WG: interesting case

From: Nebe, Thomas C. (thomas.nebe@ikc.ma.uni-heidelberg.de)
Date: Mon Nov 08 1999 - 17:09:14 EST


> Second thinking after calculating the low absolute numbers of T and B
> cells leads to immunodeficiency (clinical signs of immunodeficiency,
> family history, probably SCID).
> Thomas
> 
> Hello Maryalice,
> why do you think its not reactive?
> Missing light chain and lymphoma?
> Hemophagocytosis and (esp. T) lymphoma occurs.
> LGL lymphoma in children is very rare. We do thre colour CD2/5/7, 57/56/8,
> 3/56&16/8 to look for clonality (similar combinations work as well).
> I didn´t had a case like this yet.
> Regards
> Thomas Nebe
> 
> Dr.med. C. Thomas Nebe
> Universitaetsklinikum Mannheim
> Zentrallabor
> Theodor-Kutzer-Ufer 1-3
> D-68167 Mannheim
> Tel.  +49 621 383-3485
> FAX  +49 621 383-73 3485
>         +49 621 383-3819
> e-mail: thomas.nebe@ikc.ma.uni-heidelberg.de
> 
> Bitte besuchen Sie unsere informativen Webseiten unter
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> 
> -----Ursprüngliche Nachricht-----
> Von:	Gerhard Nebe-von-Caron [SMTP:Gerhard.Nebe-von-Caron@unilever.com]
> Gesendet am:	Montag, 8. November 1999 17:04
> An:	Nebe, Thomas C.
> Betreff:	FW: interesting case
> 
> 
> 
> -----Original Message-----
> From:	Maryalice Stetler-Stevenson [SMTP:stetler@box-s.nih.gov]
> Sent:	Wednesday, November 03, 1999 3:36 PM
> To:	Cytometry Mailing List
> Subject:	interesting case
> 
> 
> I have an interesting case. We studied a 5 y year old Chinese boy who had
> hemolytic anemia secondary to hemophagocytic syndrome in infancy, a
> fibrosarcoma of the chest wall that was successfully resected, and
> refractory, persistent Salmonella infection of the bone, lymph nodes, and
> blood.  Despite this he is functional and active.  Family history is
> notable for a sibling who died at age 2 in China from diarrhea. We get
> peripheral blood on the kid- WBC is 3.29 k/ul with 28.3% lymphocytes. 72%
> of the lymphs are NK cells. T-cells are normal, no increase in CD56+,
> CD57+
> or CD16+ T-cells and only 5% CD8+CD3+. The B-cells are normal. I am
> concerned at this number of NK cells- the Asian genetic backgroud adds to
> this concern and I wonder about an NK- LGL lymphoproliferative disorder.
> How can I assess clonality in these NK cells? Before I get much of a
> chance
> to worry about this- we review a previous lymph node biopsy which has a
> B-cell lymphoproliferative disorder- namely monoclonal(determined on
> paraffin sections), plasmacytoid B-cells. Morphology is c/w a marginal
> zone
> lymphoma. The monoclonal B-cells are likely surface light negative. They
> are CD20 negative. Maybe we missed them in the blood but our numbers are
> good- we can identify what is there- so maybe we didn't miss it. I would
> like feed back on 2 issues from the clinical flow community.
> 
> 1. What do you do if you think you have an NK-LGL lymphoproliferative
> process? I haven't had a specimen before that I considered this diagnosis
> before.
> 
> 2. Have you seen this peripheral blood profile in a patient with marginal
> zone lymphoma or other plasmacytoid neoplasm?
> 
> 	Maryalice
> 
> 
> Maryalice Stetler-Stevenson
> Director Flow Cytometry Unit
> Laboratory of Pathology, NCI, NIH << Datei: ATT02915.ATT >> 



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