[CIS PIDD] [cis-pidd] LES vs atypical lymphoproliferation?

[Dr. Carsten Speckmann]

  I am confused by the lymphocyte numbers: The range of total 
lymphocytes is between 900-2000 (per µl I assume).
The percentage of CD4, CD8, CD19 and NK from January 12th suggests very 
low numbers (much lower than total lmphocytes). What is the total number 
of CD3 (in relation to CD3CD4 and CD3CD8? CD3 TCRg/d expansion? How are 
levels of IgG A M E? If the patient has low T B NK cells and 
autoimmunity consider late onset ADA (i.e. if IgE is elevated).

Kind regards, Carsten Speckmann

-- 
Dr. med. Carsten Speckmann
Facharzt
Zentrum fuer Kinderheilkunde und Jugendmedizin
Centrum fuer Chronische Immundefizienz - CCI
Universitaet Freiburg
Mathildenstr. 1
79106 Freiburg
Germany

phone: +49 (0)761-270 43010
mail: carsten.speckmann at uniklinik-freiburg.de
web: www.cci.uniklinik-freiburg.de


Am 22.02.13 12:50, schrieb Wilmer Cordova Calderon:

>

>

>

> Best regards

> This is a difficult case to me. The patient have leucocitos low and 

>  predominance linphocytes

> Persistent  ANA positive with nephritis nephrotic syndrome

> Hematology: Think its Lupus  but Reumathology dont think so.

> I think there is a combination.

> What do you think?

>

> general Information

> female patient

> 6 years

> laboratory

> ANA 1/1000

> AMO: Reactive

> AMO: pancytopenia

> TAC: retroperitoneal lymphadenopathy> 23mm

> AMO (nov/12) erythroid and megakaryocytic hyperplasia.

> Biopsy: no infiltrative

> Node biopsy: lymphoid hyperplasia

> hematuria, proteinuria

> 2011 autoimmune hemolytic anemia. Treated with corticosteroid

> Physical Examination:

> gingivorrhagia

> Petechiae, lymphadenopathy, hepatomegaly, splenomegaly

>

> Hemograma

>

> 	

>

> 12/1/13

>

> 	

>

> 8/1/13

>

> 	

>

> 3/1/13

>

> 	

>

> 29/12/12

>

> 	

>

> 24/12

>

> 	

>

> 23/12/12

>

> 	

>

> 22/1/12

>

> Leucos

>

> 	

>

> 1290

>

> 	

>

> 2100

>

> 	

>

> 1370

>

> 	

>

> 1660

>

> 	

>

> 1900

>

> 	

>

> 1700

>

> 	

>

> 3000

>

> Eos

>

> 	

>

> 10

>

> 	

>

> 6

>

> 	

>

> 13

>

> 	

>

> 25

>

> 	

>

> 20

>

> 	

>

> 9

>

> 	

>

> 15

>

> Seg

>

> 	

>

> 15

>

> 	

>

> 24

>

> 	

>

> 13

>

> 	

>

> 13

>

> 	

>

> 20

>

> 	

>

> 24

>

> 	

>

> 8

>

> Linfos

>

> 	

>

> 70

>

> 	

>

> 65

>

> 	

>

> 64

>

> 	

>

> 51

>

> 	

>

> 49

>

> 	

>

> 51

>

> 	

>

> 68

>

> Monos

>

> 	

>

> 5

>

> 	

>

> 4

>

> 	

>

> 9

>

> 	

>

> 10

>

> 	

>

> 8

>

> 	

>

> 8

>

> 	

>

> 7

>

> Plaquetas

>

> 	

>

> 4000

>

> 	

>

> 30000

>

> 	

>

> 100000

>

> 	

>

> 9000

>

> 	

>

> 	

>

> 	

>

> Hb

>

> 	

>

> 10

>

> 	

>

> 	

>

> 10

>

> 	

>

> 10

>

> 	

>

> 	

>

> 	

>

> PCR

>

> 	

>

> 0.9

>

> 	

>

> 1.17

>

> 	

>

> 1.15

>

> 	

>

> 2.36

>

> 	

>

> 10

>

> 	

>

> 	

>

> C4

>

> 	

>

> 28

>

> 	

>

> N

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> C3

>

> 	

>

> 124

>

> 	

>

> N (90-180)

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> Ferritina

>

> 	

>

> 840+

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> Proteínas orina

>

> 	

>

> 636 mg/24h

>

> 	

>

> 5408

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> Anti dsDNA

>

> 	

>

> Negativo

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> Anticardiolipina

>

> 	

>

> Negativo

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> B2glicoproteína

>

> 	

>

> 0.3 -

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> ANA

>

> 	

>

> 1/1000

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> Coombs

>

> 	

>

> Negativo

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> DHL

>

> 	

>

> 862

>

> 	

>

> Elevado

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> FR

>

> 	

>

> 4.4

>

> 	

>

> Negativo

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> Citometría medula osea

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> B maduros

>

> 	

>

> 1.05%

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> B intermedios

>

> 	

>

> 0.84%

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> CD4

>

> 	

>

> 1.1%

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> CD8

>

> 	

>

> 1.9%

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> T CD4+CD8+

>

> 	

>

> 0.09%

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> T CD4-CD8-

>

> 	

>

> 0.15%

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> NK

>

> 	

>

> 0.12%

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> 	

>

> coombs directo indirect POSITIVE

> Neutropenia severa

>

>

> -- 

> *Atte

> *

>

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