[CIS-PAGID] Rosai-Dorfman and lymphopenia

[Tamara Pozos]

Hello all-

Last week I met a 2.5 year old boy with rapid-onset massive disseminated adenopathy and a nasopharyngeal mass, pathology consistent with Rosai-Dorfman including emperipolesis.   Lymphocyte phenotyping repeatedly shows significant T (absolute 600) and B (ABS 100)  lymphopenia - NK were low initially but normalized.   He was not always lymphopenic, in late April ALC 2500 on CBC .
 
Past history negative for recurrent infections or growth issues - he actually looks quite well aside from the nodes.  IgG, A, M, E, dip/tet, pneumo titers fine. Mitogen responses OK, but antigen responses not done due to inadequate number of lymphocytes.  EBV and HHV-6 serologies consistent with past exposure.  ALPS considered - DNTs ABS 18, 1.5%; Hgb and platelets are normal.  Abdominal ultrasound normal. 


Mother has sarcoidosis, per report, and has received steroids intermittently.  
 
Questions-
1) What experience do you have with immunologic disease in young children with Rosai-Dorfman?  Published reports indicate a spectrum from no immune involvement to transient lymphopenias in self-limited cases to fatalities from infections and severe immunedysregulation.  Are there other diagnoses I should consider or genetic tests you would suggest, esp. given his mother's diagnosis?
 
2) Need for treatment - A short course of Decadron early in his course was helpful in shrinking nodes transiently, but they recurred and steroids aren't appealing for the long term. My hematology/oncology colleagues would like to treat with cladrabine as the lymph nodes are very large and may be slightly affecting his ability to swallow. I'm somewhat hesitant to make him more cytopenic, (he is on Bactrim).  Thoughts on chemotherapy? Other immunomodulators? 




Thank you so much for your time and collective experience-
Tamara 








Tamara Pozos, MD PhD
Pediatric Infectious Diseases and Immunology 
Children's Hospitals and Clinics of Minnesota 
Mail Stop 70-504 
Garden View Building, 5th Floor 
345 Smith Avenue North 
St. Paul, MN  55102 
tamara.pozos at childrensmn.org 
651-220-6444 (office)

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