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

[Aristoteles Alvarez Cardona]

Dear All.

I would appreciate your inputs in this case:

14yo hispanic female from non consanguineous parents.

Personal historyof:
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.
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