. 1 a Bone marrow smear with atypical cells having high N/C ratios and plasmacytoid appearance. b Case 1 showing strong CD33, CD4 and CD56. c Case 2 showing atypical cells in the blastic area, positivity for CD2, CD4 and CD56
encountered positive markers include CD2, CD7, CD38, CD33, CD43, CD45 RA, CD123, CD117, CD68, TCL 1, BCL 11a, CLA, TdT and MxA [1]. Other common differentials include acute myeloid leukemia, extra nodal NK/T cell lymphoma, nasal type and certain T cell lymphoma particularly the gamma/delta type. Detailed immunophenotypic profiling along with clinical findings will help arrive at the diagnosis in most of the cases. Certain myelomonocytic neoplasms may involve proliferation of plasmacytoid dendritic cells, but these are mature and CD56 negative. Our cases did not fit the lineage specificity of myeloid/ B/T/NK cells. The aggressive clinical course, lack of
123
typical lineage markers (MPO/CD14/64/11c/19/cy CD3), limitation of molecular testing and positivity for CD4/56/ 33/7 led us to type the lesion. The response to therapy has further strengthened the diagnosis.
References 1. Facchetti F, Ungari M, Marocolo D, Lonardi S, Vermi W (2009) Blastic plasmacytoid dendritic cell neoplasm. Hematol Meet Rep 3(3):1–3
