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

[prockops at MSKCC.ORG]

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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