[CIS PIDD] [cis-pidd] cyclic neutropenia in a 14 yo non SLE female?

[Jason W. Caldwell]

Periodic fever syndromes could be on the differential.
Familiar med fever, traps, hyper IgD.
Sent from my iPhone

On May 2, 2014, at 8:48 PM, "Aristoteles Alvarez Cardona" <dr.aristoteles at yahoo.com.mx<mailto:dr.aristoteles at yahoo.com.mx>> wrote:

Dear All.

I would appreciate your inputs in this case:

14yo hispanic female from non consanguineous parents.

Personal history of:
Atopic dermatitis and mild allergic rhinitis.
Acquired hypoparatiroidism receiving levothyroxine.
Not severe or recurrent infections

She was sent to me because recurrent events of chills and malaise along with neutropenia.

Unremarkable physical examination.

Lab:
Absolute neutrophils by day: February 28th (1122) March 4th (546)  7th (1472)  11th (1722)  14th (2400) 18th (3162) 21st (1700)

Hb 13.95 Platelets 310,000. Leukocytes 5000 Neutrophils 32% Lymphocytes 56% Monocytes 10% No abnormal cells.

Previous neutropenic states around 400 absolutes.

IgG 974mg/dl, IgA 192mg/dl, IgM102mg/dl,  IgE 89.3U/ml

Anti thyroid antibodies 246 (0-12)
ANA, Sm and Antiphospholipid antibodies, all negative.
C3 103.4 (55-120) C4 19.1 (20-45)

Unremarkable Urinalysis

Now, by definition cyclic neutropenia should have a nadir below 200 absolute neutrophils and improves with age, she is now 14 and only agreed to have 2 weekly blood tests but here is an evident decrease on neutrophils every 3 weeks.

She has been free of severe infections but she suffers from chills and discomfort every month (nadir?) not affecting her QoL. She is doing well on everyday life.

I am sending her to hematology for a multidisciplinary approach and follow up.

We have suggested antibiotics if needed but, should I just keep an eye on her once in a while? should I test for anti neutrophil Ab? considering she is otherwise fine.

Thank you on advance.

Dr. Aristoteles Alvarez Cardona
Alergia e Inmunología Clínica Pediátricas
Universidad Autonoma de Aguascalientes.


---

The CIS-PIDD listserv is supported by:

[http://www.clinimmsoc.org/UserFiles/image/cis-pidd-list-logo_v1.jpg]
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org<mailto:info at clinimmsoc.org>

Not a member of CIS? Please visit www.clinimmsoc.org<https://cis.execinc.com/edibo/Signup> to join!

You are currently subscribed to cis-pidd as: jcaldwel at wakehealth.edu<mailto:jcaldwel at wakehealth.edu>.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824410.ddf818e4358b184e64d2b6672b9125e2&n=T&l=cis-pidd&o=44835285


---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=44835442
or send a blank email to leave-44835442-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org