Autoimmune Neutropenia: Please may I ask if... - Behçet's UK

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Autoimmune Neutropenia

NikD
NikD

Please may I ask if anyone has had experience of autoimmune neutropenia as I have developed this secondary to my Behcets. Basically I have developed an antibody which is destroying my own neutrophils. This is following a year of being unwell with several illnesses and being unable to take my immunosuppressants to manage my Behcets. The treatment is steroids and immunosuppressants to suppress the antibody which initially will drop my neutrophils further putting me at risk of infection but then they should return to normal.

I was wondering if anyone has had this and seen it improve?

Thanks 😊

6 Replies
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Hi my son had neutropenic sepsis as part of his last flare. He was treated with filgrastim and then iv steroids, he made a complete recovery so stay positive. He was really poorly for several weeks low platelets, virtually non existent neutrophils, low bp, low iron, he lost 8kg in 4 weeks, temp was 41+, erythema nodosum, inflammation in gall bladder, spleen, parotid and lymph glands, small bowel and rectal stump (got ileostomy from ulcerative colitis), joints etc. They had thought HLH or severe infection, he had numerous tests, scans, biopsies before they concluded it was Behcets flare and gave iv steroids, he responded quite quickly and then home to continue with prednisolone 40 mg. this was then gradually reduced (and stopped) and resumed humira. This was 20 months ago and has only had minors such as ulcers since then. Good luck, hope it’s samefor you x

NikD
NikD in reply to Leonsmum

Thanks for your reply and hope your son stays well 😊

I was diagnosed with hypogammagobulenemia back in 2008 at the Mayo Clinic in Minnesota after years of being in and out of the hospital with pneumonia and they believe I had probably had it from birth due to the ear infections and abscesses that I used to get all the time. I have to be infused every three weeks with IgG's to help keep it under control. I still end up being hospitalized once or twice a year for a few weeks at a time because I need IV steroids' and antibiotics. I'm only able to use Colicine and Plaquenil to treat my Behcets because the immunosuppressants' would make my other condition worse. I'm also followed by Oncology because my light chains and gamma kappa's are always off. I was diagnosed with Behcets at Mass General in Boston 7 years ago. Now my IgA's have depleted in half and my immunologist says there is nothing they can do about that so I will be open to other infections. Last February I got really sick and there is a good possibility I may have Covid but because I tested positive for Flu B they weren't able to get me tested for Covid and now I am in Heart Failure, that's why they think I may have had it and it effected my heart... Any way's I looked up your diagnosis and it's very similar to mine so I wonder if we all end up or are more susceptible to these other conditions due to that. My dermatologist and Rheumatologist really want me on the immunosuppressants as my skin is being destroyed. I am only 53 by the way...

NikD
NikD in reply to nysunshine12828

Hi sorry to hear about your health, you’re definitely having a tough time. I am generally very fit and this all started with a stroke 3 1/2 years ago which I have fully recovered. Behcets was diagnosed as the blood clot in my brain is thought to have been caused by the inflammation associated with the disease. My rheumatologist is confident that my neutrophils will return to normal with the right treatment and I have a lot of confidence that she is right.

I know having one autoimmune condition makes you susceptible to others and quite often people may have 2 autoimmune disorders.

With regards to Covid I have had this and had antibodies in August. I was on steroids at the time if contracting COVID-19 which I think reduced the impact of the symptoms however I do believe that this is having a long term effect on my condition as I have had shingles twice since June and blood picture overall not great since Covid.

I hope you continue to be well and thank you for your reply. 😊

nysunshine12828
nysunshine12828 in reply to NikD

Wow, people just don't understand that when you have covid it's not like, yup I had it and recovered. Many are having issues long after. I'm sorry you went through that. I wonder if you being on steroids' did change the impact? There are so many unknowns with it and there are many that are considered long haulers.

It makes sense really that you have had issues with shingles afterwards because there are a lot of people that have neurological issues afterwards and you know every time you get sick and or are steroids' it just opens you to more.

Hope you continue to recover. Stay safe

Hoping you & your physicians find that balance. I lived 6 months with a 0.2 white blood cell count with almost no absolute neutrophils and felt my best. But of course the risk of dying/infection made my docs nervous.With rituxan infusions & daily revlimid i am doing very well with a wbc count around 4.0

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