Humoral Immune Deficiency

by Brian Alverson, MD

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    00:01 In this lecture, I'm going to review the basic humoral immune disorders in children.

    00:08 Basically we're talking about disorders where children can't mount a humoral or blood response to these organisms.

    00:17 These patients are more prone to encapsulated organisms.

    00:20 Examples include Strep pneumoniae, H. flu (generally non typeable) and Neisseria meningitidis.

    00:28 So, these patients will typically present with sinopulmonary infections.

    00:33 Different humoral immune deficiencies present at different ages and in different ways.

    00:39 Let's review them by age group.

    00:42 In children who are 0-6 months , these children will generally present if they have a severe humoral deficiency.

    00:48 Examples of this would be X-linked agammaglobulinemia or Hyper IgM syndrome which is quite rare.

    00:56 Between 2 and 6 years of age, children will often present if they have IgA deficiency, a selective IgG deficiency, hypogammaglobulinemia, or hyper IgE syndrome or Job syndrome.

    01:11 There's some variability here, for example, some patients with hypogammaglobulinemia will not show up too much later in life even maybe when they're 18.

    01:20 But this is generally when they present.

    01:22 Flip side, patients will present much later if they have common variable immune deficiency It will be very unusual to present before the age of 6.

    01:32 The average age is in the 20s.

    01:34 Or they may have acquired disease.

    01:37 An acquired example of a humoral immune deficiency would be HIV or lupus.

    01:42 So, let's start with Job syndrome.

    01:45 Patients with Job syndrome have very high levels of IgE, in the thousands.

    01:51 This patients will typically present with coarse facies.

    01:56 Although it's hard to pick it up in a child, based on their appearance alone.

    02:00 Coarse facies means large ears, large nose, large chin, large eyes, generally large sub units on the face.

    02:09 But coarse facies is not typically how we make the diagnosis.

    02:13 We usually make it because of their skin and their infections.

    02:17 So these patients will have recurrent sinopulmonary infections.

    02:21 By sinopulmonary, I mean sinosinus and pneumonia or otitis media.

    02:26 They can also develop eczema and will have significant eczema, and they can develop recurrent cellulitis of the skin.

    02:34 This is classic for Job syndrome.

    02:37 Treatment for these patients is supportive.

    02:40 We provide them antibiotics for their bacterial infections.

    02:44 And we generally, need to to support them because this is a very challenging disease to live with.

    02:49 That's where the biblical name of Job comes from.

    02:53 These patients feel like life is consistently a trial.

    02:56 So, we have to support them.

    02:58 The next is IgA deficiency.

    03:02 The vast majority of patients with IgA deficiency have no symptoms.

    03:09 But a smaller percentage will present in 4 different possible ways.

    03:15 One, they may present with recurrent sinopulmonary infections.

    03:20 We treat them for each of their own infections and you might consider giving IVIG to supplement the effect of that treatment.

    03:29 Patients may present with GI disorders such as celiac or inflammatory bowel disease.

    03:34 In this case, we'll treat the disease and again consider IVIG.

    03:38 They may present with autoimmune disease, such as lupus or JIA.

    03:43 Again, treat disease, consider IVIG.

    03:46 And lastly, they may present uniquely with anaphylaxis to transfusion of blood or IVIG which we were considering to give them.

    03:55 These patients may require desensitization to blood products, which is a complicated procedure.

    04:03 Let's switch gears now to X-linked agammaglobulinemia or hypogammaglobulinemia.

    04:09 The issue with these patients is they have relatively no or little immunoglobulins in the blood.

    04:17 This is typically in males because it's X-link recessive.

    04:21 And these patients will often present very severely, early in childhood with failure to thrive.

    04:28 Later in childhood or an early adult presentation is typical of hypogammaglobulinemia but not agammaglobulinemia.

    04:38 Typically, these patients will have recurrent otitis media.

    04:42 They will have recurrent sinusitis, recurrent pneumonia and we'll treat them with regular IVIG infusions every 3 weeks.

    04:51 We're going to provide them with the IVIG they need to survive.

    04:56 Let's switch gears one more time to common variable immune deficiency.

    05:00 This is a B-cell deficiency.

    05:03 These patients have a high risk of auto-immune disorders like autoimmune hemolytic anemia or immune thrombocytopenic purpura.

    05:12 They're also at a high risk for malignancies such as lymphoma or gastric carcinoma.

    05:18 And typically these patients present later.

    05:22 The average age of patients is 26 years old, but they may present earlier also.

    05:27 These patients are going to be treated with IVIG.

    05:32 So, we'll treat them and provide them with the IVIG they need to keep them going.

    05:37 So, that's my summary of the humoral immune deficiencies in children.

    05:41 Thanks for your time.

    About the Lecture

    The lecture Humoral Immune Deficiency by Brian Alverson, MD is from the course Pediatric Allergy and Immunology. It contains the following chapters:

    • Humeral Immune Deficiencies
    • Job Syndrome
    • Selective IgA Definciency
    • X-linked Agammaglobulinemia or Hypogammaglobulinemia
    • Common Variable Immune Deficiency

    Included Quiz Questions

    1. Patients are at increased risk for malignancy
    2. These patients present with T-cell deficiency
    3. These patients are treated with steroids
    4. These patients have a low risk of autoimmune disease
    5. These patients typically present in the first 5 years of life
    1. Encapsulated bacteria
    2. Catalase negative bacteria
    3. Catalase positive bacteria
    4. Non-encapsulated bacteria
    5. Adenoviruses
    1. IgA deficiency
    2. Hyper IgM syndrome
    3. HIV
    4. Lupus
    5. X linked Agammaglobinemia
    1. IgE
    2. IgG and IgM
    3. IgG
    4. IgM
    5. IgA

    Author of lecture Humoral Immune Deficiency

     Brian Alverson, MD

    Brian Alverson, MD

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