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blastic plasmacytoid dendritic cell neoplasm M / 78 (22667)

blastic plasmacytoid dendritic cell neoplasm M / 78
Subtitle: B10-19212

Type:
lymph node and skin

Sender:
ugnius

2010-07-13 08:04
INCTR - EBMWG Hematopathology Online

78yrs old male with erythremia history in 1999 (no histo verified). Due to systemic lymphadenopathy the lymph node biopsy and skin biopsy from generalized papular eruption were taken.
HISTO: NODE: The diffuse infiltration of medium sized cells with oval and irregular nuclei with focal "pale" zones. SKIN: Leukemoid perivascular periadnexal infiltrates (not included yet and not IH-typed).
IH: LCA+; Bcl2+; CD4+ (low quality); CD43+; CD56+/-; CD7+; TdT(-/+); CD68(-); CD20/Pax5/CD3/CD2/CD5(-); MPO(-); CD117(-)(focal positivity in T cell enreached pale areas?).

PROPOSED DIAGNOSIS: Plasmacytoid dendritic cell tumor in the lymph node and skin.
The link: https://www.ipath-network.com/inctr/object/view/19542

Thankyou.

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hoellers 2010-07-13 09:08	Dear Ugnius, very interesting case, indeed. Did you also stain for other T-cell markers, like CD2, CD5, CD7 and CD8 and cytotoxic markers (TIA-1, GranzymeB ...). I was wondering, if this could be a T-cell lymphoma.
ugnius 2010-07-13 09:43	Thanx: CD43+ CD4+; CD3/CD2/CD5(-); CD7+ (patchy). GranB and CD8 pending.
ugnius 2010-07-13 09:44	All add IH photos will be appended on arrival.
leoncini 2010-07-13 12:56	I am also wondering, if this could be a T -cell lymphoma
ugnius 2010-07-13 13:14	Please find CD5(-), CD7+ (faint) and fragmented skin biopsy HE.
tzankov 2010-07-13 16:54	There are several argumanets in favor of a plasmacytoid dendritic cell neoplasia (Cd4+, CD56+, TdT dim+, cytomorphology). Would you like to stain the slide for CD123 and perfoem thin HE and Giemsa stained slides?
ugnius 2010-07-13 17:05	Unfortunatelly I'm out of CD123 and block is external, so quality would not be better. Appologies.
torlakovic 2010-07-19 05:31	I think that this could be a blastic plasmacytoid dendritic cell neoplasm, but myeloid/monoblastic differentiation needs to be ruled out in the first place. CD123 will not make a difference as it is positive in some cases of myeloid leukemia. Lysozyme would actually be useful as it is regularly negative in blastic plasmacytoid dendritic cell neoplasm and is generally available. As monoblatic leukemias may be both CD34 and MPO negative, they however, will not be missed by lysozyme. Cytology in blood or bone marrow could actually be diagnostic in some cases. Cytogenetic results could also be helpful. NPM1 by IHC may also be informative if this is a monoblastic process. If lysozyme is negative, EBV-association needs to be ruled out. If there is blood or bone marrow involvement, flow cytometry would also be helpful.
ugnius 2010-07-20 11:58	Thank you a lot. HE and Giemsa recut, but quality is unfortunatelly not better...
hurwitz 2010-07-20 17:46	According to all what has been said, the most probable diagnosis is: Blastic plasmacytoid dendritic cell neoplasm (myeloid monocytoid differentiation to be ruled out)
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Last modified: 2010-07-20 11:53:21