[CIS PIDD] [cis-pidd] NLH and rituximab

Tue May 26 13:28:07 EDT 2015

We had a very dramatic case referred to Rainbow Babies and Childrens' Hospital from Pennsylvania. A boy of about 4-5 years old had a history of antibody deficiency with infections, NLH (causing recurrent rectal prolapse), hemolytic anemia s/p splenectomy, diabetes secondary to steroids, and inability to maintain protective serum IgG levels despite IVIG doses > 1 gr/kg/mo.  Whilre in Cleveland, he had an exacerbation of hemolytic anemia which was treated with Rituximab. This resulted in resolution of the episode of anemia, a marked decrease in GI symptoms, cessation of rectal prolapses, and a marked rise in the serum IgG level. The latter was presumably due to decreased GI losses.  In retrospect, the biopsy of the lymphoid nodules reported "plasmacytoid cells with positive staining for CD20", but no one had ever suggested using Rituximab as a primary therapy for the GI problems, it was a very happy serendipitous effect of its use for the hemolytic anemia.  Subsequently, he continued to do quite well on easily manageable IgG replacement and Rituxin once or twice a year for recurrences of hemolytic anemia.

Mel Berger

(Now I wonder if he has a PI3K or PKC mutation)

-----Original Message-----
From: Sullivan, Kathleen [mailto:SULLIVANK at email.chop.edu]
Sent: Tuesday, May 26, 2015 1:08 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] NLH and rituximab

Nodular lymphoid hyperplasia in CVID.

Thanks everyone!

Kate
Kate Sullivan, MD PhD
Wallace Chair
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363

> On May 26, 2015, at 12:57 PM, Church, Joseph <JChurch at chla.usc.edu> wrote:
>
> Sorry Kate, but what is NLH?
> Joe Church
>
> -----Original Message-----
> From: Sullivan, Kathleen [mailto:SULLIVANK at email.chop.edu]
> Sent: Tuesday, May 26, 2015 7:09 AM
> To: CIS-PIDD
> Subject: [cis-pidd] NLH and rituximab
>
> I have a CVID patient who has been denied rituximab for NLH by her insurance company for lack of data.  It is true that there are no published studies.  I think they will accept “collective knowledge”.
>
> If you have treated a patient with CVID and NLH with rituximab successfully, would you be so kind as to weigh in on this list serve so I can compile the information and present it to the insurance company?
>
> If others have faced this- perhaps we could channel these data into a short report so it is out there for others to use.
>
>
> Kate
> Kate Sullivan, MD PhD
> Wallace Chair
> Chief of Allergy Immunology
> ARC 1216 CHOP
> 3615 Civic Center Blvd.
> Philadelphia, PA 19104
> (p) 215-590-1697
> (f) 267-426-0363
>
>
>
>
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