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

Thu Aug 7 19:56:44 EDT 2014

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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