[CIS-PAGID] Leucopenia with no monocytes and low B cells

[Pere Soler Palacin]

Hi Eleonora, would you consider X-linked neutropenia (WASP) since neutropenia and monocytopenia are common? 



P. 



Pere Soler Palacín, MD, PhD. Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron.     Assistant Professor. Universitat Autònoma de Barcelona.                                                       
Passeig de la Vall d'Hebron 119-129. 
08035 Barcelona. Spain. 
Tel: 0034934893140. Fax: 0034934893039. 
E-mail: psoler at vhebron.net ; 34660psp at comb.cat . Web: www.upiip.com . 
  

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Pere Soler Palacín, MD, PhD. Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron.     Assistant Professor. Universitat Autònoma de Barcelona.                                                       
Passeig de la Vall d'Hebron 119-129. 
08035 Barcelona. Spain. 
Tel: 0034934893140. Fax: 0034934893039. 
E-mail: psoler at vhebron.net ; 34660psp at comb.cat . Web: www.upiip.com . 
  

No imprimir aquest correu ajudarà a preservar el medi ambient. 
Si vostè no és el destinatari del missatge, o l'ha rebut per error, si us plau notifiqui-ho al remitent i destrueixi el missatge amb tot el seu contingut. Està prohibida la distribució no autoritzada del contingut d'aquest missatge. 

No imprimir este correo ayudará a preservar el medio ambiente. 
Si usted no es el destinatario del mensaje, o lo ha recibido por error, notifíquelo por favor al remitente y destruya el mensaje con todo su contenido. Está prohibida la distribución no autorizada del contenido de este mensaje. 

----- Mensaje original ----- 
De: "Eleonora Gambineri" <eleonora.gambineri at unifi.it> 
Para: pagid at list.clinimmsoc.org 
Enviados: Miércoles, 21 de Septiembre 2011 19:39:18 
Asunto: [CIS-PAGID] Leucopenia with no monocytes and low B cells 



Dear all, 

I would like your inputs on the case below: 

8y/o male 

Past medical history: 
·           recurrent otitis and pharyngitis until adenoidectomy (4 yo) 
·           4yo: chickenpox with normal course 
·           Since 6yo: episodes of aphthous stomatitis every2-3 months, sometimes associated with fever. 

·           August 2010: CBC -> leucopenia  

Laboratory work up: 

October 2010: 
·           WBC: 3300/μL, Neutrophils 26.6% (absolute value: 877/ μL) Monocyte 0% 
·           CMV, EBV, Toxoplasmosis: negative 
·           Celiac disease screening: negative 
·           ANA, ENA, pANCA: negative 
·           Anti-neutrophils Ab: negative 
·           Immunoglobulins: within normal range 

·           Lymphocyte subsets:    B cells 9% (absolute value 240/ μL),  T cells CD3+ 87% (av: 2310), T cells CD3+CD4+ 48% (av: 1270/ μL), T cells CD3+CD8+ 22% (av: 580/ μL),  NK cells 4% (110/ μL) 

Genuary 2011 

·           Bone Marrow Aspiration:    hypocellular for the age of the patient, dysmyelopoiesis 

May 2011: fever + arthritis 
·           WBC: 3200/μL, Neutrophils 21.7% (absolute value: 690/μL) Monocyte 0% , Hemoglobin 10.3 g/dl, platelet    153000/μL 
·           Parvovirus B19 blood PCR:  positive 
·           Lymphocyte subsets:    B cells 7% (absolute value 176/ μL),  T cells CD3+ 92% (av: 2480/ μL), T cells CD3+CD4+ 54% (av: 1457/ μL), T cells CD3+CD8+ 27% (av: 742/ μL),  NK cells 1% (39/ μL) 

·           CD3+TCRαβ+CD4-CD8- (DNT cells): 1,3% of CD3+lymphocyte 

Bone Marrow Aspiration:   
·           Normal cellularity with decreased myeloid series 
·           TERC gene and TINF2 gene: no mutations 
·           FISH chromosome 7, 8 LSI 21 (21q22.13-q22.2): no chromosomal abnormalities 

·           Parvovirus    B19 PCR:  positive 

July 2011: 
·           WBC: 2470/μL, Neutrophils 22.8% (absolute value: 563/μL) Monocyte 0.4% , Hemoglobin 13 g/dl, platelet    138000/μL 
·           Parvovirus B19 blood PCR: negative 

·           Clinically well 

 Currently ongoing:  FAS induced apoptosis assay, B memory cell phenotype, specific antibodies (i.e. anti-pneumococcus, anti-tetanus) 




Any thoughts/suggestions is really appreciated! 

Thanks. 

BEst wishes, 

Eleonora 








******************************************************************* 
Dott.ssa Eleonora Gambineri 
Ricercatore Universitario 


Universita' degli Studi di Firenze, Dipartimento di Scienze per la Salute della Donna e del Bambino 
Ospedale Pediatrico "Anna Meyer", Dipartimento di Oncoematologia-Unità TMO 
Viale Gaetano Pieraccini, 24 
50139 FIRENZE 
Tel 055 5662405 (ufficio) 
       055 5662606 (reparto T.M.O.) 
Fax 055 4221012 
e-mail: eleonora.gambineri at unifi.it ;  e.gambineri at meyer.it 


Eleonora Gambineri, MD 
Researcher/Assistant Professor 


University of Florence, Department of Sciences for Woman and Child's Health 
"Anna Meyer" Children's Hospital, Department of Haem/Onc-BMT Unit 

Viale Gaetano Pieraccini, 24 
50139 FIRENZE 
ITALY 
Tel +39 055 5662405 (office) 
       +39 055 5662606 (BMT Unit) 
Fax +39 055 4221012 
e-mail: eleonora.gambineri at unifi.it ;  e.gambineri at meyer.it 
******************************************************************** 






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