[CIS PIDD] [cis-pidd] ADA SCID with partial reconstitution and chronic leptomeningitis/encephalitis

[João Pedro Marques Farela Neves]

Mycobacteria? 
CSF with Low glucose, elevated protein + lymphs 
leptomeningitis
granulomatous inflammation…

João Farela Neves
Primmary Immunodeficiencies unit
Hospital Dona Estefânia
Lisbon
Portugal


A 08/08/2013, às 20:37, "Boyce, Thomas G., M.D." <Boyce.Thomas at mayo.edu> escreveu:


> ?listeria

>  

> From: bounce-43682766-183824398 at lists.clinimmsoc.org [mailto:bounce-43682766-183824398 at lists.clinimmsoc.org] On Behalf Of Christine M. Seroogy, MD

> Sent: Thursday, August 08, 2013 7:42 AM

> To: CIS-PIDD

> Subject: [cis-pidd] ADA SCID with partial reconstitution and chronic leptomeningitis/encephalitis

>  

>  

> Dear Colleagues,

>  

> We are caring for a 14 y/o with ADA SCID s/p non-conditioned haploidentical BMT x 2 with a 4 month history of intermittent fevers, headaches, weight loss, myalgias.  He has 100% donor T cells with adequate function and low T cell numbers, on IG therapy, and off of PEG-ADA for years but empirically restarted 2 weeks ago.  His most recent brain/spine MRI demonstrated enhancement of meninges and some parenchymal enhancement (worsening from previous studies).  His CSF has increased lymphs/monos with high protein and low glucose.He had a brain bx 10 days ago:  with no viral inclusions, fungi or bacteria visible (EM and special stains including AFB). There is granulomatous inflammation with no discrete granulomas with numerous macrophages and T cells (CD4:CD8 ratio 3:1), no B cells. One possibility is a neurosarcoid-like picture. Imaging elsewhere does not demonstrate obvious organ involvement outside the CNS.

>  

> His notable history in the past year is one episode of ITP responsive to 4 doses of rituxan and non-infectious uveitis responsive to topical steroids.  

>  

> He has had an extensive infectious disease work-up and my question to the group is if there is an infectious agent we may be missing? Have any of you seen such a case?

> Here is the infectious work up to date:

>  

> Studies: 

> Bacterial-- 

> 4/29/13 Culture, CSF negative 

> 4/29/13 Lyme PCR (CSF) negative 

> 4/29/13 Bartonella PCR (CSF) negative 

> 4/29/13 AFB smear/culture (CSF) negative 

> 7/11/13 blood, peripheral: negative 

> 7/12/13 blood, peripheral: negative 

> 7/14/13 CSF culture, negative 

> 7/14/13 AFB smear & culture, CSF Negative 

> 7/14/13 16s ribosomal PCR, CSF negative 

> 7/26/13 CSF culture negative 

> Viral-- 

> 4/8/13 EBV PCR negative 

> 4/8/13 CMV PCR negative 

> 4/8/13 Flu A/B, RSV PCR negative 

> 4/9/13 Enterovirus PCR negative 

> 4/23/13 Respiratory viral panel (WI state lab): negative 

> 4/29/13 West Nile PCR (CSF) negative 

> 4/29/13 adenovirus PCR (CSF) negative 

> 4/29/13 CMV PCR (CSF) negative 

> 4/29/13 Arbovirus Ig panel (CSF) Negative 

> 4/29/13 HHV-6 PCR (CSF) Negative 

> 4/29/13 HHV-8 PCR (CSF) negative 

> 4/29/13 HSV PCR (CSF) negative 

> 4/29/13 Enteroviral PCR (CSF) negative 

> 4/29/13 VZV PCR (CSF) negative 

> 4/29/13 EBV PCR (CSF) negative 

> 5/1/13 Enterovirus RNA PCR, stool negative 

> 7/8/13 Resp viral panel (Viracor): positive for rhinovirus 

> 7/8/13 Enterovirus PCR negative 

> 7/11/13 BK virus qPCR: negative 

> 7/12/13 JC virus PCR (blood): positive (very low levels: initially postive at 42 cycles, repeat positive at 36 cycles) 

> 7/12/13 adenovirus PCR: negative 

> 7/12/13 enterovirus PCR: negative 

> 7/14/13 CMV PCR (CSF) negative 

> 7/14/13 JC virus PCR (CSF): negative 

> 7/14/13 BK virus PCR (CSF) negative 

> 7/14/13 Enterovirus PCR (CSF) negative 

> 7/14/13 Adenovirus PCR (CSF) negative 

> 7/14/13 EBV PCR (CSF) negative 

> 7/14/13 LCM IgG/M (CSF) pending 

> 7/14/13 Herpes simplex PCR (CSF) negative 

> 7/16/13 respiratory viral panel (Viracor) positive for rhinovirus 

> 7/16/13 Herpes simplex PCR (blood) negative 

> 7/18/13 blood JC virus qPCR <500/mL 

> 7/23/13 Blood HTLV PCR Negative 

> 7/23/13 Blood HIV RNA, qPCR Not detected 

> 7/26/13 VZV PCR from brain biopsy negative 

> 7/26/13 EBV PCR from brain biopsy negative 

> 7/26/13 CMV PCR from brain biopsy negative 

> 7/26/13 adenovirus PCR from brain biopsy negative 

> 7/26/13 adenovirus PCR from CSF negative 

> 7/26/13 enterovirus PCR from brain biopsy negative 

> 7/26/13 coxsackie/echovirus PCR from rectal swab negative 

> 7/26/13 coxsackie/echovirus PCR, throat swab negative 

> 7/26/13 EBV RTPCR, CSF negative 

> 7/26/13 VZV RTPCR, CSF negative 

> 7/26/13 CMV RTPCR, CSF negative 

> 7/26/13 enterovirus RTPCR, CSF negative 

> 7/26/13 enterovirus culture from CSF, brain, and dura negative at 10 days 

> 8/2/13 respiratory virus panel (in-house) negative 

> Fungal-- 

> 4/29/13 Fungal culture, CSF negative 

> 4/29/13 Histoplasma Ag (CSF) negative 

> 4/29/13 Blastomyces Ag (CSF) negative 

> 4/29/13 Cryptococcal AG, CSF negative 

> 7/14/13 Blastomyces antigen, urine negative 

> 7/14/13 Histoplasma antigen, urine negative 

> 7/14/13 CSF fungal culture NGTD 

> 7/14/13 Cryptococcus antigen, CSF negative 

> 7/26/13 Fungal culture, CSF NGTD 

> Other-- 

> 4/29/13 Mycoplasma PCR CSF negative 

> 7/14/13 Mycoplasma PCR CSF negative 

> 7/14/13 Toxoplasma PCR CSF negative 

>  

> Thank you

>  

> Chris

>  

> 

> Christine M. Seroogy MD,  FAAAAI

> Associate Professor

> University of Wisconsin

> Department of Pediatrics

> Division of Allergy, Immunology & Rheumatology

> 1111 Highland Avenue

> 4139 WIMR

> Madison, WI  53705-2275

> phone: 608-263-2652

> fax: 608-265-9721

>  

>  

> 

> 

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