Re: ALPS patients

From: Dr. Martin R. Hadam (Hadam.Martin@mh-hannover.de)
Date: Fri Mar 09 2001 - 16:28:35 EST


On Thu, 8 Mar 2001 18:15:43 -0500, Maryalice Stetler-Stevenson wrote:

>We recently had an ALPS patient (20yo, lymph adenopathy) lymph node
>FNA with a high number of homogeneous abnormal T cells- 64% CD3+,
>CD2+, CD5+, CD7-, CD57+, CD38+, CD4-, CD8- and CD25-. I thought this
>was very high numbers and was concerned abouyt a developing lymphoma
>(they do get them you know, and besides, tumors happen). Has anyone
>ever seen this high a % homogeneous abnormal T's in ALPS before?

 Yes, even in peripheral blood (but you sure need to watch out for
developing malignancies). What I find even more interesting is that the
cells in your patient are also CD7-negative; such data are rarely
provided if ever. I've seen this phenotype in two (pediatric) cases.
Seems to me that the marker combination CD3+CD4-CD7-CD8- lends itself
to readily detect such a subset even when occurring with less abundance
e.g. in peripheral blood. CD7 could then be used to differentiate from
"normal" double-negatives.


 Martin R. Hadam
 Kinderklinik - Medizinische Hochschule
 D-30623 Hannover
 Germany
 Email: Hadam.Martin@MH-Hannover.de



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