[CIS PIDD] [cis-pidd] Evans Syndrome, iatrogenous CVID, granulomatous LIP

[Nacho Gonzalez]

I´d try to be sure ALPS has been r/o: B12, sFASL ? (despite DNT borderline)
The best approach for GLILD was recently published JCI Jan (AZP + RTX
better than one alone) http://www.ncbi.nlm.nih.gov/pubmed/22930256

We have tried in one patient (AZP and RTX) and worked. In other, RTX alone
improved GLILD for several years.

Hope this helps,

Luis Ignacio Gonzalez-Granado
Immunodeficiencies.
Hematology. Oncology
Hospital 12 octubre.
Madrid. Spain



2013/11/6 Markus Seidel <markus.seidel at medunigraz.at>


> Dear Colleagues,

>

> what would be your suggestions in the therapeutic dilemma in a young adult

> who

>

> has been treated successfully with MMF for 5 years for Evans Syndrome,

> under that time had a CVID-like B-cell phenotype (<100/µL B cells, absence

> of csmB cells, IgG2 and IgA-def.), did not require Ig-substitution, no

> severe infections,

>

> initially had severe pancytopenia, autoimmune hemolytic anemia,

>

> who then developed granulomatous lymphocytic interstitial pneumonitis

> early this year (LIP, either as consequence of iatrogenous CVID or

> occupational dust exposure; no infectious agent identified)

>

> and when IS was terminated, had recurrence of severe thrombocytopenia

> (<10.000/µL), neutropenia (600-900/µL), autoantibodies against neutrophils,

> platelets, pos. Coombs test, but recovered clinically from LIP, which now

> appears to be stable in Chest CT.

>

> his mother suffered from granulomatosis with polyangiitis (GPA) – but no

> other than blood-cell tissue- or nuclear autoantibodies are detectable in

> the patient.

>

>

>

> I fear that rituximab or other systemic IS would exacerbate his LIP, which

> has been stable for 4 months now under rapamycin (the latter did not

> improve his thrombocytopenia, though); the B cells recovered to >200/µL and

> >2% csmB cells after discontinuation of MMF, but are now lower again under

> rapamycin, DNT cells borderline increased 3-5% of CD3;

>

> Romiplostim works short time (1-2 weeks), but doesn’t look like a very

> good long term option;

>

> Splenectomy?, Danazole?, AZT?

>

> ...SCT?

>

>

>

> Thanks for your comments or thoughts,

>

> Sincerely,

>

> Markus Seidel

>

>

>

>

>

> Markus G. Seidel, M.D., Assoc.Prof.

>

> Consultant| Dept.of Pediatric Hematology-Oncology | Univ.Clinics of

> Pediatric and Adolescent Medicine | Medical University Graz | Auenbruggerpl.

> 34/2 | A-8036 Graz | Austria | T. 0043 316 385 80215| F. 0043 316 385

> 13717 | Secr. 0043 316 385 13485 |

>

> Coordinator of the Working Group for Pediatric Immunology of the Austrian

> Society of Pediatrics and Adolescent Medicine

>

>

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