Autoimmune Neutropenia

Decreased White Blood Cell Counts in Children

Jun 6, 2007 Elaine Moore

Autoimmune neutropenia, which primarily occurs in infants, is characterized by antibodies that destroy neutrophilic white blood cells, increasing the risk of infection.

Primary autoimmune neutropenia (AIN) is an autoimmune disorder first reported in 1975 that primarily occurs in infancy. In autoimmune neutropenia, the immune system produces autoantibodies directed against the neutrophilic protein antigens in white blood cells known as granulocytic neutrophils (granulocytes, segmented neutrophils, segs, polysegmented neutrophils, polys). These antibodies destroy granulocytic neutrophils. Consequently, patients with autoimmune neutropenia have low levels of granulocytic neutrophilic white blood cells causing a condition of neutropenia. Neutropenia causes an increased risk of infection from organisms that the body could normally fight easily.

Who is Affected?

Primary autoimmune neutropenia has been reported as early as the second month of life although most cases are diagnosed in children between 5 and 15 months of age. Girls have a slightly higher risk of developing AIN than boys. In neutropenia discovered at birth or shortly after birth, a diagnosis of allo-immune neutropenia (from maternal white blood cell antibodies passively transferred to the infant) is more likely.

Neutropenia

In infants neutropenia is defined by absolute neutrophil counts less than 1000/uL. After the first year of life neutropenia is defined by absolute counts less than 100/uL. Neutropenia may be primary in which is the only blood abnormality seen. In secondary neutropenia, other primary conditions occur, including other autoimmune diseases, infections, and malignancies. Neutropenia is considered chronic when it persists for more than 6 months.

Symptoms and Disease Course

Neutropenia, which may be discovered on routine blood tests, typically causes benign infections even when the condition is severe. Ear infections (otitis media) are the most common infection seen in autoimmune neutropenia and typically infection responds to antibiotic treatment alone. Infections associated with primary AIN are usually mild and limited, including skin infections such as impetigo, gastroenteritis, upper respiratory tract infections, and ear infections. Rarely, cellulitis and abscesses may occur.

Studies of children studied for up to six years showed that most cases of autoimmune neutropenia resolved spontaneously after a median of 17 months. In 80 percent of patients, neutropenia persisted for 7 to 24 months.

Diagnosis

Patients with autoimmune neutropenia are diagnosed on the basis of blood tests showing neutropenia and the presence of granulocyte-specific antibodies. In some cases, tests for granulocyte-specific antibodies need to be repeated several times before a positive result is seen. Bone marrow aspiration, if performed, is typically normal or it can show increased cell production with a variably diminished number of segmented granulocytes.

As association with prior parvovirus B19 has been made but this hasn’t been confirmed. Similar to the platelet deficiency idiopathic thrombocytopenic purpura, vaccines are suspected of triggering this disorder.

Treatment

Treatment consists of corticosteroids to reduce autoantibody production, antibiotics to prevent infection and granulocyte colony-stimulating factor (G-CSF) to temporarily increase neutrophil counts. In cases of severe infection or the need for surgery, intravenous immunoglobulin therapy may be used.

Resources:

Juergen Bux, Georg Behrens, Gudrun Jaeger, and Karl Welte, Diagnosis and Clinical Course of Autoimmune Neutropenia in Infancy: Analysis of 240 Cases, Blood Vol 91, Jan 1998: 181-186.

Susumu Inoue, Autoimmune and Chronic Benign Neutropenia, eMedicine from WebMed, June 13, 2006, accessed Sept 29, 2006.

The copyright of the article Autoimmune Neutropenia in General Medicine is owned by Elaine Moore. Permission to republish Autoimmune Neutropenia in print or online must be granted by the author in writing.

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32 Comments

Comments

Aug 28, 2008 10:48 AM

Guest :

my infant has this teyp of illens

Oct 18, 2008 8:25 PM

Guest :

my son has had autoimmune chronic neutopenia for three years now and has had success controlling it with neupogen treatments but his condition still remains the same.

