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

[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


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