ALPS patients

Maryalice Stetler-Stevenson stetler at box-s.nih.gov
Wed Mar 14 13:46:46 EST 2001


ALPS is Autoimmune LymphoProliferative Syndrome.


See reference below


Lim, M. S., Straus, S. E., Dale, J. K., Fleisher, T.,
Stetler-Stevenson, M, ., Strober, W., Sneller, M. C., Puck, J. M.,
Lenardo, M. J., Elenitoba-Johnson, K. S. J., Lin, A. Y., Raffeld, M.,
and Jaffe, E.:  Pathologic findings in human autoimmune
lymphoproliferative syndrome.  Am. J. Pathol. 153:1541-1550, 1998.






>  What is ALPS?
>
>Tim Singleton
>
>  >>> "Dr. Martin R. Hadam" <Hadam.Martin at mh-hannover.de> 03/09/01 04:28PM >>>
>
>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 at MH-Hannover.de

Maryalice Stetler-Stevenson
Director Flow Cytometry Unit
Laboratory of Pathology, NCI, NIH

"Learn the rules so you know how to break them properly."
The Dalai Lama
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