According to the numbers you gave, this patient has an absolute lymphocyte count of 920 cell/ul. The number of NK cells is 663/ul, with the remainder (258/ul) being T and B cells. This represent a profound decrease in T cells. It is suggestive of an immunodeficiency, perhaps a variant of severe combined immunodeficiency (SCID) with relative and absolute elevation of NK cells. This would explain the increased susceptiblity to malignancies (fibrosarcoma, hemophagocytic syndrome and lymphoma) and the persistent salmonella infection. The history of a sibling dying at age two with a diarrheal disease is also in favour of an inherited immunodeficiency. Further investigations along this line may be of help. Mohamed Elkhalifa, MD, PhD University of South Alabama >From: Maryalice Stetler-Stevenson <stetler@box-s.nih.gov> >To: Cytometry Mailing List <cytometry@flowcyt.cyto.purdue.edu> >Subject: interesting case >Date: Wed, 3 Nov 1999 11:35:35 -0400 > > >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 ______________________________________________________ Get Your Private, Free Email at http://www.hotmail.com
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