[CIS PIDD] [cis-pidd] Asking for advice in AIHA-combined immunodeficiency

Fri Aug 8 03:25:38 EDT 2014

Dear Dr. Prockop and Notarangelo,

Thanks for your kind replies.
The lymph subsets and proliferation tests were performed 6 months ago.
At that time the patient was taking PRD 7.5mg qd (0.11mg/kg). So no
influence in the results is expected.
CARD11 is a good point. We´ll look for transitional B cells. Thank you
for clarifying

Best wishes,

Luis Ignacio Gonzalez-Granado
Immunodeficiencies Unit.
Pediatrics
Hospital 12 octubre
Madrid. Spain

2014-08-08 1:56 GMT+02:00 Notarangelo, Luigi
<Luigi.Notarangelo at childrens.harvard.edu>:
> Dear Dr Gonzalez,
>
> This is likely a co bi ed immunodeficiency. You mentioned markedly increased proportion of CD19+ CD21lo, and perhaps you were thinking that this could represent autoimmune prone B cells. However, use of CD21 alone is not sufficient. It would be important to look also at CD38. These could be transitional B cells (CD19+ CD21lo CD38+) as opposed to the autoimmune prone CD19+ CD21lo CD38lo. A marked increase of transitional B cells associated with severe CD4 lymphopenia might indicate CARD11 mutations.
> With regard to treatment, I would not be too worried about rituximab, but MMF is also a possibility, as suggested by Dr Prockop
>
> Best regards
>
> Luigi D Notarangelo
>
> Sent from my iPad
>
> Luigi D. Notarangelo, MD
> Jeffrey Modell Chair of Pediatric Immunology Research
> Division of Immunology, Boston Children's Hospital
> Professor of Pediatrics and Pathology, Harvard Medical School
> Karp Research Building, Room 10217
> 1, Blackfan Circle
> Boston, MA 02115
> USA
>
> Tel: 617-919-2277
> FAX: 617-730-0709
>
>> On Aug 7, 2014, at 7:34 PM, "prockops at MSKCC.ORG" <prockops at MSKCC.ORG> wrote:
>>
>> Dr Gonzalez
>>
>> I am not clear from you note if the immune phenotype and function was
>> performed while on low or high dose steroids?
>>
>> We have recently been able to successfully avoid rituximab in a
>> patient with post-transplant AIHA with MMF. There is a small/limited
>> literature on this approach in adults and canines....
>>
>> Best,
>> Susan Prockop
>>
>> Assistant Attending
>> Pediatric BMT Service
>> Memorial Sloan-Kettering Cancer Center
>> prockops at mskcc.org
>> 212-639-6715
>>
>>
>>
>> -----Original Message-----
>> From: Nacho Gonzalez [mailto:nachgonzalez at gmail.com]
>> Sent: Thursday, August 07, 2014 4:13 AM
>> To: CIS-PIDD
>> Subject: [cis-pidd] Asking for advice in AIHA-combined immunodeficiency
>>
>> Dear all,
>>
>> this is an update for the patient posted on Aug 15, 2012  [14y.o Chinese boy with autoimmune haemolytic anemia and profound CD4 lymphopenia].
>>
>> Despite 2 years free of infections and stable disease under steroid maintenance treatment (prednisone 5-10 mg qd) he has had a new hemolytic crisis with increased reticulocyte count for the last 2 weeks (290.3 x1000/µl,  10,5%). Bili 2 - 3.7 mg/dL.  Hb (in june, 12.3 g/dL  now 10.2 g/dL).
>>
>> Steroids have been increased up to 15 mg qd last week. He has never received Rituximab. I am concerned about its use in this patient (total lymph 737/uL (CD3+ 378, gd 19%, CD4+ 94 /uL , CD8+ 186/uL,
>> CD19+ 284/uL, CD56+/CD3- 62/uL.  %Lymph T CD4+CCR7+CD45RA+  3.2% [N >
>> 25%].  %Lymph T CD8+CCR7+CD45RA+  12%  [N > 28%].  %CD4+CD45RA+CD31+ 3% [N> 26%]. Normal B-cell compartment except for  %CD19+CD21Low 60% [N < 9%]. Proliferation tests: anti-CD3 5024 cpm [N > 9000], CD3 +
>> CD28 2016 cpm [N > 80000] PHA 16428 cpm [N > 50000] IonoPMA 69372 cpm [N > 60000].
>> Calcium flux assesment is normal
>> Normal IgG,A,M.
>> RAG1/2, ADA, Artemis: wt
>>
>> Any input will be welcomed, specially concerning treatment Thanks for your help,
>>
>> Luis Ignacio Gonzalez-Granado
>> Immunodeficiencies Unit.
>> Hospital 12 octubre. Madrid. Spain
>>
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