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Table 1 Extensive lab evaluation for infection, malignant, and rheumatologic diseases as underlying etiology of MAS

From: Progressive, refractory macrophage activation syndrome as the initial presentation of anti-MDA5 antibody positive juvenile dermatomyositis: a case report and literature review

Initial Lab Evaluation

Labs

Result (Classification)

Blood/Urine/Fungal/Anaerobic/Stool Cultures

Negative

Covid-19 PCR and IgG & IgM

Negative

EBV/CMV IgG & IgM

Negative

Hepatitis A/B/C- Ab Panel

Nonreactive

Leptospira Culture & Ab

Negative

Bartonella Henselae/Quintana- Ab IgG & IgM

Negative

Rickettsia Typhi- Ab IgG & IgM

Negative

Legionella- Ag(urine)

Negative

Mycoplasma pneumonia- Ab IgM

Negative

HIV-1 + 2 Ab/HIV P24 Ag

Negative

Microbial/viral/fungal/protozoal free DNA

No statistically significant levels detected

Coccidioides-Abs IgG &IgM

< 0.2 (Normal)

Blastomyces- Ab

Negative

Histoplasma Capsulatum-Ag(urine)

Negative

Cryptosporidum/Giardia- Ag(stool)

Negative

Enterovirus RNA (stool)

Negative

Blood Smear

Anemia, thrombocytopenia, neutropenia, lymphopenia

Bone Marrow Biopsy (smear)

No evidence of malignancy or HLH

Bone Marrow Biopsy (FISH)

All test values within normal limits

Bone Marrow Biopsy (Leukemia/Lymphoma panel)

No evidence of B/T cell lymphoproliferative disorder

Bone Marrow Biopsy (Cytogenetics)

No clonal abnormalities

ANA

Negative

RF

< 15 (Normal)

Cyclic Citrullinated Peptide IgG & IgA

9 (Normal)

Centromere B-Ab

< 0.2 (Normal)

SCL-70 Extractable Nuclear-Ab

< 0.2 (Normal)

Ribonucleoprotein Extractable Nuclear-Ab

0.2 (Normal)

Smith Extractable Nuclear-Ab

< 0.2 (Normal)

SS-A-Ab

1.5 (High)

SS-B-Ab

< 0.2 (Normal)

DNA DS-Ab

< 1 (Normal)

Creatinine Kinase

217 (High)

Aldolase

14.9 (High)

Smooth Muscle-Ab

87 (High)

Cardiolipin/Beta-2 Glycoprotein IgG & IgM

Negative

Liver-Kidney-Ab

0.8 (Normal)

Mitochondrial- Ab

< 20.0 (Normal)

Parietal Cell- Ab

1.2 (Normal)

Von Willebrand Factor-Ag

239 (High)