[CIS PIDD] [cis-pidd] Antibody mediated destruction and eosinophilia

[CIS-PIDD]

Hi everyone! I am looking for some help with a complicated baby that I am currently caring for. Thank you in advanced!

The patient is a now 10-month-old female infant whom I met back in September 2015 at 5 months of age. She was referred to me from her PCP after she presented with low platelets. On physical exam she has no physical sequelae, no lymphadenopathy, and is developmentally appropriate. Initial labs I did showed she wasn’t a straightforward ITP type patient as her counts showed platelets of 14, WBC 19 (ANC 200, absolute eos 3,470). She tested positive for rhino/enterovirus at the time and we considered her to have a viral mediated process going on that suppressed her counts. Other than bruising and a petechial rash she was well appearing. I decided to admit and she received two doses of IVIg during that admission. She appeared to respond to the IVIg over the course of several days with her platelet count going to 368 and her ANC 1.27, Eos 4.43. I was concerned with her eosinophilia however given lack of any secondary effects decided to monitor.

Her counts fell by the following week with platelets of 29, ANC 90 and eosinophils of 640. She received another dose of IVIg with plan for a bone marrow. She was noted to have a Hgb of 7.4 overnight prior to the procedure and was transfused. She had positive warm autoantibodies on her type and cross. She was also noted to have anti-neutrophil antibodies and positive platelet antibodies. She didn’t respond to IVIg this time, a bone marrow was done at this time.

The bone marrow biopsy returned interesting findings that showed a background of “damage” with marked increase in the number of eosinophils seen. She had otherwise normal cellularity with an increase in granulopoiesis and megakaryopoiesis and diminished erythropoiesis. The reticulin stain showed mild increase in fibrosis. Cytogenetics was normal.

Other tests that were sent prior to initiating steroids include C1Q binding (elevated at 7.6), C3, C4 and total complement were all normal. Also checked FECP eosinophilic cation protein that was markedly elevated at 140. Lymphocyte subsets have been checked twice and both times were normal.

Given the lack of malignant changes seen and the continued decrease in counts I started her on oral steroids at 2 mg/kg/day. She showed a response in her platelets and ANC, however she did require another pRBC transfusion. She has never had a robust reticulocyte count given the level of anemia she has experienced. We checked for infectious causes (parvo, EBV, CMV) which have been negative. Also of note, her MCV has steadily climbed over time, starting at 77 now in the 90s.

I was able to wean down on her steroids once a week for several weeks until I reached 4.5 mg/day (she weighs ~7.5- now 8 kg). When I wean to 3 mg/day she then has a rebound in her counts falling and I have to go back up on the steroids, this has happened three times now. Interestingly when her ANC and Hgb fall there is a small but noticeable increase in her eosinophils, her platelets have been stable and not thrombocytopenic.

>From my standpoint she appears to have both a destruction issue (with the antibody production that has persisted, especially with her positive type and screen) as well as some degree of production of red cell issue given the inappropriate reticulocyte count in the face of anemia. The issue of the increased eosinophils also bothers me, she did have a peak of over 4,000 near the beginning and they have been less than 500 since steroids. However as soon as I start to lift the immunosuppression they do start to come back.

I repeated her bone marrow recently and it looks basically normal for age at this point, there is no longer an increase in eosinophils (but she is also immunosuppressed).

Any thoughts would be wonderful.

Wendy


Wendy Darlington, MD, MAPP

Clinical Instructor, Department of Pediatrics

Section of Hematology/Oncology

The University of Chicago Medicine

Comer Children's Hospital

5841 Maryland Ave, MC 4060

Chicago, IL 60637

773-702-6808

pager 3680

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