[CIS PIDD] [cis-pidd] Neutropenia in XLA

[CIS-PIDD]

Is there any involvement of other hematologic lineages? Has a bone marrow aspiration or biopsy been done? I am not aware of isolated neutropenia in XLA, but have seen this in the absence of anemia in an X-HIM patient that was eventually attributed to chronic parvovirus B19 infection. PCR could detect viremia, parvovirus inclusions in marrow biopsies will corroborate the diagnosis and in situ hybridization can confirm the infection.
All the best,Soheil Chegini

      From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
 To: CIS-PIDD <cis-pidd at lyris.dundee.net> 
 Sent: Friday, October 13, 2017 1:51 PM
 Subject: [cis-pidd] Neutropenia in XLA
   
Dear all, we have a 13 moths old boy with genetically confirmed XLA. At diagnosis he presented recurrent URTI, agammaglobulinemia and neutropenia. Despite immunoglobulin replacement therapy for 5 months with correct IgG levels (Ig G = 1300 mg/dL), mild to moderate neutropenia persists requiring intermittent G-CSF administration. Do you have any experience with long-term neutropenia in XLA? As far as I know neutropenia occurs at the time of diagnosis and it resolves after IgRT is started and normal IgG trough levels achieved. 
Best regards from BCN,
P.

Pere Soler Palacín, MD, PhD, MSc.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron.
Vall d'Hebron Research Institute (VHIR)  
Assistant Professor. Universitat Autònoma de Barcelona (UAB)     
Director of the Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies (Barcelona)                                                 
Pg. de la Vall d'Hebron, 119-129
08035 Barcelona. Catalonia.
Tel. 0034934893140  /  Fax 0034934893039
psoler at vhebron.net  /  34660psp at comb.cat
Web: www.upiip.com
My ORCID
ResearchGate: http://www.researchgate.net/profile/Pere_Soler-Palacin
LinkedIn: www.linkedin.com/in/pere-soler-palacin
 
 
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