[PAGID] 14 mo old with lymphopenia

[dmvascon at usp.br]

I also agree that XLP is a very good hypothesis, as well as XLA, that  
could be associated with cytopenias (mainly neutropenia) during severe  
infections.
Is there any evidence of hemophagocytosis?
Could any other herpesvirus be associated with the development of the  
clinical features in XLP?

All the best,

Dewton Vasconcelos
University of São Paulo

Citando "Junker, Anne" <ajunker at cw.bc.ca>:


> Rule out XLP as cause of acquired B cell deficiency.

>

> Anne Junker, MD

> Associate Professor, Division of Infectious & Immunological Diseases,

> Medical Director, Pediatric Specialty programs at BC Children's Hospital

> Associate Director (Clinical), Child & Family Research Institute

> Room K4-223

> Children & Women's Health Centre

> 4480 Oak Street, Vancouver, BC   V5H 3V4

> ( office: 604-875-3591 7 fax: 604-875-2414  * ajunker at cw.bc.c þ  

> bcchildrens.ca

>

>

> -----Original Message-----

> From: pagid-bounces at list.clinimmsoc.org   

> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Ashish Kumar

> Sent: Monday, May 07, 2007 12:24 PM

> To: pagid at list.clinimmsoc.org

> Subject: [PAGID] 14 mo old with lymphopenia

>

>

> Dear Colleagues,

>

> I need help with a patient and would appreciate your input.

>

> This 14 month old boy developed bilateral pneumonia (interstitial pattern on

> imaging) associated with pancytopenia. Bone marrow biopsy and aspirate was

> normal. With antibiotics, his pneumonia and pancytopenia resolved except

> that he is persistently lymphopenic with total WBC of 4-6 and ALC of 1.0.

> Subset analysis shows no B cells (CD19 and CD20 were both zero). CD4:CD8

> ratio and percentages are normal although absolute numbers are low, because

> of absolute lymphopenia. IgG was low at 254, but IgA was 76 and IgM 29. T

> cell mitogen stimulation was normal and he has reactive titres to Diphtheria

> and tetanus. Levels of ADA and PNP were higher than normal (probably due to

> regenerating cells).

>

> He was healthy for the first 12 months of life, but in the last 3 months has

> had 3 episodes of otitis and 2 episodes of pneumonia (including the present

> one). Bronchoscopy did not show any anatomic abnormality and the cultures

> grew candida albicans and Adenovirus. From his blood, viral PCRs grew HHV6

> 120,000 copies/ml. There is no family history of immune deficiency or other

> illnesses. He is also not gaining weight - looks malnourished with distended

> abdomen and loss of subcutaneous fat, but development seems ok.

>

> What do you think he has? BTK analysis is pending.

>

>

> Ashish Kumar M.D., Ph. D.

> Assistant Professor

> Pediatric Hematology/Oncology/Blood and Marrow Transplantation

> University of Minnesota

>



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