[CIS-PAGID] Rituximab and Chronic ITP

[Dewton Vasconcelos]

Dear Rafael

I think that despite low IgG it is not possible to ascertain that your 
patient has CVID.
Other diseases can present low IgG and autoimmunity, for example the 
hyper IgM syndromes.
Among these, CD40 and CD40L deficiencies are more related to cellular 
defects and some of them present CNS infections such as JC or 
enteroviral infections, and neutropenia is common in these patients.
Think in the possibility of CVID, some of these patients present a ALPS 
phenotype with hematological autoimmune manifestations.
In the context of Rituximab, nowadays is not useful to look for B cell 
subpopulations, but if the patient improves we can test him later.
Low CD4s and NK cells can be associated to the primary disease (a PID?) 
or to the severe infection (it is common to see this feature in patients 
presenting disseminated tuberculosis or cryptococcosis).

All the best,

Dewton Vasconcelos
University of São Paulo

Rafael Firszt wrote:

> I am seeing a 14 yo boy with a history of chronic ITP and neutropenia who got Rituximab in March of this year.  He was just admitted to hospital with encephalitis of unknown cause. Before Rituximab was given he had an IgG of 478.  Since being admitted he got a dose of IVIG but no functional studies have ever been done on him.  He has no history of other infections.

>

> His Enumeration drawn last week shows:

> CD4:CD8 Ratio:   ratio  * 1.05

> % CD19:  %  * 12

> % Natural Killer Cells:    * 3 L

> % CD3:  * 84

> % CD2:  %   86

> Absolute CD4   * 373 L

> CD4+CD45RO+ cells   * 249

> Absolute CD45RA   * 112 L

> Absolute CD8   * 356

> Absolute CD19   * 105

> Absolute Natural Killer Cells   * 30 L

> Absolute CD3  * 747

>

> Therefore he has low NK cells, low CD4 and low CD45RA.  It is interesting that his B cell count is normal.

>

> He has ongoing work-up for his encephalitis but ID feels it is most likely virally-induced.

>

> I can't test him for function because of recent IVIG.

>

> With history of ITP, neutropenia, initial low IgG and now encephalitis he likely has a form of CVID (I think).

> Based on this history I several questions:

>

> 1) Any other investigations?  (I have mitogen studies pending)

> 2) Would you continue IVIG monthly assuming he has CVID or would you stop and re-evaluate his function after several months to confirm the possible CVID

> 3) In CVID or after rituximab, have any of you seen low NK cells and low CD4 and low CD45RA in either of these situations?

>

> Thanks for any help

>

> Rafael Firszt

> University of Utah

>

>

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