[CIS PIDD] [cis-pidd] Raynaud's in XLA

[CIS-PIDD]

Hi Richard,

have you checked for LGL leukemia? Gulbu, Kate (CHOP) and Steve & various other colleagues in NIH have described a series of XLA patients with LGL-L/ LGL lymphoproliferation in XLA during follow up (I think still only as a poster). Appears around that age…

No other suggestive autoimmune features? Cytopenias (RBC/PLT/PMN/Ly)? Blood smear/ T cell clonality?

Mikko



Mikko Seppänen
Director, Adjunct Professor, MD, PhD
Hospital District of Helsinki and Uusimaa, Children’s Hospital, Rare Disease Center
P.O.Box 280, 00029 HUS |Lastenlinnantie 2, Helsinki
Senior Consultant
Hospital District of Helsinki and Uusimaa, Inflammation Center, Adult Primary Immunodeficiency Outpatient Clinic
mikko.seppanen at hus.fi<mailto:mikko.seppanen at hus.fi>
+358 9 47180201| +358 50 4279606
faxi +358 9 47174703
www.hus.fi<http://www.hus.fi/>; www.harvinaissairaudet.fi<http://www.harvinaissairaudet.fi/>

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Lähettäjä: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] Puolesta CIS-PIDD
Lähetetty: 2. tammikuuta 2018 23:02
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net>
Aihe: [cis-pidd] Raynaud's in XLA

I have followed a 39 yo XLA for 34 years. Other than sinusitis twice a year he has had no problems. He now has Raynaud's of the hands for the past 2-3 years that is worsening.

Is the generally recommended treatment, calcium channel blocker, ideal in this setting?
Thanks,
Richard

--
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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