[CIS PIDD] [cis-pidd] 8yo girl with refractory IBD advise on further immunosuppressive treatment

[Carla Gianelli]

Hello,

I was thinking that Mesenchymal stem cells (MSCs) could be one treatment
option in this patient current situation.

http://www.ncbi.nlm.nih.gov/pubmed/23564182
http://www.ncbi.nlm.nih.gov/pubmed/23296948

Best,

Carla Gianelli

Servicio de Inmunología
Hospital Ramón y Cajal.
Madrid , Spain.





2014-12-08 12:28 GMT+01:00 John Ziegler <j.ziegler at unsw.edu.au>:

>  NBT?
>
>
>
> *From:* Jane Peake [mailto:j.peake at uq.edu.au]
> *Sent:* Monday, December 8, 2014 11:17 AM
> *To:* CIS-PIDD
> *Subject:* [cis-pidd] 8yo girl with refractory IBD advise on further
> immunosuppressive treatment
>
>
>
> I have recently been asked to see this girl that has been under the care
> of gastroenterology colleagues. They are asking what further
> immunosuppression may be appropriate to control her disease. She has
> responded poorly to previous immunosuppressive treatment trials. Any
> thoughts would be very much appreciated.
>
> Kind regards
>
> Jane
>
>
>
> 8 yo girl with early onset aggressive IBD (refractory proctitis).
>
> Blood has been sent for whole exome sequencing.
>
> *Background:*
> Multiple therapies trialled for aggressive proctocolitis including
> surgical and maximal medical therapies
>
> *History:*
>
> Age of onset at 5 years. Severe diarrhoea with blood and mucus, tenesmus
> and faltering growth
> - Early colectomy w ensuing ileostomy and a number of exploratory
> laparotomies for suspected fistulisation/collections around age 6-8y
> - limited response to multiple immune modulators including high dose
> steroids ( IV methylpred and oral pred), Sulfasalazine, AZT, 6MP
> - Failed trials of infliximab (x4) limited / no response and complicated
> by intercurrent severe aphthous ulcerations and buccal/pharyngeal mucositis
> - Possibly some mild response to azithromycin augmented with doxycilicine
> in recent past (last year) but no significant clinical improvement w
> ongoing proctitis
> - Most recent endoscopy revealed marked lymphocytic oesophagitis with
> aphthous ulceration likely correlated w active IBD
>
>
>
> *Systems review: *
>
> Aphthous ulcerations as above. No arthritis. No skin rashes No description
> of uveitis or eye involvement, No bruising, No lymphadenopathy or
> hepatosplemegaly
>
> *Progress:*
> Most recent course of disease complicated by ~12 weeks of giant aphthous
> ulceration to buccal mucosa, active proctitis w suspected fistulisation and
> associated growth faltering/weight loss
>
>
>  Currently inpatient
>
> - MRI shows probable coloenteric fistula and awaiting surgical opinion
>
> - Commenced on bactrim for eradication of MRSA on buccal/tongue
> - Commenced on azithromycin as immune modulator
> - Oral dexamethasone washes to manage buccal disease
> - No other immune suppression – awaiting possible further surgery
> - Still on full enteral diet but clearly very limited solid/fluid intake w
> ensuing weight loss and crossing centiles
>
>
> *Immune function:*
> No evidence of other immune deficiency, autoinflammatory syndrome or other
> autoimmune condition
> -moderate hypergammaglobulinaemia(IgG:15 (7-15), IgA1.8 (0.7-2.5), IgM 0.9
> (0.4-2.3)) but HIV negative
> -Normal isohaemagluttinins 64
> -Normal vaccine titers (tetanus)>1.11
> - normal CBC
>
> - Normal lymphocyte subsets (CD3: 1.49 (0.6-2.4), CD4:0.96 (0.4-1.6), CD8
> 0.5 (0.1-1.0), CD19 0.59 (0.08-0.58), CD56 0.34 (0.05-0.5),
>
> - Normal ACE level
>
> -Normal complement levels C3 1.22(0.9-1.8) and C4:0.35 (0.1-0.4) and
> CH50>1000 (>520)
> -Negative ANA, ENA, dsDNA, antiSMA, anti LKM
> -.Positive ASCA IgA 120 (<20), ASCA IgG 57 (<20) and PR3-ANCA: 72 (<20)
> -Normal ferritin (requires regular iron infusions) but variable elevation
> on CRP 10-20 and ESR ~30
> -No HLAB27 or HLAB51 testing yet
>
> -Hypercellular bone marrow with reactive lymphocytes and some plamacytoid
> forms
>
> ---
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