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

[Church, Joseph]

Consider macrophage activation syndrome in this patient with autoimmune disease, neutropenia, thrombocytopenia, organomegaly and elevated ferritin level.

Joe Church, MD
Children's Hospital Los Angeles


From: Wilmer Cordova Calderon [mailto:wilmer.cordova at gmail.com]
Sent: Friday, February 22, 2013 3:51 AM
To: CIS-PIDD
Subject: [cis-pidd] LES vs atypical lymphoproliferation?



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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Atte

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