> 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 > http://www.ma.uni-heidelberg.de/inst/ikc/ > > -----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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