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

[Sullivan, Kathleen]

I would consider LGL disease.  Often hard to diagnose but associated with various cytopenias that are difficult to treat.

Kate
On Dec 26, 2013, at 9:54 AM, Christine M. Seroogy, MD wrote:


> 

> 

> 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: 

> 

> Continued medical management of ruptured appendix with surgically placed drain after wash-out. Growing two species MDR Pseudomonas, VRE, along with scant Candida.

> Status-post 4 doses of rituxan with last dose 2 weeks ago.

> Cellcept 600mg/m2/dose IV for 2 weeks

> Repeat bone marrow last week with essentially absent granulocytic/monocytic precursors and persistence of lymphoid aggregates, paucity of B cells. Other lineages are normal.

> Receiving WBC transfusions daily for 2 weeks.

> Infectious work-up (EBV, HIV, adeno, CMV) negative. Anti-neutrophil antibodies are positive.

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



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