[PAGID] Request for advice - ? patient with CVID

[Berger, Melvin]

If the patient has had infections with strep. pneumoniae while on IVIg, I suggest you adjust the IVIg dose/interval to be sure she has protective pneumococcal titers at the trough (I learned that fronm Ralph Shapiro). 
 
What is the status of her liver, biliary tree and panreas ? Could she have pancreatic insufficiency ?   If the liver is enlarged and/or the LFts are chronically elevated, I would suggest a biopsy. Do you have any idea what happened when she got the ascites and splenomegaly ?  Does she have waxing and waning granulomatous hepatitis. After reviewing the liver and intestinal histology, including flow cytometry, consider a TNF antagonist (if it is granulomatous) or Rituxin if the intestine has a lymphocytic infiltrate which is CD20+ . 
 
Another possibility is intestinal lymphangiectasia- which might explain the maladsorption, and if she is hypoproteinemic, that may have exacerbated at a point in tie contributing to the ascites. Is her albumin borderline or low ?  Intestinal lymphangiectasia could certainly contribute to her lymphocytopenia.  
 
Melvin Berger, M.D., Ph.D.
Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237
 
Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Abraham, Roshini S., Ph.D.
Sent: Thu 9/11/2008 2:34 PM
To: pagid at list.clinimmsoc.org
Cc: Rank, Matthew A., M.D.
Subject: [PAGID] Request for advice - ? patient with CVID



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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