[CIS PIDD] [cis-pidd] CVID patient with agranulocytosis

[Christine M. Seroogy, MD]

Thank you all for the helpful responses. Here is some follow up.

We looked carefully for LGL in our patient and this was ruled out.

He has now received 4 doses of hATG and CsA was started.  One week after the last hATG dose, his neutrophils and monocytes have started to normalize.  Hopefully soon the surgeons will be willing to remove the necrotic appendix.

His healthy sister is being HLA-typed in case we need to go that route and if she is a match, an easier decision.   Unfortunately, the insurance company has denied WES/WGS stating it was "experimental".

Chris



From: <Cunningham-Rundles>, "charlotte.cunningham-rundles at mssm.edu<mailto:charlotte.cunningham-rundles at mssm.edu>" <charlotte.cunningham-rundles at mssm.edu<mailto:charlotte.cunningham-rundles at mssm.edu>>
Reply-To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Date: Thursday, December 26, 2013 12:22 PM
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Subject: Re: [cis-pidd] CVID patient with agranulocytosis

I saw a very similar case from Yale this year, carried as "CVID" ; a young woman  who developed  intractable neutropenia,  not responsive to G-CSF for about a year + , also just transplanted. So far, so good.

I suspect that is the only way to go here. But save DNA, as likely this is something genetic and important to know about.

Good luck and regards,

Charlotte

Charlotte Cunningham-Rundles MD PhD
David S. Gottesman Professor
Department of Medicine
Mount Sinai School of Medicine
1425 Madison Avenue
212 987 5593 ( fax)
212 659 9268 ( phone)




From: "<Christine M. Seroogy>", MD <cmseroogy at pediatrics.wisc.edu<mailto:cmseroogy at pediatrics.wisc.edu>>
Reply-To: "cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>" <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Date: Thursday, December 26, 2013 9:54 AM
To: "cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>" <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Subject: [cis-pidd] CVID patient with agranulocytosis



Dear Colleagues,

I am seeking advice on alternative therapies for a 20 y/o CVID patient with severe autoimmune neutropenia.

He presented 8 years ago with autoimmune hemolytic anemia and met criteria for CVID.  He was in good health on SQIG until Sept. of this year when his neutrophil count started to drop.  This was initially attributed to medication side effect (abilify started in Feb 2013 for bipolar disease).  The medication was stopped without improvement in his neutrophil count.  He was given neulasta with brief increase in neutrophil count.  He was hospitalized at an outside facility with fever and neutropenia in Oct 2013.  Bone marrow biopsy showed decreased granulocytic precursors, lymphoid aggregates (CD4+).  He was treated with prednisone and high-dose IVIG without response.  Unfortunately, he developed a surgical abdomen and was found to have a ruptured appendix.  This was medically managed.  He was subsequently hospitalized at our facility (mid Nov 2013).  Pertinent data:


  1.  Continued medical management of ruptured appendix with surgically placed drain after wash-out. Growing two species MDR Pseudomonas, VRE, along with scant Candida.
  2.  Status-post 4 doses of rituxan with last dose 2 weeks ago.
  3.  Cellcept 600mg/m2/dose IV for 2 weeks
  4.  Repeat bone marrow last week with essentially absent granulocytic/monocytic precursors and persistence of lymphoid aggregates, paucity of B cells. Other lineages are normal.
  5.  Receiving WBC transfusions daily for 2 weeks.
  6.  Infectious work-up (EBV, HIV, adeno, CMV) negative. Anti-neutrophil antibodies are positive.
  7.  Receiving monthly IVIG at 500 mg/kg and on 20 mg prednisone daily.

Unfortunately, his neutrophil count has not improved.  He is clinically "stable" with the present management and our surgical team will not remove his ruptured appendix with walled off abscess at this time given his poor healing risk. With his bone marrow evolving to an agranulocytosis picture, we are considering other treatment options.  One thought is to discontinue the cellcept and start cyclosporin.

Have others treated such a patient? Any thoughts on additional approaches would be appreciated.

Thank you

Chris


Christine M. Seroogy MD,  FAAAAI
Associate Professor
University of Wisconsin School of Medicine and Public Health
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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