Oct 31, 2008 7:47 PM

Guest :

My son has autoimmune neutropenia too. He is now 18 months old and takes neupogen injections every three days. If other moms would like to discuss this condition and how it affects their children, or are looking for a mom who has been through similar situations, I would love to chat. My email is kellyeroach@yahoo.com

Nov 18, 2008 4:31 PM

Guest :

my daughter has had this for 2 1/2 years. she is 4 now. she is off and on neupogen depending on her infections and count. we were recently told she may not outgrow it till the age of ten. this really is such a heartbreaking illness. take care to all!

Nov 21, 2008 9:16 AM

Guest :

My son was diagnosed at 9 months but was sick starting at 6 months. He threw up everything, had a red face and always was warm. One day he had a high fever for 3 days and his hands and feet were puffy. The intial visit, the dr. said it was just a cold but I persisted and she ordered blood work. My son has a severe low count at 0.05. It has been very low for 9 months now. He does not take the injections at this time. We have been told that he should outgrow by age 5 or so. Diet and rest play a huge part in living healthy. He gets really sick every 3 months where he has to be admitted.He's had baby measles, chest infection and a few ear infections. He literally threw up everything up to age 15 months. He has good days and bad days. The bad days...well...he is achy all over, doesn't eat, hardly drinks, sleeps a ton and is just not himself. Yet, he can bounce right back the next day. On the outside he is a healthy looking boy, he's off the growth charts by 20%. This has been life changing especially with older kids. No swimming pools, no play groups, no drop -in classes or travel. Supportive family and friends make it easier.

Dec 12, 2008 2:27 PM

Guest :

my daughter has this illness but we have never heard of the neupogen treatment. she was diagnosed at ten months and is now three and a half. She attends Crumlin hospital in Dublin.

Dec 18, 2008 2:03 PM

Guest :

my son first had problems at 8 months, he was addmitted to hospital with sickness, it was discovered his count was only 0.01, but it took months before he was sent to addenbrookes. we go every 6 months, i find the blood tests really heartbreaking as he screams everytime. i was always told that the majority of children outgrow this by 2, my son is nearly 3 and the highest his count has ever been is 0.5. i would really like to hear from other parents living through this isolating illness, im only 23 and have a daughter of 1 who is about to be tested for the same condition. my email is lucy.baldwin@sky.com. i would also like to say MERRY CHRISTMAS!!!

Jan 7, 2009 8:03 PM

Guest :

My husband has this blood disorder. He was diagnosed at 2 years old and is now 26 and still taking the neupogen shots. We just took my 15 month old daughter to be tested and her neutrophil count came back at .2 and she is slightly anemic. We are taking her to see a pediatric hematoligist/oncologist friday. I would like to hear from other people who are dealing with the same illness. My email is Kandiangel17@yahoo.com. I have a few questions so if anyone knows the answers that would really help me. Is this condition hereditary? Has anyone had this disease longer than a few years? What are the symptoms that your child has? My daughter has had numerous ear infections (enough that they wanted to put in ear tubes, I did not have the operation done because I was not comfortable with the procedure),she gets boils on her diaper area that they have tested and it has come back as a staph infection, she also gets painfull white sores in her mouth and she now has tonsillitis. My husband also gets the mouth sores and boils on his body. I would love to hear from anyone who is also dealing with this disease.

Jan 16, 2009 1:32 PM

Guest :

My daughter was diagnosed January 2008. She gets ill very quickly and this past year our second home has been Yorkhill. The only meds she is on is an antibiotic every night although she has been given GCSF which i have my fears about. It feels abit better knowing we are not facing this alone. Would like to hear from anyone in the same place and would like to get in touch my email is jacquimck@live.co.uk

Mar 7, 2009 4:04 PM

Guest :

My little girl is nearly 4, she became ill and had her first blood test at 21 months, which showed her neutrophils ay 0.2, since then her neutrophil counts each month have varied between 0.01 and 1.1 (higher when ill).
She is on prophalctic anti-biotics and responded to GCSF once. We live in hope of her recovery and fear her infections (we tend to be hospitalized every 2 - 3 mths for IV anti-biotics).BEST WISHES TO ALL ESPECIALLY THOSE IN SIMILAR CIRCUMSTANCES

Mar 11, 2009 3:01 PM

Guest :

My 1 year old son was diagnosed with this condition when he was 9 months old. He is now almost 1 ½ and although the doctor said he should grow out if it fairly quickly I just feel like that is not the case. He gets neupogen shots once a week but his ANC counts continue to be all over the map, never really getting over 500 or .5. They say the normal count should be around 1500 or 1.5. They are now talking about giving him the shot twice a week. I feel like he is already missing out on great experiences because we have to keep him away from other kids. Anyways, if anyone ever needs to talk my email is kt.edgar@yahoo.com.
Good luck everyone, my thoughts and prayers are with you all.

