Patient’s laboratory evaluation for etiology of secondary HLH
| Laboratory results . |
|---|
| Infectious |
| Blood and urine cultures: negative |
| Nasopharyngeal viral swabs: positive for enterovirus but repeat negative |
| Enterovirus blood PCR: negative |
| EBV PCR: negative |
| EBV IgG: positive; IgM: negative |
| CMV IgG: positive; IgM: negative |
| HIV antibody: negative |
| HIV RNA PCR: negative |
| Leishmaniasis antibody: negative |
| Endotracheal tube culture: normal flora |
| Bronchoalveolar lavage: negative |
| CSF encephalitis panel*: negative |
| Fungitel: negative |
| Galactomannan: negative |
| Histoplasmosis antibody: negative |
| Histoplasmosis antigen: negative |
| Blastomyces antibody: negative |
| Blastomyces antigen: negative |
| Hepatitis A IgG: positive; IgM: negative |
| Hepatitis B surface IgG: positive |
| Hepatitis B core IgG: negative |
| Hepatitis C IgG: negative |
| Malaria prep: negative |
| Strongyloides antibody: negative |
| Brucella IgG: negative; IgM: negative |
| Malaria prep: negative |
| Parvovirus: negative |
| Quantiferon gold: indeterminate |
| PPD: negative |
| Immunologic |
| IgG: 1890, IgA:400, IgE: 1124, IgM: 97 (mg/dL) |
| Normal vaccine response to tetanus and diphtheria, present but low response to Haemophilusinfluenzae type b |
| CD3+: 310/mm3 (CD4 <200/mm3) |
| CD56: 0/mm3 |
| CD19: 212/mm3 |
| Rheumatologic |
| ACE: 40 U/L (normal range, 13-100 U/L) |
| C3: 173 mg/dL (normal, 80-156 mg/dL) |
| C4: 30.5 mg/dL (normal, 12-43 mg/dL) |
| Anti-RNP: 110 AU/mL (normal < 100 AU/mL) |
| Anti-SSA/SSB: <100 AU/mL (normal <100 AU/mL) |
| ANA titer: 80, nucleolar (normal <40) |
| Anti-dsDNA: 2 iU/mL (normal ≤4 iU/mL) |
| Anticardiolipin IgG and IgM: negative |
| Lupus anticoagulant: negative |
| Antiproteinase 3 antibody: <1.0 AI (normal <1.0 AI) |
| Antimyeloperoxidase antibody: <1.0 AI (normal <1.0 AI) |
| Periodic fever syndrome panel†: negative |
| ADAMTS13 activity: 55% (assay can be inhibited by hyperbilirubinemia, normal >67%) |
| Creatine kinase: 49 iU/L (normal, 30-150 iU/L) |
| Cryoglobulins: positive |
| Haptoglobin: 151 mg/dL (normal, 43-212 mg/dL) |
| Oncologic |
| CT head, chest, abdomen negative for malignancy |
| Bone marrow flow cytometry negative for leukemia; cytogenetics normal |
| Laboratory results . |
|---|
| Infectious |
| Blood and urine cultures: negative |
| Nasopharyngeal viral swabs: positive for enterovirus but repeat negative |
| Enterovirus blood PCR: negative |
| EBV PCR: negative |
| EBV IgG: positive; IgM: negative |
| CMV IgG: positive; IgM: negative |
| HIV antibody: negative |
| HIV RNA PCR: negative |
| Leishmaniasis antibody: negative |
| Endotracheal tube culture: normal flora |
| Bronchoalveolar lavage: negative |
| CSF encephalitis panel*: negative |
| Fungitel: negative |
| Galactomannan: negative |
| Histoplasmosis antibody: negative |
| Histoplasmosis antigen: negative |
| Blastomyces antibody: negative |
| Blastomyces antigen: negative |
| Hepatitis A IgG: positive; IgM: negative |
| Hepatitis B surface IgG: positive |
| Hepatitis B core IgG: negative |
| Hepatitis C IgG: negative |
| Malaria prep: negative |
| Strongyloides antibody: negative |
| Brucella IgG: negative; IgM: negative |
| Malaria prep: negative |
| Parvovirus: negative |
| Quantiferon gold: indeterminate |
| PPD: negative |
| Immunologic |
| IgG: 1890, IgA:400, IgE: 1124, IgM: 97 (mg/dL) |
| Normal vaccine response to tetanus and diphtheria, present but low response to Haemophilusinfluenzae type b |
| CD3+: 310/mm3 (CD4 <200/mm3) |
| CD56: 0/mm3 |
| CD19: 212/mm3 |
| Rheumatologic |
| ACE: 40 U/L (normal range, 13-100 U/L) |
| C3: 173 mg/dL (normal, 80-156 mg/dL) |
| C4: 30.5 mg/dL (normal, 12-43 mg/dL) |
| Anti-RNP: 110 AU/mL (normal < 100 AU/mL) |
| Anti-SSA/SSB: <100 AU/mL (normal <100 AU/mL) |
| ANA titer: 80, nucleolar (normal <40) |
| Anti-dsDNA: 2 iU/mL (normal ≤4 iU/mL) |
| Anticardiolipin IgG and IgM: negative |
| Lupus anticoagulant: negative |
| Antiproteinase 3 antibody: <1.0 AI (normal <1.0 AI) |
| Antimyeloperoxidase antibody: <1.0 AI (normal <1.0 AI) |
| Periodic fever syndrome panel†: negative |
| ADAMTS13 activity: 55% (assay can be inhibited by hyperbilirubinemia, normal >67%) |
| Creatine kinase: 49 iU/L (normal, 30-150 iU/L) |
| Cryoglobulins: positive |
| Haptoglobin: 151 mg/dL (normal, 43-212 mg/dL) |
| Oncologic |
| CT head, chest, abdomen negative for malignancy |
| Bone marrow flow cytometry negative for leukemia; cytogenetics normal |
ACE, angiotensin converting enzyme; AI, antibody index; ANA, antinuclear antibodies; CMV, cytomegalovirus; CSF, cerebrospinal fluid; CT, computed tomography; EBV, Epstein-Barr virus; Ig, immunoglobulin; PCR, polymerase chain reaction; PPD, tuberculosis skin test.
Encephalitis panel tested: NMDA receptor antibody, VGKC-complex antibody, CAD65 antibody, GABA-B receptor antibody, AMPA receptor antibody, ANNA-1, ANNA-2, ANNA-3, AGNA-1, PCA-1, PCA-2, PCA-Tr, amphiphysin antibody, CRMP-5-IgG.
Periodic fever panel genes tested: ELA2, LPIN2, MEFV, MVK, NLRP3, PSTP1P1, TNFRSF1A.