[CIS PIDD] [cis-pidd] Patient question

[Oscar de la Calle-Martín]

Dear colleague,

You should look for HLA-I expressión.

Best regards,

Oscar de la Calle


2014-04-28 15:28 GMT+02:00 Prescott Atkinson, M.D. <PAtkinson at peds.uab.edu>:


>   I'd appreciate advice from the group on a case.  I am following a 43 yo

> white female with a history of repeated pneumonias since childhood, now

> with bronchiectasis, h/o ITP/splenectomy and an impressive h/o recurrent

> infections - mainly RLL pneumonias/bronchiectasis. IgM is mildly low but

> immunoglobulins are normal.  She has a h/o cervical carcinoma and now has

> been diagnosed with squamous cell carcinoma of the neck positive for p16

> suggesting HPV.  She has a h/o bleeding with normal platelet counts and her

> platelet function assay is abnormal (normal kinetics but low ATP release) -

> this has been repeated.  She developed gray hair in her 20's and has had

> bilateral hearing loss since childhood.  Response to pneumovax was

> excellent (pre-titers are shown below).  NK cell function (Cincinnati) was

> normal.

>

>

>

>

>

> * * Final Report * *

>

> The patient's platelets aggregated with all agonists tested: arachidonic

> acid, ADP and collagen, consistent with normal platelet function. However,

> we noticed decreased ATP release when her platelets were exposed to

> collagen and arachidonic acid, but not thrombin. This finding may represent

> a mild defect in her granular contents, which may explain some of her

> bleeding history. Dr. M. Marques

>

> This test was reviewed and approved by the signing pathologist. 10/15/2013

> 15:25:07 CDT

>

> *

> __________________________________________________________________________________

> Flow Cytometry Clinical Information *

>

> The patient is a 43 year old female with a clinical history of IgA

> deficiency. The patient's current WBC is 10,700 cells/microliter with a

> differential of 52% segs, 38% lymphs, 9% monos, and 1% basos. The platelet

> count is 273,000 cells/microliter and the hemoglobin is 12.3 g/dL.

>

> The following cell markers were analyzed by flow cytometry; the data is on

> file in the Laboratory: CD2, CD3, CD4, CD5, CD8, CD10, CD16, CD19, CD20,

> CD23, Mu, Delta, Kappa, Lambda, CD56, CD57 and CD45.

>

> This test was developed and its performance characteristics determined by

> UAB Pathology. It has not been cleared or approved by the U.S. Food and

> Drug Administration. The FDA has determined that such clearance or approval

> is not necessary. This test is used for clinical purposes. This laboratory

> is certified under the Clinical Laboratory Improvement Amendments of 1988

> (CLIA) as qualified to perform high complexity clinical laboratory testing.

>

> * FC Gross Description *

>

> The specimen is received in a single tube labeled with the patient's name

> and containing 3 mL of peripheral blood. The specimen is processed by the

> whole blood lysis technique.

>

> Dr. Reddy/Dr. Hanna

>

> 10/15/2013 10:21:48 CDT

>

> * FC Diagnosis Peripheral blood: - Mild CD4+ T-cell predominance; CD4:CD8

> ratio of 3.6 *

>

>  Vishnu Reddy MD

>

> Verified: 10/15/13 13:37 VR/CAH

>

> Reviewed by: Hanna, Courtney Alexandra MD MD

>

> * FC Pathologist Comment *

>

> The Wright stained peripheral smear shows normal hematopoetic elements.

>

> From the CBC data, the absolute lymphocyte count is 4066 cells/microliter.

>

> Flow cytometry analysis shows that 67% of the lymphocytes are CD3+ T cells

> for an absolute T cell count of 2,732 cells/microliter. About 51% of the

> lymphocytes are CD4+ T cells, while 14% are CD8 T cells for a ratio of 3.6.

> Subsets of the T cells co-express CD56 (0.4% of the total lymphocytes),

> CD57 (2% of the total lymphocytes), and CD16 (0.2% of the total

> lymphocytes). About 9.1% of the lymphocytes are NK lineage cells. A total

> of 26% of the lymphocytes are B cells (absolute count = 1,038

> cells/microliter), with a small subset of CD10+ B cells (CD10+, CD19+) (

> 0.1% of lymphocytes). A total of 2.5% of the lymphocytes co-express CD19

> and low density CD5. About 95% of the B cells show bright expression of

> surface IgM and IgD with polyclonal light chains, with about 2.5% of the B

> cells being surface IgM and IgD negative.