Mar 14, 2009 2:01 PM

Guest :

sat 14th March 09
Hi my daughter was diagnosed with this horrible condition when she was 7 mths old she is know 2 1/2 years old.We are under the haemotology dept in Newcastle Upon Tyne.She mainly suffers with repeated ear and chest infections but has also required surgery for an abcess and hospital admissions for various other skin conditions etc.I feel isolated unsupported and frustrated as a parent to a child with this condition.I would be pleased to hear from anybody.gilly.angus@sky.com

Mar 15, 2009 10:40 AM

Guest :

My daughter has this also. She is almost 2 yrs old and we found out when she was 7 mon old. She had a boil on her bum and was not able to fight it. We give her weekly GCSF injections to keep her counts up. She is admitted in the hospital on a regular basis. She also had a bone marrow biaspy done when she was 8 mon old and it showed NO signs of cancer - thank god. It is really hard to try and work and have a normal life with this. No play groups, day care ect. But she is worth it. My daughter looks like a normal child on the outside. Her counts when we first found out were usually 0.0 or 0.1 . With the injections she now keeps counts at about 1.8 or sometimes higher. She has also battled with a lot of mouth ulsers. Her mouth gets so sore. I would love to talk to anyone who has a child with this illness as I do not know of anyone else who battles this. I live in Nova Scotia, Canada. My email is rubemandy@hotmail.com . I would love to hear from all of you. It is great support I think. Thank you. I wish everyone all the best.

Mar 28, 2009 9:19 PM

Guest :

I am not alone! It is heartbreaking but also comforting to read your stories. My 19 month old daughter has just been diagnosed with this illness and I am just doing my research to try and understand it. From her first two weeks of life she has been sick, battling skin infections and ear infections, throat infections, pneaumonia and just the constant state of unwell. Being the fourth child I thought it was just bad luck until she started loosing weight and became more and more irritable, unable to settle at night and have an uninterrupted sleep. Blood test after blood test showed anemia and infections. We have now had her on prophylactic antibiotics for 6 weeks and she has gained a little weight and had 3 uninterrupted nights!! She has still been unwell for the 6 weeks needing full courses of antibiotics inbetween. Luckily we have not needed the injections yet and my heart goes out to all those children who do need this. It was a relief to hear that there is a reason why my baby is so unwell and now I can adjust our lifestyle to accomodate this. Like so many of you out there are already doing, I will have to rethink playdays and afterschool group activities in a bid to give her a fighting chance. It is a heartbreaking illness and it just isn't fair, but we are so lucky in that there are things that can be done to help our children live with this. There are so many worse things that our children could be diagnosed with and I thank my lucky stars every day that this is all she has. It is hard and isolating, don't get me wrong, I understand , but I am a glass half full kind of girl! We can deal with this and having contact with others going through the same thing helps a huge amount!

Mar 28, 2009 9:34 PM

Guest :

I am not alone! It is heartbreaking but also comforting to read your stories. My 19 month old daughter has just been diagnosed with this illness and I am just doing my research to try and understand it. From her first two weeks of life she has been sick, battling skin infections and ear infections, throat infections, pneaumonia and just the constant state of unwell. Being the fourth child I thought it was just bad luck until she started loosing weight and became more and more irritable, unable to settle at night and have an uninterrupted sleep. Blood test after blood test showed anemia and infections. We have now had her on prophylactic antibiotics for 6 weeks and she has gained a little weight and had 3 uninterrupted nights!! She has still been unwell for the 6 weeks needing full courses of antibiotics inbetween. Luckily we have not needed the injections yet and my heart goes out to all those children who do need this. It was a relief to hear that there is a reason why my baby is so unwell and now I can adjust our lifestyle to accomodate this. Like so many of you out there are already doing, I will have to rethink playdays and afterschool group activities in a bid to give her a fighting chance. It is a heartbreaking illness and it just isn't fair, but we are so lucky in that there are things that can be done to help our children live with this. There are so many worse things that our children could be diagnosed with and I thank my lucky stars every day that this is all she has. It is hard and isolating, don't get me wrong, I understand , but I am a glass half full kind of girl! We can deal with this and having contact with others going through the same thing helps a huge amount!

