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Q:

1/5/2020
I have a question about specific antibody deficiency. I have a patient who is a 36 year-old female referred for immunodeficiency evaluation by PCP. This year she had a C-section that resulted in an infected wound. She also had three episodes of mastitis, and each episode was treated with antibiotics. Recently she had a left jaw lump, in which she saw ENT and they were unclear on what it was and sent the patient for CT of jaw and started the patient on Augmentin. Jaw lump improved with antibiotics and the patient did not get CT done. She denied history of sinusitis, bronchitis, pneumonia, otitis media or gastrointestinal infection. Her level of IgG, IgM and IgA was normal. Lymphocyte screen showed normal absolute counts of B cell, T cell and NK cell. She had protective titers to Tetanus and Diphtheria. Her Pneumococcal titers 4 weeks after Pneumovax administrated showed 12/23 protective titers (~52% response). Even though her presentation of repeated mastitis is not generally seen in specific antibody deficiency, would the diagnosis of specific antibody deficiency be appropriate in this patient? Would she be an increased risk of sinopulmonary infections in the future due to poor response to Pneumovax? Should she receive Prevnar? I appreciate any guidance.

A:

I do not favor a diagnosis of specific antibody deficiency with these laboratory data and clinical presentation. The interpretation of polysaccharide antibody response is complicated without a clear consensus on the range of normal, the timing of antibody measurement, and the definition of a protective titer. You did not provide the antibody titers prior to immunization and whether she was vaccinated with the conjugated vaccine as a child. I do not think the conjugated pneumococcal vaccine is necessary at this time since she has not experienced sinopulmonary infections and Streptococcus pneumoniae is not a likely pathogen causing the mastitis and wound infection.

One thought might be to verify she is not a carrier for X-linked NAPH oxidase deficiency as soft tissue infections do occur in some women due to variability in the normal neutrophil number secondary to random X chromosome inactivation (lyonisation) [1]. I have also seen individuals with mannose binding lectin deficiency who have frequent soft tissue infections and early complement defects may also be associated with recurrent bacterial infection. Large data sets however do not show a risk with mannose binding lectin deficiency in adult disease (2).

In summary, I do not think your patient is at risk of sinopulmonary infection and do not recommend her receiving the conjugated pneumococcal vaccine. I would consider obtaining a dihydrorhodamine assay to evaluate carrier status of NAPH oxidase deficiency and maybe a CH50 and mannose binding lectin level. I would also be sure she does not have other reasons for soft tissue infection, such as diabetes.

I hope this information is of assistance in your practice.

1. Battersby AC, Cale CM, Goldblatt D, Gennery AR. Clinical manifestations of disease in X-linked carriers of chronic granulomatous disease. J Clin Immunol 2013;33:1276-84.
2. Dahl M, Tybjaerg-Hansen A et al. A population based study of morbidity and mortality in mannose-binding lectin deficiency. J Exp Med 2004;199:1391-99.

Respectfully submitted
Dennis Ledford, MD, FAAAAI

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