[CIS-PAGID] Severe hypogammaglobulinemia from lymphangectasias

[Verbsky, James]

Sub q is a good option particularly since she is ill

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On Jan 20, 2012, at 10:15 AM, "Iyengar, Rajashri S., M.D." <RIYENGAR at PARTNERS.ORG<mailto:RIYENGAR at PARTNERS.ORG>> wrote:

Hi,

I have a 2 year old patient with tuberous sclerosis, seizure disorder, and lymphangectasias who currently is in respiratory failure from an RSV infection. She is followed for her secondary hypogammaglobulinemia and initially presented with an IgG of 50 eight months ago. Her immunoglobulin function (vaccine titers to tetanus, Hib, and pneumococccus) was normal, and she has been on IVIG for the past 8 months with increasing IgG levels.She is also on weekly albumin infusions due to her severe protein loss from her lymphangectasias. However, during this recent illness she has been losing protein very rapidly and it has been difficult to maintain IgG levels above 150 despite every 1-2 week IVIG infusions at 500mg/kg. Given her normal immunoglobulin function, would it be advisable to maintain her at lower immunoglobulin levels (closer to 100) given her rapid protein loss? Would subcutaneous IVIG be a better option for her as an inpatient (as well as an outpatient)?

Thanks again,

Shuba Iyengar, MD, MPH
Attending in Pediatric Allergy/Immunology
Massachusetts General Hospital
Division of Pediatric Pulmonary, Allergy/Immunology





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