Apr 29, 2009 6:24 PM

Guest :

I just wanted to let you know there is hope for your child to grow out of this horrible condition. My daughter was diagnosed at 6 weeks and by age 3 she was released from her hematologist. It was a day that I never thought would every come. It was a rough ride for me because my husband is in the military and I was doing it all alone while he was fighting in the war, but you just have to pray and be strong for your child and you will get through it. My daughter is 6 years old now and you would never believe that she was ever affected, she is very healthy thanks to a wonderful doctor and nursing staff.

Currently I am battling another immune deficiency with my son who is 16 months old and is very similar to AIN but they are calling his IgG subclass deficiency or Common Variable Immune Deficiency. My son has not had a week without an ear infection at 10 months they placed ear tubes and is still infected. He was placed in the hospital in March for a week because of an infection in his lymph nodes and an abcess in his neck which spread to his blood. It was horrible and I know you all have been through the same typeof situations. My prayers are with you and your families. If you have any questions or just need someone to talk to about the condition I would love to hear from you. V/r Courtney
marksgirls@gmail.com

May 13, 2009 5:59 AM

Guest :

I too have Autoimmune Neutropenia. I am now 34 years old. I was only diagnosed with it in 2001. I was told that it wasn't hereditory and would not pass onto my children, which was true in my first son. he is now 6 and fine. My second son however is now 3 and has been diagnosed with the same disorder.
I suffered all my life - with tonsillitis (then had them removed when i was 8), then absecces and boils, and very severe mouth ulcers and gum disease. i have had 2 near fatal infections when i was under 10. But having said that my parents didn't know what i had then so didn't realise how seriously ill i was.
I find it very hard now to watch my son suffering when i now exactly how much it hurts him, its like suffering with it twice over. Now i'm older my infections are a lot less frequent and less severe. But i do now make sure i eat well, take vitamins and also get enough sleep. this last one is hard when my son can't sleep because he is so ill.

I understand all of your problems and worries.
If you would like to get in touch I would love to hear from anyone in similar circumstances. my email is linda.crook@gmail.com

Jul 3, 2009 10:48 AM

Guest :

My daughter was diagnosed with AIN several months ago and we are still reeling from the diagnosis. She is now 16 mo old and since her diagnosis we have primarily kept her home, only taking her out to go for rides in the car and over to grandma's house. I have 2 older kids ages 6 and 5 so I am finding this difficult to manage with their school and other activities.
Before her diagnosis, she was in and out of the hospital every 3-4 wks with staph infections, respiratory infections, ear infections, you name it. She gets a staph infection any time her skin is pierced (e.g. bug bite or scrape), she gets ear infections everytime she gets congested, etc. Thank God we now know what is going on with her little body.
Her neutrophil count has yet to exceed 160 total (usually she's at 0 but she's gone to 2% of total white blood count of around 8,000).
I would love to connect with other families in a similar situation. I am curious how you all are adjusting your lifestyle and what you are and are not doing with your little ones. I'm also curious about the GCSF and neutropen injections as our hemotologist did not recommend any such treatment and basically told us we have to wait for her to outgrow this.
God Bless-
lauriebagley@qwest.net

Jul 24, 2009 12:21 AM

Guest :

My son is 11 months old and had blood work done at 1o months to check for an iron deficiency. He was fine there but I was told he might have an infection and to come back in a month for more blood work. I did this yesterday 7/22 and I am not told he has a "low neutrophil count" but the Dr said he wasn't worried and told us to come back in a month. Well, now we go back in two weeks because I refuse to wait that long. Also, my son doesn't go to daycare and has little contact with other children so he has only been sick once and never ran a fever. I hope and pray that this isn't his diagnosis but I don't know what else it could be. Please contact me if anyone has any advice vitavetagrl@hotmail.com

Jul 27, 2009 11:47 AM

Elaine Moore :

