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

[Kumar, Ashish]

We have a patient exactly like this, a 22 yo female. Her marrow showed complete myeloid aplasia and lymphoid aggregates. She responded promptly to ATG and Cyclosporin (aplastic anemia therapy). She had a normal ANC by the last dose of ATG. However, the response was short lived and in a month everything was back to the same. We thought of LGL too, but these T-cells are polyclonal. We found a donor and are moving to BMT.
Ashish

Ashish Kumar, MD, PhD
Cancer and Blood Diseases Institute
Division of Bone Marrow Transplant and Immune Deficiency
Cincinnati Children's Hospital Medical Center

On Dec 26, 2013, at 9:56 AM, "Christine M. Seroogy, MD" <cmseroogy at pediatrics.wisc.edu<mailto:cmseroogy at pediatrics.wisc.edu>> 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:


  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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