Clinicopathological analysis of 46 cases with CD4+ and/or CD56+ immature haematolymphoid malignancy: reappraisal of blastic plasmacytoid dendritic cell and related neoplasms.

Abstract:

AIMS:In this study, we aimed to investigate the clinicopathological features of CD4+ and/or CD56+ immature haematolymphoid malignancy (iHLM), including blastic plasmacytoid dendritic cell neoplasm (BPDCN). METHODS AND RESULTS:We analysed the clinicopathological features of 46 patients diagnosed consecutively with CD4+ /CD56+ iHLM. These cases were categorised into three groups based on their immunohistochemical expression of three plasmacytoid dendritic cell (pDC) markers [CD123, CD303 and T cell leukaemia/lymphoma (TCL1)]: cutaneous BPDCN (n = 35), non-cutaneous BPDCN (n = 6) and non-BPDCN-type CD56+ neoplasms (n = 5). Compared to non-cutaneous BPDCN, cutaneous BPDCN was associated with an older median age at onset (72 years versus 45 years, P < 0.05), and higher positivity for CD4 (P < 0.05), CD123 (P < 0.05) and 2-3 pDC markers (89% versus 50%, P = 0.05). Cutaneous BPDCN was divided into terminal deoxynucleotidyl transferase (TdT)+ and TdT- subgroups, which did not differ in prognosis, although TdT+ cases showed a lower median onset age (66 years versus 79 years, P < 0.05) and higher frequency of extracutaneous lesions (P < 0.05). Compared to the BPDCN groups, non-BPDCN-type CD56+ neoplasm cases showed higher cytoplasmic CD3 positivity (P < 0.05) and less frequent BCL-2 expression (P < 0.05), and lacked cutaneous lesions. However, the survival curves overlapped. Notably, one case involved an unusual composite neoplasm, comprising CD56+ lymphoblastic lymphoma and mature CD56+ cytotoxic T cell lymphoma. CONCLUSIONS:Our present data support the recognition of cutaneous BPDCN as a homogenous entity, in contrast to the non-cutaneous form. Additional research is warranted to characterise non-BPDCN-type CD56+ neoplasms.

journal_name

Histopathology

journal_title

Histopathology

authors

Suzuki Y,Kato S,Kohno K,Satou A,Eladl AE,Asano N,Kono M,Kato Y,Taniwaki M,Akiyama M,Nakamura S

doi

10.1111/his.13340

subject

Has Abstract

pub_date

2017-12-01 00:00:00

pages

972-984

issue

6

eissn

0309-0167

issn

1365-2559

journal_volume

71

pub_type

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