Hi,
Your son may just have a low white count, which isn't that unusual in kids. A low white count can occur in viral infections and it can be caused by medications. What you're describing doesn't sound like autoimmune neutropenia. Best to you, Elaine

Jul 30, 2009 8:17 AM

Guest :

Hello,
Our life has changed totally in the last few days. We had a baby boy on July 25th 2009 and he was born early (by 6 weeks, 34 weeks, 1 day). The Docs informed us everything is normal and being pre-term, he has no problem. The very next day we were told that the baby's White Blood Cells (WBC), dropped to 5.5, which was 8.5 the previous day. The WBC is not the concern, the Actual Neutrophill Count (ANC) dropped to 0 the next day. Initially, they thought it could be an infection and they put him on anitbiotics. The numbers came up, and yesterday the 28th the ANC came up to 1300 and today 29th it went back to 0.
The Doctors are totally confused. They think it could be Severn Congenital Neutropenia (SCN) OR Auto Immune Neturopenia OR Cyclic Netropenia OR just and Infection. They are confused because if its SCN they think the ANC will not increase so high and if it's Cyclic, the numbers will not fall the very next day. One day its up, the next day it's down. They are going to watch his response to anitbiotics for another fews days. We were told that we will not know the test results for another couple of weeks. He is in NICU and may have to be transferred to isolated room quite soon.
Has anyone experienced this kind of behavior with newborns? Any response would really help us. We are ready to face the truth. We cannot wait for few weeks to know the results.
Does my 4 days old son have Neutropenia? OR is this just an Injection?
Thanks a lot.
Dina

Jul 30, 2009 9:13 AM

Elaine Moore :

Hello Dina,

Based on your baby's absolute neutrophil count, he has a condition neutropenia. Other tests are needed, such as antibodies to granulocytes (neutrophils), to see if the neutropenia is a primary condition or a consequence of infection or some other condition.
The important thing is that your baby's neutropenia has been diagnosed and he's receiving treatment. Often, children with neutropenia outgrow it after 13 months or so. Best to you and your family, Elaine

Aug 18, 2009 8:49 PM

Guest :

My son is showing symptoms. His diagnosis will be complete in a couple weeks. Does anybody have good news? Maybe a recovery someday? What can I do to help? pedersen124@hotmail.com

Sep 3, 2009 4:19 PM

Guest :

I thought that it was just me but when I came across this article and saw all the others involved with this disease I felt a little better. My 16 year old son was diagnosed in May 2009 only he also has ITP which is basically killing all of his wbc, neutrapils, and platelets. No one can really understand this disease unless they have a child with it and I thank you all for sharing your stories with me. I now know that I actually have it easy compared to many of these stories. God Bless all of you and your children.

Sep 29, 2009 12:05 PM

Dina :

Hello,
My son was born with very low ANC. It was 0 the second day. We did all tests and following are the results:
ELA2 - Negative - Test done at GeneDx (Done only once)
HXA1 - Negative - Test done at GeneDx (Done only once)
Antibodies in Mom's Blood - Positive - Test done at American Red Cross (Done only once)
Antibodies in baby's blood - Negative - Test done at American Red Cross (Done only once)
Based on the above results his neutorpenia was confirmed as Neonatal Alloimmune. And the doctors gave him GCSF, one dose (on 8/2/09). The next day on, his ANC peaked to 9000 and dropped slowly and reach 500 on Monday 9/28/09.
From 8/2 to 9/28 we checked his ANC every week and it kept dropping and it was around 1200 for the last few weeks.
Now the doctors are not quite sure if it's Alloimmune and advising we go for a Bone Marrow Aspiration before another GCSF dose.
Have you seen any similar case? Has anyone shown good response to GCSF and had a good ANC for 7-8 weeks before it dropped with one dose of GSCF?
Is Bone Marrow necessary? My baby is just 2 months old and not sure of the side effects of sedation and rest.
Any input will be greatly appreciated.
Thanks,
Dina

Sep 29, 2009 12:16 PM

Elaine Moore :

Hi Dina,
I'm a clinical laboratory scientist and not a physician so I don't think I can help you except that I can say that bone marrow aspirations are often needed to confirm hematological disorders and the laboratory often runs them on children. In the long run, this can help with diagnosis and early treatment.
Best to you and your family, Elaine

Oct 15, 2009 12:14 PM

Guest :

My DD was diagnosed with neutropenia at 9 month, and has been going to doctors every two weeks for last four months to see what her real cause is. Is there some sort of support forum for this? If there's any email distribution list, i'd like to get in as well... It's really helpful to hearing that my child's not the only one. my email is mikani9@gmail.com. Free to email me also!.