>

>    * Result Name*

>

> * Current Result*

>

> * Normal Range*

>

> WBC (10^3/cmm)

>

> * 10.70* 10/14/2013

>

> 4.00 - 11.00

>

> RBC (10^6/cmm)

>

> * 3.79* 10/14/2013

>

> 3.80 - 5.20

>

> Hgb (gm/dL)

>

> * 12.3* 10/14/2013

>

> 11.3 - 15.2

>

> Hct (%)

>

> * 38* 10/14/2013

>

> 33 - 45

>

> MCV (fL)

>

> * 100* 10/14/2013

>

> 80 - 96

>

> MCH (pg)

>

> * 32* 10/14/2013

>

> 27 - 33

>

> MCHC (gm/dL)

>

> * 32* 10/14/2013

>

> 32 - 36

>

> Platelet (10^3/cmm)

>

> * 273* 10/14/2013

>

> 150 - 400

>

> RDW (%)

>

> * 14.1* 10/14/2013

>

> 11.0 - 16.0

>

> MPV (fL)

>

> * 9* 10/14/2013

>

> NRBC Inst (10^3)

>

> * 0.0* 10/14/2013

>

> Neutrophils (%)

>

> * 52* 10/14/2013

>

> 35 - 73

>

> Abs.Neutrophils (10^3/cmm)

>

> * 5.55* 10/14/2013

>

> Lymphocytes (%)

>

> * 38* 10/14/2013

>

> 15 - 52

>

> Monocytes (%)

>

> * 9* 10/14/2013

>

> 4 - 13

>

> Eosinophils (%)

>

> * 0* 10/14/2013

>

> 0 - 5

>

> Basophils (%)

>

> * 1* 10/14/2013

>

> 0 - 2

>

> IgG 1 (mg/dL)

>

> * 540* 10/14/2013

>

> 382-929 -

>

> IgG 2 (mg/dL)

>

> * 363* 10/14/2013

>

> 241-700 -

>

> IgG 3 (mg/dL)

>

> * 72* 10/14/2013

>

> 22-178 -

>

> IgG 4 (mg/dL)

>

> * 0.8* 10/14/2013

>

> 4.0-86.0 -

>

> IgG Total (mg/dL)

>

> * 1,050* 10/14/2013

>

> 694-1618 -

>

> Pneumo Sero 1 (mcg/mL)

>

> * 0.88* 10/14/2013

>

> Pneumo Sero 3 (mcg/mL)

>

> * 1.95* 10/14/2013

>

> Pneumo Sero 4 (mcg/mL)

>

> * 0.76* 10/14/2013

>

> Pneumo Sero 5 (mcg/mL)

>

> * 12.21* 10/14/2013

>

> Pneumo Sero 6B (mcg/mL)

>

> * 3.67* 10/14/2013

>

> Pneumo Sero 7F (mcg/mL)

>

> * 3.64* 10/14/2013

>

> Pneumo Sero 8 (mcg/mL)

>

> * 1.81* 10/14/2013

>

> Pneumo Sero 9N (mcg/mL)

>

> * 2.68* 10/14/2013

>

> Pneumo Sero 9V (mcg/mL)

>

> * 1.10* 10/14/2013

>

> Pneumo Sero 12F (mcg/mL)

>

> * 3.11* 10/14/2013

>

> Pneumo Sero 14 (mcg/mL)

>

> * 5.95* 10/14/2013

>

> Pneumo Sero 18C (mcg/mL)

>

> * 8.01* 10/14/2013

>

> Pneumo Sero 19F (mcg/mL)

>

> * 17.58* 10/14/2013

>

> Pneumo Sero 23F (mcg/mL)

>

> * 0.96* 10/14/2013

>

> Plt Agg

>

> 10/14/2013

>

>

>

> T. Prescott Atkinson, MD PhD, Professor and Director

>

> Division of Pediatric Allergy, Asthma & Immunology

>

> University of Alabama at Birmingham

>

> Tel: 205-939-9072

>

> Fax: 205-975-7080

>

> ---

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-- 
------------------------------------------------------------------------------------------------------------

Dr. Oscar de la Calle-Martín
Laboratoris, Planta -2, Bloc B,
Servei d'Immunologia, Hospital de Sant Pau
C/ San Quintin 89, E-08041 Barcelona, SPAIN
MOVIL SANT PAU 37386 / 682 745 510
93 553 7265 (SECRETARIA), 93 553 7386 / 93 553 7546 (DESPACHO)
odlcalle at gmail.com

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