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

[Markus Seidel]

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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