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
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