[CIS PIDD] [cis-pidd] Baby with intestingal lymphangiectasia

Wed Jul 22 17:26:43 EDT 2015

Dear Dr. Church,
Protein malnutrition can be an additional aggravating factor in her case. In one of my adult patients with intestinal lymphangiectasia we tried IgG supplementation as SC push every other day and could get his IgG up from under 200 into 400's. Then he started octreotide that substantially improved his condition, and reduced his GI protein loss and abdominal symptoms. His immunoglobulins went up into 500's, but he experienced adverse effects that failed to improve with reduction of dose and frequency, and he had to stop IgSC. Then his IgG stabilized close to 400 without exogenous IgG. 
Have you exhausted nutritional interventions to minimize her protein loss and improve her protein assimilation?
Another potential approach might be sirolimus that has shown promise in lymphangiomatosis, and might similarly lead to obliteration of abnormal lymphatic vessels in her gut and limb at lower doses that could precipitate immunosuppression in a patient who is at much higher risk. Still, the balance may be in her favor, if all other options are exhausted.
Best regards,
Soheil Chegini, M.D.Exton Allergy & Asthma Associates656 West Lincoln Hwy.Exton, PA 19341Phone: (610) 269-3066Fax: (610) 269-8615
      From: "Church, Joseph" <JChurch at chla.usc.edu>
 To: CIS-PIDD <cis-pidd at lyris.dundee.net> 
 Sent: Wednesday, July 22, 2015 3:31 PM
 Subject: RE: [cis-pidd] Baby with intestingal lymphangiectasia
   
 <!--#yiv9969829406 _filtered #yiv9969829406 {font-family:Calibri;panose-1:2 15 5 2 2 2 4 3 2 4;} _filtered #yiv9969829406 {font-family:Tahoma;panose-1:2 11 6 4 3 5 4 4 2 4;}#yiv9969829406 #yiv9969829406 p.yiv9969829406MsoNormal, #yiv9969829406 li.yiv9969829406MsoNormal, #yiv9969829406 div.yiv9969829406MsoNormal {margin:0in;margin-bottom:.0001pt;font-size:12.0pt;font-family:"Times New Roman", "serif";}#yiv9969829406 a:link, #yiv9969829406 span.yiv9969829406MsoHyperlink {color:blue;text-decoration:underline;}#yiv9969829406 a:visited, #yiv9969829406 span.yiv9969829406MsoHyperlinkFollowed {color:purple;text-decoration:underline;}#yiv9969829406 p {margin-right:0in;margin-left:0in;font-size:12.0pt;font-family:"Times New Roman", "serif";}#yiv9969829406 p.yiv9969829406MsoAcetate, #yiv9969829406 li.yiv9969829406MsoAcetate, #yiv9969829406 div.yiv9969829406MsoAcetate {margin:0in;margin-bottom:.0001pt;font-size:8.0pt;font-family:"Tahoma", "sans-serif";}#yiv9969829406 span.yiv9969829406BalloonTextChar {font-family:"Tahoma", "sans-serif";}#yiv9969829406 span.yiv9969829406EmailStyle20 {font-family:"Arial", "sans-serif";color:#1F497D;}#yiv9969829406 .yiv9969829406MsoChpDefault {font-family:"Calibri", "sans-serif";} _filtered #yiv9969829406 {margin:1.0in 1.0in 1.0in 1.0in;}#yiv9969829406 div.yiv9969829406WordSection1 {}-->Thanks, All.     IF I were to try Ig it would be via SC route.  But should I even try in a patient with no infections?    Richard:  I think the reason this kid has no IgA is because she is losing so much Ig via her gut.  An IgG of 83 is pretty darn low for a protein losing process.  We see a fair number of PLEs secondary to Fontan heart procedures, and they usually aren’t this low.    Our lab does not normalize mitogen preps for lymphocyte counts.  So, I’m not too worried about her T-cells.    Regarding ticks vs fleas vs both, we will continue to watch her carefully.    JC    From: Richard Wasserman [mailto:drrichwasserman at gmail.com]
Sent: Wednesday, July 22, 2015 12:16 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Baby with intestingal lymphangiectasia    Joe, Why is there no IgA? That concerns me a little. Most of the PLE's do pretty well without supplementation. Does your lab normalize mitogen result for lymphocyte count? I'd hate to give her two problems but while most dogs have ticks OR fleas some have ticks AND fleas. Richard Wasserman Dallas    On Wed, Jul 22, 2015 at 1:28 PM, Nacho Gonzalez <nachgonzalez at gmail.com> wrote: Dear Dr. Church,    the last 2 XLA patients I have seen recently were under the first year of life. They had poor vascular access... every IVIG infusion was a pain. Now they are under SCIG and doing fine. But going back to your patient, I do not think she needs IgG replacement therapy if she is free of infections.    Best regards,    Luis Ignacio Gonzalez-Granado Immunodeficiencies Unit Pediatric Hematology & Oncology Unit Hospital 12 octubre Madrid. Spain    2015-07-22 20:19 GMT+02:00 Verbsky, James <jverbsky at mcw.edu>: 
Not sure if this will work as well in teliangiectasia but in other PLE states subQ has been beneficial…might be tough in an 18mo..but it’s a thought   james   From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Wednesday, July 22, 2015 1:08 PM
To: CIS-PIDD
Subject: [cis-pidd] Baby with intestingal lymphangiectasia   Colleagues:   I have consulted on an 18 month-old girl with intestinal lymphangiectasia and unilateral lymphedema.  She has experiencedno infectious complications.   However, labs demonstrate             Serum albumin 2.3 g/dL             IgG      83 mg/dL             IgA      <7 mg/dL             IgM      27 mg/dL                    Tetanus antibody 0.49 IU/mL (considered protective)             Haemophilus influenzae antibody 0.48 mcg/mL (considered unprotective)             Pneumococcal antibody response to Prevnar 13 positive (>1.3 mcg/ml) for 10 of 12 serotypes tested.             CD3+ 46% (1399/mcL)             CD4+ 14% (328/mcL)             CD8+ 46% (1113/mcL)             CD19+ 24% (590/mcL)             NK 16% (391/mcL)             PHA and PWM responses decreased (~ 25% of concurrent control)   The child is clearly losing protein, immunoglobulins and T-cells (CD4+ > CD8+), but has fair circulating antibody responses to tetanus toxoid and conjugated pneumococcal vaccine.   I would appreciate your opinions regarding immunoglobulin replacement therapy.   Joe Church, MD Children’s Hospital Los Angeles     
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