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

[CIS-PIDD]

Hi Richard,

I prefer low dose nifedipine over amlodipine.  Also consider referral to rheumatology to help differnetiate between primary and secondary Raynaud's, they can run some very comprehensive panels now.

Best and Happy New Year,

John

John B. Meiser, M.D., P.A. 
Allergy and Asthma Center of Texas
6946 Lebanon Rd.
Frisco, TX 75034
www.aactmd.com
office:  972-377-9987
fax:  972-377-9906

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>  -------Original Message-------
>  From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
>  To: CIS-PIDD <cis-pidd at lyris.dundee.net>
>  Subject: VS: [cis-pidd] Raynaud's in XLA
>  Sent: 03 Jan '18 00:27
>  
>  
>  
>  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
>  
>  +358 9 47180201| +358 50 4279606
>  faxi +358 9 47174703
>  
>  www.hus.fi; www.harvinaissairaudet.fi
>  
>  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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