[PAGID] Request for advice - ? patient with CVID

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

Thanks, Dr. Berger for your comments.  She does not have any evidence of
granulomatous hepatitis and she also does not have any B cells
anywhere...see additional information below.

 

Here is an update:

 

Some outside medical records showed the following:

 

Spleen pathology - no B cell by flow, no GCs

Full thickness small intestinal biopsy - "moderate intraepithelial
lymphocytosis and total villous atrophy, compatible with
CVID-associations" per pathologist.  Flow on intestinal cells: Negative
for CD20 and CD138.

 

3 previous BM biopsies done elsewhere:-

 

Most recent - 11/2006 - Flow for CD19, 20, 19/34, 138 and 22 were all
negative, ~ 1% CD19lambda or kappa.

Previous one - 06/2004 - None of the above B cell markers were
detectable.

The first BM in 1999 did not include flow but noted very few plasma
cells by morphology.

 

Matthew Rank

Roshini Abraham

________________________________

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Berger, Melvin
Sent: Thursday, September 11, 2008 11:45 PM
To: pagid at list.clinimmsoc.org
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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