Hyper IgM syndrome: Recurrent pyogenic infections in absence of neutrophils?

[whatdidigetinto]

Hyper IgM syndrome is characterized by low neutrophils. If this is true how am i getting pyogenic infections? Because Pus = neutrophils. So if no neutrophils, where am i getting the pus from?

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[Omar Little]

Hyper IgM syndrome is characterized by low neutrophils. If this is true how am i getting pyogenic infections? Because Pus = neutrophils. So if no neutrophils, where am i getting the pus from?

Is it really associated with high neutrophils? I thought it was a problem of class switching from IgM to IgG/IgA/IgE because of a problem with CD40/CD40L during B-cell maturation.

Pyogenic infections happen because you don't have good opsomization. See Pathoma page 17

 

[medpack87]

Hyper IgM syndrome is characterized by low neutrophils. If this is true how am i getting pyogenic infections? Because Pus = neutrophils. So if no neutrophils, where am i getting the pus from?

Hyper IgM syndrome occurs because of defective class switching. There is no neutrophil deficiency here.

Maybe you are confusing with Hyper IgE syndrome, also called Job's disease. In this one, you have coarse facial features, and also abscesses, caused by a deficiency in chemotaxis. So neutrophils cannot come into the area where the infection is occurring. But other cellular debris, and other WBC's are going to be around producing the pus, you just can't fight them appropriately.

 

[whatdidigetinto]

Firecracker and the internet disagree with you. There is a neutrophil deficiency in hyper IgM.

 

[medpack87]

Firecracker and the internet disagree with you. There is a neutrophil deficiency in hyper IgM.

Oh well. I do not own firecracker. Anyway, the guys at DIT say that we shouldn't over think these inmunodeficiency questions too much, because most of them are currently in research and are very complicated, they might have more characteristics or mechanisms than what we are required to know for the USMLE.

Sorry if i cannot give you a proper answer for why there is abscess formation in hyper IgM, if neutrophil defiency is part of the syndrome... but I would suggest to just go with what FA, Kaplan, DIT or more STEP1 oriented sources say.

 

[whatdidigetinto]

Firecracker is a step 1 oriented source.

 

[sharklasers]

Firecracker and the internet disagree with you. There is a neutrophil deficiency in hyper IgM.

Firecracker is a step 1 oriented source.

Your responses come off as rude for some reason, maybe not the best way to ask for help.

Either way, maybe neutrophils are low because they are being used up?

Who knows, either way, if they test Hyper IgM I'd be willing to bet that decreased neutrophil levels will NOT be what they are going for.

 

[coolforschool]

Your responses come off as rude for some reason, maybe not the best way to ask for help.

Either way, maybe neutrophils are low because they are being used up?

Who knows, either way, if they test Hyper IgM I'd be willing to bet that decreased neutrophil levels will NOT be what they are going for.

Yeah, that person seems snarky. I have a few of those in my class. Whatever.

"Neutropenia is a common feature of XHIGM and may result from a defective, stress-induced, CD40-dependent granulopoiesis as myeloid progenitors express CD40 molecules. Autoantibodies to neutrophils are generally absent."

http://emedicine.medscape.com/article/889104-clinical#a0218

 

[SLC]

Either way, maybe neutrophils are low because they are being used up?

Exactly...

What do we know about IgM and complement fixation? That might give you an idea as to why a blood test would/could show neutropenia in hyper IgM...

 

[whatdidigetinto]

How am I the rude one? You didn't answer my question the first time, suggested I use different sources and told me not to care about the level of detail. How is that helpful at all?

 

[carddoc33]

How am I the rude one? You didn't answer my question the first time, suggested I use different sources and told me not to care about the level of detail. How is that helpful at all?

You seem pleasant.

I'm guessing there's neutropenia, because neutrophils are the only defense you have. Increased pus=increased neutrophils in pus=decreased neutrophils in blood.

 

[Jamiu22]

How am I the rude one? You didn't answer my question the first time, suggested I use different sources and told me not to care about the level of detail. How is that helpful at all?

just let it go bruh!!

 

[coolforschool]

Lol. Did no one read my post, or was that not good enough? This thread should be dead.

How am I the rude one? You didn't answer my question the first time, suggested I use different sources and told me not to care about the level of detail. How is that helpful at all?

It's not what you say but how you say it, my man.

just let it go bruh!!

Haha.

 

[medpack87]

Yeah, that person seems snarky. I have a few of those in my class. Whatever.

"Neutropenia is a common feature of XHIGM and may result from a defective, stress-induced, CD40-dependent granulopoiesis as myeloid progenitors express CD40 molecules. Autoantibodies to neutrophils are generally absent."

http://emedicine.medscape.com/article/889104-clinical#a0218

This one is the best answer for your question.