[PAGID] Request for advice - ? patient with CVID

[Routes, John]

Sorry for the late reply
Agree with Mel¹s points
This patient almost certainly has multisystemic granulomatous disease with
lung, liver and possible GI tract involvement (either primary due to GI
tract granulomas or secondary to severe portal hypertension)‹in my
experience, steroids are usually ineffective in treating this disease (did
the patient have improvement in LIP by PFTs/CT?)---
-I am particularly worried that the patient has severe granulomatous liver
disease and portal hypertension-----A liver bx and depending on the result a
measurement of portal vascular pressures are needed----need info on
prognostic labs for advanced liver disease (Na, albumin, PT etc)
Would be happy to discuss the case in detail by phone
Jack


John M. Routes, MD
Chief, Section of Allergy and Clinical Immunology
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Department of Pediatrics
Children's Hospital of Wisconsin
Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874
 
Phone: 414-456-4802; 456-4803
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu
 




From: "Berger, Melvin" <Melvin.Berger at UHhospitals.org>
Reply-To: <pagid at list.clinimmsoc.org>
Date: Thu, 11 Sep 2008 23:44:51 -0500
To: <pagid at list.clinimmsoc.org>
Conversation: Request for advice - ? patient with CVID
Subject: RE: [PAGID] Request for advice - ? patient with CVID

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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