[CIS PIDD] Chronic leukocytosis

[Ochs, Hans]

I agree with Ricardo, LAD has to be ruled out.
Consanguinity?
Sepsis and pneumonia are not common in LAD.
Can she make pus?
Does she have peridontal disease?
hans


Hans D. Ochs, MD, Dr. med
Professor of Pediatrics | Jeffrey Modell Chair of Pediatric Immunology Research
Center for Immunity and Immunotherapies
Seattle Children's Research Institute | University of Washington

206-987-7450 OFFICE
206-987-7310  FAX
allgau at u.washington.edu<mailto:allgau at u.washington.edu>, hans.ochs at seattlechildrens.org<mailto:hans.ochs at seattlechildrens.org>

OFFICE   1900 Ninth Avenue, Seattle, WA 98101
MAIL      M/S C9S-7
WWW     seattlechildrens.org<http://seattlechildrens.org/>

Assistant:
Dawn Marie Pares
206-884-7418   OFFICE
dawnmarie.pares at seattlechildrens.org<mailto:dawnmarie.pares at seattlechildrens.org>

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Sorensen, Ricardo
Sent: Saturday, August 11, 2012 11:24 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] Chronic leukocytosis

Wilmer,

As you point out in your differential, this could be a form of LAD. When did his cord fall off?

I would Amos Etzioni for his opinion and advise directly: etzioni at rambam.health.gov.il<mailto:etzioni at rambam.health.gov.il>

Ricardo Sorensen

From: pagid-bounces at list.clinimmsoc.org<mailto:pagid-bounces at list.clinimmsoc.org> [mailto:pagid-bounces at list.clinimmsoc.org]<mailto:[mailto:pagid-bounces at list.clinimmsoc.org]> On Behalf Of Wilmer Cordova Calderon
Sent: Tuesday, August 07, 2012 2:46 AM
To: pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>
Subject: [CIS PIDD] Chronic leukocytosis

Best regards, this a very difficult case because I dont have flow citometry and others advances test
Here I sending all information that can have
Its a long time leukocytosis and hematology dont think in a malignancy (3 year with it)
The patient is eutrophic, but with  recurrent and chronic infections
Ill thanks a lot your suggestions and comment


Age: 3 years
female
Date of birth: March 31, 2009
BACKGROUND
Product of 2nd pregnancy
Cesarean delivery, 8 months, birth weight 2900 g
Fall of umbilical cord at 10 days
Complete Vaccines: up years of age.
The 1st hospitalization: 15 days old FEVER OVER PNEUMONIA (WBC 70 000), is hospitalized for 1 month 15 days.
The 2nd hospitalization 1 year 2months, VIRAL BRONCHITIS (WBC 80 000), hospitalized for 1 month
infections
Cellulite: 5 times: internal angle left eye, left elbow, right thigh, vagina (2)
Otitis media: 4 times
Pneumonia: 1 time
Bronchitis 1 time
Sepsis: 2 times.
Apparently healthy relatives
Transfusion of red blood cells: once
Bone marrow aspirate 31/3/9, at the age of 1 year 11 months, without alteration, NO leukemia
Final control of CBC:
Leukocytes (55 000)
Eosinof (2%)
Segm (75%)
Lymphatic (18%)
Mon (4%)
Hb (7.7)
Retic (0.4%)
Serum iron (35)
Transfer (273)
% Sat transf (12.8%)
Protein electrophoresis 29/11/11: Hypoalbuminemia. Polyclonal increase of gamma globulin. Increased alpha 1 and 3.
DIAGNOSTICS:
Chronic leukocytosis:
Myeloproliferative syndrome. Leukocyte Adhesion Deficiency
Moderate chronic anemia - severe
Recurrent infection syndrome
Recurrent otitis media
Chronic otitis media left: fungal
recurrent cellulitis
chronic gingivostomatitis
Atopy: allergic rhinitis may

HEMOGRAMA/FECHA









Recuento/Fecha

28/01/2011

15/02/2011

24/02/11

07/03/11

14/03/11

28/01/11

01/06/11

03/09/11

02/11/11

01/02/12

21/02/12

03/04/12

12/06/12

02/07/12

30/07/12

Leucocitos n° absoluto/células/μL

40200

23100

33800

28500

23400

40 200

23 960

50 500

30 280

66 390

33 100

28 800

43 460

36 380

55 000

Neutrófilos %/ células/μL

26130

14553

24336

20805

13338

26 532

15 094,8

32 825

19 984,8

53 775,9

28 135

19 296

31 291,2

27 285

44 000

Linfocitos % células/μL

12060

6930

6760

6270

7258

12 060

6 948,4

13 635

8 478,4

6 639

4 634

7 776

9 561,2

6 548,4

7 700

BASOFILOS

402

0

0

0

234

402

0

505

0

0

0

0

0

727,6

0

Monocitos

804

1383

1690

1425

2106

804

1 677,2

2 020

1 816,8

4 647,3

0

1 152

2 607,6

1 819

2 200

Eosinófilos

402

231

1014

0

234

402

239,6

1 515

0

1 327,8

331

576

1 100

Hemoglobina.

9.3

9.1

9.1

8.6

8.6

9,30

10,10

10,40

10,00

9,00

9,00

10,10

8,50

7,30

7,70

Hematocrito:

30

30

30

27

27

30

32

34

32

29

29

31

27

24

25

Plaquetas:

532

450

406

427

532 000

300 000

400 000

374 000

409 000

250 000

335 000

502 000

525 000

Elementos adicionales: RETICULOCITOS

0.9

2.7

1.2

0,9%

0,7%

1,4%

0,7%

0,8%

1,9%

1,5%

1,1%

1,90%

0,4%

COMENTARIO

ALGUNOS HEMATIES FRAGMENTADOS

LINFO ATIPICO 1%

--
Atte
Wilmer Córdova
Inmunología Alergología
Instituto Nacional de Salud del Niño - Perú
http://www.isn.gob.pe<http://www.isn.gob.pe/>



-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/pipermail/pagid/attachments/20120813/523b8092/attachment.htm>