SLE

7. AST 20
ALT 24
AlkP 142
T Bili 2.74
D Bili 1.7
TP 4.3
Alb 2.2
LDH 180
D-Dimer +ve
Coomb’s test -ve
WBC 1.7 N71%
PCV 20.8 L 11%
PLT 14
PT20.1
PTT 40.2
INR 1.9

14.  Aspirate 80% cellular, no monoclonal populations present
 Marked hemophagocytosis
 Inclusions suspicious for histoplasma present - PAS and GMS stain positive for budding yeast

21. Estimated involvementClinical features
93-97%Hepatomegaly, splenomegaly, fever
40%Edema
33-34%Lymphadenopathy, jaundice
33%
Neurologic symptoms 33% Seizures, mental status
changes/encephalopathy, ataxia, posterior
reversible encephalopathy syndrome (PRES)
31%Rash Erythroderma, petechiae, purpura
variableOtherVariable Respiratory dysfunction/ARDS,
severe hypotension, bleeding, renal dysfunction,
SIADH

22. Estimated involvementLaboratory abnormality
Ferritin
93-100%> 500 ng/ml
42%> 5000 ng/ml
35-58%> 10000 ng/ml
Cytopenia
84%Anemia
79%Thrombocytopenia
42%Neutropenia
87-100%Elevated LDH
76-90%Elevated liver enzymes
62%Hypofibrinogenemia
44%Hypertriglyceridemia

27. Even in presence of underlying autoimmune disease, must consider that etiology of HLH is
infectious, especially if received biologic therapy

31. othersJIASLEFHLH
rituximab
(EBV-
related)
methylprednisolonemethylprednisolonedexamethasoneCorticosteroids
Cyclophosphamide
Rituximab
alemtuzumab
Etoposide
ant thymocyte globulin
alemtuzumab
Cytoablation
(B andT cell)
abataceptcyclosporine A (jSLE)cyclosporine AT-cell directed therapy
IFN-y monoclonal abIFN-y blockade
IL-1B
Recombinant IL-18BP
IL-33IL-1 cytokine family
blockade
tocilizumabIL-6 blockade
TNF blockade
JAK inhibition
IVIGJAK inhibition
HSC transplant
Others

34. Thank you