[PAGID] giant cell hepatitis and AIHA

[Jack Bleesing]

We have seen similar cases: all boys (looked at IPEX as well - none found so far) following the cited reference.

All have responded to rituximab; none needed to be transplanted. No relapse to far.

Regards,

JB

---------------------------------------------------------------------------
Jack J.H. Bleesing, M.D., Ph.D.
Cincinnati Children's Hospital Medical Center
Division of Hematology/Oncology
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)
Jack.Bleesing at CCHMC.org
http://www.cincinnatichildrens.org/immunodeficiencies/


>>> "Jung, Lawrence" <ljung at chsomaha.org> 05/09/08 12:25 PM >>>

Colleagues,

 

Your thoughts on the following case, please.

 

This is a 3 yr old boy presented at about 1 year of age with severe
autoimmune hemolytic anemia following a few months later with fulminant
hepatitis that ultimately resulted in liver failure requiring a liver
transplantation.  Giant cell hepatitis was noted on pathology.
Infectious etiology not found.  This presentation was fairly
characteristic of the described syndrome of Giant cell hepatitis with
AIHA.  (J. Ped Hematol Oncol. 26:837 - 839, 2004).  

 

No IPEX mutation found (Cincinnati Children's  Genetics).  CD3#-1860 CD4
-1485, CD8- 340; CD19-367.  IgA and IgM were low (can't find the data at
this moment).  IgG half-life - 3 weeks after IVIg.  I don't believe
there was any immune work-up until after liver transplantation and
immunosuppression.

 

He was placed on various immunosuppressive therapies: po and pulse
steroid, rituximab, cellcept, FK506.  2 course of rituxmab were
ineffective in controlling AIHA.  Currently he is on rapamycin which is
able to stabilize his condition.  He is also on bi-weekly IVIg because
of hypogammaglobulinemia, probably due to the immunosuppression
including rituximab.  

 

He continues to show disease activity with episodic jaundice, high retic
counts, anemia and elevated AST (800-900) ALT (400 - 600).  He rapamycin
level is about 10 - 12.

 

My questions to the group, especially to those experienced in this
disorder are:

(1) what should be done next to save his transplanted liver?  The family
is resistant to the use of high dose steroid.  

(2) How high can we push rapamycin?  Would cytoxan be useful?  More
rituximab?

 

Any comments will be welcomed.

 

Larry Jung

Omaha, NE

 



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