images in haematology
Blastic plasmacytoid dendritic cell neoplasm
A 58-year old woman presented with a dermal tumour over her right scapula, which had slowly progressed over the previous 9 months. There were no systemic symptoms. Physical examination revealed only a painless solitary violaceous nodule over the right scapula with a diameter of approximately 3 cm (left). Histologically, extensive fields and nests of tumour cells in a background of a few pre-existing lymph follicles and scattered lymphocytes were observed. Many tumour cells had a blastic appearance with round, slightly polymorphic nucleoli and scant eosinophilic cytoplasm (right). Immunohistochemistry showed positive staining with CD2, CD3, CD43, CD4, terminal deoxynucleotidyl transferase, CD56 and CD68. Weak positive staining was observed for CD33 and CD38. Proliferation activity of approximately 50% was demonstrated with MIB1. Flow cytometric immunophenotyping showed strong expression of CD56 and CD123 and weak expression of CD7 and CD45. Cytogenetic analysis showed a normal 46, XX karyotype. A diagnosis of blastic plasmacytoid dendritic cell neoplasm was made on the basis of the histological features, immunohistochemical staining and flow cytometric evaluation. Expression of CD4, CD56 and CD123 is characteristic of this disease. Typically, as in this patient, dot-like cytoplasmic staining pattern is found with CD68. This rare myeloid
ª 2013 John Wiley & Sons Ltd British Journal of Haematology, 2014, 164, 757
malignancy frequently presents in the skin as solitary or multifocal nodules and has a poor prognosis with a median overall survival of 12–19 months due to the development of fulminant leukaemia. In our patient there was no bone marrow or cerebrospinal fluid involvement. A computer tomography scan of the neck, chest and abdomen showed no enlarged lymph nodes or splenomegaly. The patient was treated with a single cycle of systemic daunorubicin and cytarabine, followed by an allogeneic haematopoietic stem cell transplantation. Due to the high recurrence rate of this disease within the central nervous system, six intrathecal prophylactic treatments with methotrexate and dexamethasone were concurrently administered. The patient is still undergoing treatment. Although blastic plasmacytoid dendritic cell neoplasm is an uncommon condition, it should be considered in the differential diagnosis of cutaneous lymphoma and myeloid sarcoma. Sofie L. Roodbergen1, Johannes Hofland2, King H. Lam3, Petra K. Dikrama1, Annemiek Broyl2 and Kim Monkhorst3 1
Department of Dermatology, Erasmus Medical Centre, 2Department of
Haematology, Erasmus Medical Centre, and 3Department of Pathology, Erasmus Medical Centre, Rotterdam, The Netherlands. E-mail: [email protected]
First published online 25 November 2013 doi:10.1111/bjh.12666
Blastic plasmacytoid dendritic cell neoplasm
A 58-year old woman presented with a dermal tumour over her right scapula, which had slowly progressed over the previous 9 months. There were no systemic symptoms. Physical examination revealed only a painless solitary violaceous nodule over the right scapula with a diameter of approximately 3 cm (left). Histologically, extensive fields and nests of tumour cells in a background of a few pre-existing lymph follicles and scattered lymphocytes were observed. Many tumour cells had a blastic appearance with round, slightly polymorphic nucleoli and scant eosinophilic cytoplasm (right). Immunohistochemistry showed positive staining with CD2, CD3, CD43, CD4, terminal deoxynucleotidyl transferase, CD56 and CD68. Weak positive staining was observed for CD33 and CD38. Proliferation activity of approximately 50% was demonstrated with MIB1. Flow cytometric immunophenotyping showed strong expression of CD56 and CD123 and weak expression of CD7 and CD45. Cytogenetic analysis showed a normal 46, XX karyotype. A diagnosis of blastic plasmacytoid dendritic cell neoplasm was made on the basis of the histological features, immunohistochemical staining and flow cytometric evaluation. Expression of CD4, CD56 and CD123 is characteristic of this disease. Typically, as in this patient, dot-like cytoplasmic staining pattern is found with CD68. This rare myeloid
ª 2013 John Wiley & Sons Ltd British Journal of Haematology, 2014, 164, 757
malignancy frequently presents in the skin as solitary or multifocal nodules and has a poor prognosis with a median overall survival of 12–19 months due to the development of fulminant leukaemia. In our patient there was no bone marrow or cerebrospinal fluid involvement. A computer tomography scan of the neck, chest and abdomen showed no enlarged lymph nodes or splenomegaly. The patient was treated with a single cycle of systemic daunorubicin and cytarabine, followed by an allogeneic haematopoietic stem cell transplantation. Due to the high recurrence rate of this disease within the central nervous system, six intrathecal prophylactic treatments with methotrexate and dexamethasone were concurrently administered. The patient is still undergoing treatment. Although blastic plasmacytoid dendritic cell neoplasm is an uncommon condition, it should be considered in the differential diagnosis of cutaneous lymphoma and myeloid sarcoma. Sofie L. Roodbergen1, Johannes Hofland2, King H. Lam3, Petra K. Dikrama1, Annemiek Broyl2 and Kim Monkhorst3 1
Department of Dermatology, Erasmus Medical Centre, 2Department of
Haematology, Erasmus Medical Centre, and 3Department of Pathology, Erasmus Medical Centre, Rotterdam, The Netherlands. E-mail: [email protected]
First published online 25 November 2013 doi:10.1111/bjh.12666
