[CIS PIDD] [cis-pidd] Recurrent pneumonia, hypergammaglobulinemia
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Mon Oct 17 16:28:05 EDT 2016
With weight loss, s/p kidney transplant with chronic immune suppression and monoclonal IgA, I would be concerned for B-cell malignancy. He is the right age for IgA heavy chain disease (aka immunoproliferative small intestinal disease, IPSID), as is mentioned on the IF report, but heavy chain disease as a whole is pretty rare, and alpha heavy chain dz is most often seen in Mediterranean countries. On the other hand, alpha HCD is a type of maltoma, and this lymphoma is increased after solid organ transplant. Not sure how respiratory symptoms relate. IPSID should give malabsorption. Does he have diarrhea?
--
Rob Sokolic
Medical Officer
Center for Biologics Evaluation and Research
Office of Cellular, Tissue and Gene Therapies
U.S. Food and Drug Administration
Tel: 240-402-5564
Robert.Sokolic at fda.hhs.gov<mailto:Robert.Sokolic at fda.hhs.gov>
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From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Monday, October 17, 2016 4:07 PM
To: CIS-PIDD
Subject: [cis-pidd] Recurrent pneumonia, hypergammaglobulinemia
I have followed an now 37yo with Prader-Willi and chronic renal failure s/p a transplant that was successful for several years and was lost to chronic rejection. I have followed him for several years with occasional chest infections but over the past six months he has had recurrent bronchitis and pneumonia and a significant weight loss. Evaluation is below. I would be interested in any suggestions for further evaluation.
Thanks,
Richard Wasserman
Dallas
Lymphocyte Subset
% CD3 72 % 62-87
! Absolute CD3 1835 cells/uL 570-2400
! % CD4 33 % 32-64
! Absolute CD4 853 cells/uL 430-1800
! % CD8 35 % 15-46
! Absolute CD8 897 cells/uL 210-1200
! CD4:CD8 Ratio 0.94 ratio 0.80-3.90
! % Natural Killer Cells
[H] 27 % 4-26
! Absolute Natural Killer Cells
[H] 685 cells/uL 78-470
! % CD19 [L] 1 % 6-23
! Absolute CD19 [L] 34 cells/uL 91-610
! % CD45RA 41 % 28-71
! Absolute CD45RA 379 cells/uL 350-1100
! % CD45RO 59 % 28-72
! Absolute CD45RO 542 cells/uL 340-1150
Comment See Note
B-Cell Memory and Naive Panel
! B-cells % CD19 [L] 1 % 5-26
! B-cells Absolute CD19
[L] 33 cells/uL 58-558
! Naive B-cell %CD19+/CD27-/IgD+
29 % 29-93
! Naive BCL Abs CD19+/CD27-/IgD+
[L] 10 cells/uL 22-423
! Non-switched Memory %
5 % 2-25
! Non-switch Abs [L] 2 cells/uL 4-66
! Class-switched Memory %
20 % 3-23
! Class-switched Abs 7 cells/uL 4-62
! IgM Only Memory % 1.4 % .3-6.0
! IgM Only Memory Abs [L] 0.5 cells/uL .6-16.4
! Total Memory B-cell%CD19/CD27+
26 % 7-48
! Tot Mem BCL Absol CD19+/CD27+
[L] 9 cells/uL
Immunoglobulin G, Qn, Serum
[H] 1633 mg/dL 700-1600
Immunoglobulin A, Qn, Serum
[H] 491 mg/dL 90-386
Immunoglobulin M, Qn, Serum
42 mg/dL 20-172
Immunoglobulin E, Total
[H] 1801 IU/mL 0-100
Protein Electro.,S
Protein, Total, Serum
6.9 g/dL 6.0-8.5
Albumin 3.2 g/dL 2.9-4.4
Alpha-1-Globulin 0.2 g/dL 0.0-0.4
Alpha-2-Globulin 0.9 g/dL 0.4-1.0
Beta Globulin 1.1 g/dL 0.7-1.3
Gamma Globulin 1.6 g/dL 0.4-1.8
M-Spike [H] 0.3 g/dL Not Observed
BETA
Globulin, Total 3.7 g/dL 2.2-3.9
A/G Ratio 0.9 0.7-1.7
Immunofixation, Serum
Immunofixation Result, Serum
Note:
Immunofixation shows IgA monoclonal protein with no confirmed
light chain association, which is suggestive of
Heavy Chain Disease. Recommend retesting in 6-10 weeks.
Pneumo Ab Type 1* [L] 0.1 ug/mL >1.3
Pneumo Ab Type 3* 2.6 ug/mL >1.3
Pneumo Ab Type 4* [L] <0.1 ug/mL >1.3
Pneumo Ab Type 8* [L] 0.1 ug/mL >1.3
Pneumo Ab Type 9 (9N)*
[L] 0.3 ug/mL >1.3
Pneumo Ab Type 12 (12F)*
[L] 0.5 ug/mL >1.3
Pneumo Ab Type 14* [L] 0.4 ug/mL >1.3
! Pneumo Ab Type 17 (17F)*
[L] 1.1 ug/mL >1.3
Pneumo Ab Type 19 (19F)*
1.5 ug/mL >1.3
! Pneumo Ab Type 2* [L] 0.3 ug/mL >1.3
! Pneumo Ab Type 20* [L] 0.4 ug/mL >1.3
! Pneumo Ab Type 22 (22F)*
[L] <0.1 ug/mL >1.3
Pneumo Ab Type 23 (23F)*
[L] <0.1 ug/mL >1.3
Pneumo Ab Type 26 (6B)*
2.7 ug/mL >1.3
Pneumo Ab Type 34 (10A)*
[L] 0.2 ug/mL >1.3
Pneumo Ab Type 43 (11A)*
[L] 0.2 ug/mL >1.3
Pneumo Ab Type 5* [L] 0.3 ug/mL >1.3
Pneumo Ab Type 51 (7F)*
[L] 0.3 ug/mL >1.3
Pneumo Ab Type 54 (15B)*
[L] 0.1 ug/mL >1.3
Pneumo Ab Type 56 (18C)*
[L] <0.1 ug/mL >1.3
Pneumo Ab Type 57 (19A)*
6.3 ug/mL >1.3
Pneumo Ab Type 68 (9V)*
[L] 0.1 ug/mL >1.3
Pneumo Ab Type 70 (33F)*
[L] 0.5 ug/mL >1.3
Haemophilus influenzae B IgG
0.16 ug/mL
Tetanus Antitoxoid IgG Ab
2.39 IU/mL <0.10
Diphtheria Antitoxoid Ab
0.64 IU/mL <0.10
--
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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