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

[Church, Joseph]

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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