Dec 1, 2009 9:06 AM

Elaine Moore :

Hi,

Typically, autoimmune neutropenia resolves within a few years and doesn't return. Neutropenia can occur because of other causes such as reactions to medications but children who had it don't usually have relapses. Best, Elaine

Feb 3, 2010 5:55 PM

jessica benjamin :

Hi there my daughter is 12 months old and was diagnosed with severe neutropenia... the way it all started was back in november 2009 she got the h1n1 shot and the very next day she got a lympnode under her right cheek jaw bone... we rushed at the hospital and they gave her antibiotics for 7 days i didnt do anythig so they then gave her 3 days of iv antibiotics didnt work but the last antibiotics that were prescribed worked but once the infection disapeared and they did the blood tests it showed 0.4 in the neutropils i asked doctors up to today if its the medicatin that could have caused it and they say no.. but its been now 4 months they did a bone marrow aspiration and everything is fine they could not do the bonemarow biopsy bcause she was bleeding too much thy say.. will perform it in about 6 months... the thing is they havent prescribed or giving me anything, is there anything i can give her to make them go up? plus i am a working mother with a 6 year old in school and my daughter is in daycare with 5 other kids... should i remove her from daycare? but then again my son goes to school and he can get sick... awww i just dont know what to do...

Feb 3, 2010 6:08 PM

Elaine Moore :

Hi,
Regarding your 12 month old, I'd consider reporting this to VAERS because that's the only way they get statistics on side effects from the vaccine. There's no way to definitively prove it, but I'd suspect that her neutropenia developed as a consequence of the vaccine. The dose for newborns may be too high and I've seen this suspicion mentioned before.
Look into Transfer Factor (Colostrum) and check with your doctor or a naturopath and see if this might help. Bone marrow aspirations are very difficult to do on small children. I wouldn't follow up with one unless it's absolutely necessary. Best, Elaine

Feb 3, 2010 8:04 PM

jessica benjamin :

Hi there my daughter is 12 months old and was diagnosed with severe neutropenia... the way it all started was back in november 2009 she got the h1n1 shot and the very next day she got a lympnode under her right cheek jaw bone... we rushed at the hospital and they gave her antibiotics for 7 days i didnt do anythig so they then gave her 3 days of iv antibiotics didnt work but the last antibiotics that were prescribed worked but once the infection disapeared and they did the blood tests it showed 0.4 in the neutropils i asked doctors up to today if its the medicatin that could have caused it and they say no.. but its been now 4 months they did a bone marrow aspiration and everything is fine they could not do the bonemarow biopsy bcause she was bleeding too much thy say.. will perform it in about 6 months... the thing is they havent prescribed or giving me anything, is there anything i can give her to make them go up? plus i am a working mother with a 6 year old in school and my daughter is in daycare with 5 other kids... should i remove her from daycare? but then again my son goes to school and he can get sick... awww i just dont know what to do...

Feb 3, 2010 11:28 PM

jessica benjamin :

Hi there my daughter is 12 months old and was diagnosed with severe neutropenia... the way it all started was back in november 2009 she got the h1n1 shot and the very next day she got a lympnode under her right cheek jaw bone... we rushed at the hospital and they gave her antibiotics for 7 days i didnt do anythig so they then gave her 3 days of iv antibiotics didnt work but the last antibiotics that were prescribed worked but once the infection disapeared and they did the blood tests it showed 0.4 in the neutropils i asked doctors up to today if its the medicatin that could have caused it and they say no.. but its been now 4 months they did a bone marrow aspiration and everything is fine they could not do the bonemarow biopsy bcause she was bleeding too much thy say.. will perform it in about 6 months... the thing is they havent prescribed or giving me anything, is there anything i can give her to make them go up? plus i am a working mother with a 6 year old in school and my daughter is in daycare with 5 other kids... should i remove her from daycare? but then again my son goes to school and he can get sick... awww i just dont know what to do...

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