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

[Nacho Gonzalez]

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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