[CIS PIDD] [cis-pidd] Rituximab s.c for GLILD therapy

[CIS-PIDD]

Dear Dr Rothenfusser,
in my experience, GLILD in a 10yo boy with "cvid" (rather CID with predominant hypogamma, Bmem deficiency, and LPD) melted away during 4-6 months under a combination of one cycle of 4x375 Rituximab plus one dose of prednisone at each infusion and concomitant start of sirolimus, the latter continued over one year, now being slowly tapered. (and IgG replacement, as before).
How was your dx of glild verified? What is the underlying cvid subtype/pathomechanism, was apds excluded...?
More details per personal contact, if you wish.
Best,
Markus Seidel

Univ.-Prof. Dr. Markus G. Seidel | P?diatrische H?matologie-Onkologie | Med. Univ. Graz | Auenbruggerplatz 38 | 8036 GRAZ | Austria | Tel +43 316 385 80215 | Secr +43 316 385 13485 | Fax +43 316 385 13717 | sent from my mobile phone | please excuse typos

Am 06.10.2016 um 11:19 schrieb CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>:

Dear colleagues,

I intend to treat an adult CVID patient with GLILD with the rituximab/Azathioprin regimen suggested by John M. Routes with four weekly infusions of 375 mg/M2 i.v. that are then repeated every 4-6 month. As the patient worries about the time off work it would be more convenient for her to get Rituximab s.c. as the 1400 mg fix-dose given within a couple of minutes instead of the infusions that take several hours.
Has anybody used the sc. version of Rituximab for GLILD/CVID and could suggest a regimen that might in effect be similar to John Routes's iv. regimen? Weekly injection of Rituximab 1400 mg do not seem to be in use for Lymphoma and just giving replacing the weekly iv. doses by the sc. Dose might be too much?

Thanks for advice!

Prof. Dr. Simon Rothenfusser
Immundefekt-Ambulanz f?r Erwachsene ("AIDA")
Medizinische Klinik IV
Klinikum der Ludwigs-Maximilian Universit?t
Pettenkoferstra?e 8a, 80336 M?nchen

Tel. *49-89-4400-53568
Fax: 089/4400-53719
E-mail: Simon.rothenfusser at med.uni-muenchen.de<mailto:Simon.rothenfusser at med.uni-muenchen.de>

www.klinikum.uni-muenchen.de/Medizinische-Poliklinik-Innenstadt/de/rheumaeinheit/rheuma-ambulanz/<http://www.klinikum.uni-muenchen.de/Medizinische-Poliklinik-Innenstadt/de/rheumaeinheit/rheuma-ambulanz/>

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