[PAGID] Request for advice - ? patient with CVID

[Abraham, Roshini S., Ph.D.]

Dear PAGID colleagues,

 

We would appreciate your comments regarding the following patient who
was recently seen in our institution:-

 

  A 44 year-old female presenting with chronic lung disease and
malabsorption for a second opinion.  As a child, she had recurrent
otitis media (never requiring surgery), frequent viral upper respiratory
infections, and recurrent tonsillitis.  In 1991 she developed shortness
of breath and had infiltrates on her chest x-ray and ultimately was
diagnosed with lymphocytic interstitial pneumonitis based on open lung
biopsy. She also at the time was noted to have hypogammaglobulinemia and
was started both on prednisone and on IVIG and has been on both since.
She has attempted to wean from her prednisone, although has recurrent
lung symptoms with decreasing doses. She also throughout this time has
had recurrent sinus infections including three sinus surgeries and
recurrent lung infections in which she develops sputum that almost
nearly always grows Streptococcus pneumoniae. She has also had recurrent
yeast vaginitis.  She is currently treated with IV antibiotics on a
rotating basis as there has been growing concern by her local physicians
that she was not absorbing her oral medications.    

    In April 2006 she developed both ascites and pleural effusions and
had this fluid removed. She developed massive splenomegaly to the point
that it was interfering with her breathing and ultimately had a
splenectomy for this reason. She continued to have trouble with diarrhea
and malabsorption and currently has a diagnosis of osteoporosis thought
to be related to vitamin D malabsorption and chronic prednisone use.
She has also had Giardia on several occasions.  She currently has a
permanent intravenous access and had a brief course of TPN earlier this
year and gets IV antibiotics as well as her IVIG through it.  

    Current laboratory tests include IgA <1, IgM <5, IgG 760 (last
infusion 2 weeks ago), IgE <2.  Total leukocytes 13,500 (95% PMN, 2%
Lymph, 2% Mono, 1% Eos).  Hemoglobin 12.1 g/dl and Platelet count
257,000.  T and B cell surface marker quantification:  %CD19 was
undetectable, CD4 79/ul, CD8 51/ul.  One previous lab result from an
outside lab 2 years ago also demonstrated the absence of CD19 cells.
The patient had previous bone marrow and intestinal biopsies and we are
in the process of obtaining these records.  BTK flow assay on monocytes
showed normal protein expression.  

 

The malabsorption has been the most troubling condition for this patient
and our Gastroenterology colleagues are planning to target any microbial
overgrowth or parasite infection but are also considering additional
anti-inflammatory treatment.  I am also requesting that the local
physicians attempt to keep the trough IgG levels >800.

 

Any assistance/suggestions for diagnosis and/or treatment would be
welcome. 

 

Matthew A. Rank, MD
Roshini S. Abraham

Mayo Clinic, Rochester

 

 

 

Roshini Sarah Abraham, Ph.D., D(ABMLI)
Director 
Cellular and Molecular Immunology Laboratory 
Department of Laboratory Medicine and Pathology 
Hilton 210 e 
Mayo Clinic 
200 1st St SW 
Rochester, MN-55905 
Ph: 507-266-9292 
Ph (Secy): 507-284-4055 
Fax: 507-266-4088 

 

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