[CIS-PAGID] Pulmonary lymphangiomatosis

[Verbsky, James]

To all

Does anyone have any good thoughts on what to do with a 14yo girl with pulmonary lymphoangiomatosis.  She present about 3 weeks ago with large pleural/pericardial effusions.  W/U for autoimmune disease, malignancy, infections (TB) negative.  Recently had lymphangiography which showed abnormal lymphatics in the lungs as well as in the groin (although she has no other swelling, edema,etc).  Has been putting out between 3-4L of lymph per day through her chest tubes.  She has PLE with low albumin, low coag factors, and low IgG.  On prophylaxis since is very lymphopenic.  They started IVIG but unlikely to catch up with this (could try sub-q if an issue)

So far has had pericardial windows placed, and did a thoracic duct ligation without improvement.  Somatistatin tried with no effect.

My thoughts on what I have found in the literature is as follows..please chime in with other thoughts


1)      Sclerotherapy appears helpful

2)      Radiation

3)      Interferon-a

4)      NEJM has a case that responded to propranolol

5)      CSA, steroids have been tried with questionable success

6)      Other chemo??

Thanks for any suggestions

James


James W. Verbsky MD/PhD
Associate Professor of Pediatrics and Medical Microbiology and Genetics
Medical College of Wisconsin
Milwaukee, WI  53211
jverbsky at mcw.edu<mailto:jverbsky at mcw.edu>
414-266-6701 (phone)
414-266-6695 (fax)



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