[CIS PIDD] [cis-pidd] VS: 74yo F with Lymphopenia

Tue Oct 7 05:12:36 EDT 2014

Dear Joe,

our experience mostly corroborates what Terry expertly tells. We do not use routine empiric antibiotic prophylaxis, since a high proportion of these patients never seem to get true opportunistic infections. If these appear, it is another case.

Recurrent bacterial pneumonias are not common in these patients, COPD and possibly bronchiectasis could be the causes at least partly. If bronchiectasis, instead of IVIg we use routine measures of prophylaxis, like what ATS, ETS recommend. HRCT would also show if there is anything to suggest sarcoidosis. And if bronchiectasis, I would sample her in search of chronic NTM of lungs.

In very late onset CVID, the cellular findings You list below are often much the same, but in Your case the IgG level alone already rules this out. And defining a true S(P)AD at this age group is very problematic (no proper reference values available). If/when IVIg is stopped and if pneumonias recur despite other measures, I might check anti-PnP responses, to see if they are very low indeed/ totally absent. She should of course refrain from smoking, it interferes with TAP1/2 and ciliary function.

I often rule out thymoma in older patients with low B cells, but IgG and B cells are at too high a level that I would do this in her case (but HRCT would do it, if You tell the radiologist in beforehand that You want to rule that out as well). Indolent peripheral T/NK lymphoma I guess remains a possibility (?), but before clear symptoms is often impossible to rule out. Rashes?

We did perform one WES in a bit similar case already in his early 60's (but previous lymphopenia as long as lab records could tell), we could find nothing there either.

Yours,

Mikko

Mikko Seppänen, MD, Finland
________________________________
Lähettäjä: Church, Joseph [JChurch at chla.usc.edu]
Lähetetty: 6. lokakuuta 2014 22:20
Vastaanottaja: CIS-PIDD
Aihe: RE:[cis-pidd] 74yo F with Lymphopenia

Thank you, Terry.  I appreciate the information.  JC

From: Harville, Terry O [mailto:HarvilleTerryO at uams.edu]
Sent: Monday, October 06, 2014 12:06 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] 74yo F with Lymphopenia

Joe,

Performing evaluations on a large number of patients, we see this profile quite commonly.  Some patients are ill with chronic/recurrent infections (primarily respiratory tract), some with primarily autoimmune features, and others not ill (but, with lab obtained due to the lymphopenia).

The profile shows decreased T and B lymphocytes, but preservation of NK cells.  Most typically, the T lymphocytes will primarily express CD45RO, but very little CD45RA.  The pattern may be consistent with decreased thymopoiesis.  This may be exacerbated by the use of corticosteroids and other medications.  In some, chest irradiation may be complicating the thymic function.  In many of these patients, cardiac surgery may have been performed, and in these, I suspect that thymic tissue may have been removed during the surgery.

Overall, we treat these patients symptomatically.  We have used chronic antibiotics in those whose symptoms support the need.  We have not typically used Tmp/Smx for PJP prophylaxis, but have considered it.  (Have not seen PJP in these patients.)

Overall, we have not performed much further evaluations…but…If other anemias are present, particularly thrombocytopenia, we evaluate for hematologic abnormalities (although for us, MDS most commonly presents with low NK cell count and thrombocytopenia).

Terry Harville MD PhD
-Medical Director, Special Immunology Laboratory
-Medical Director, Histocompatibility Laboratory
-Medical Director, Immunogenetics and Transplantation Laboratory
-Specialist in Pediatric Immunology and Rheumatology
Departments of Pathology and Laboratory Services and Pediatrics
University of Arkansas for Medical Sciences
4301 West Markham
Mail Slot #502
Little Rock, AR  72205-7199

Work Phone 1........................................501.686.7257
Work Phone 2........................................501.526.7511
Work Phone 3........................................501.686.7556
Work Fax 1..........................................501.686.7443
Work Fax 2..........................................501.526.4621

Email:...................................harvilleterryo at uams.edu

Special Immunology Laboratory.......................501.364.1804
Histocompatibility Laboratory.......................501.686.7257
Immunogenetics and Transplantation Laboratory.......501.686.7374

From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Monday, October 6, 2014 9:49 AM
To: CIS-PIDD
Subject: [cis-pidd] 74yo F with Lymphopenia

Colleagues:

I was referred a 74yo F who over the last 5 years has developed lymphopenia.

She was diagnosed with “CVID” in 2007.  She had recurrent pneumonias and was started on IVIg although her total IgG level was 853.  She did well on IVIg with no further significant infections.  At ‘routine follow up’ by her PMD  T-cells were shown to be 61% (343) and 5 months later 48% (381).

However, review of her labs demonstrated the low T-cells to be a reflection of a low absolute lymphocyte count, and absolute B-cells were also low.

Most recently:
Absolute Lymphocyte count 600
CD3 45% (273)
CD3,4+ 20% (119)
CD3,8+ 24% (142)
CD19+ 15% (88)
NK  38% (230)

Lymphoproliferation to PHA and PWM were normal.

She has had multiple other medical problems:  COPD (many pack years of smoking), s/p cardiac arrest during surgery, s/p small stroke, s/p major auto accident – all 2011-2013.  Despite these factors on examination she was alert and neurologically intact with good general motor function.

I would very appreciate any suggestions as to the need for and choice of further investigations.  Also, would anyone place on prophylactic antibiotics (PJP, fungus)?

Joe Church
Children’s Hospital Los Angeles

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