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Ferritin, coagulation panel with fibrinogen; LDH, lactate, triglyceride, beta2-microglobulin, CRP, CBC with diff, peripheral smear review, CMP: order STAT
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Soluble IL2 receptor, cytokine panel (if available locally or as a send out), soluble CD163
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Cultures blood, urine, sputum, bronchoscopy
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NK function and T-lymphocyte subsets by PB flow requisition
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Pregnancy test
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Serologies: EBV, CMV, HSV, acute hepatitis screen, parvovirus
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Viral loads: EBV, CMV, HHV-6, HHV-8, HIV, parvovirus, adenovirus if prior alloSCT;
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Other:
Urine: histoplasmosis antigen; serum quantiferon
DAT, ANA (other rheumatology work-up if indicated)
Immunoglobulin panel (quantitative immune globulins, IgG subsets)
Transferrin saturation: if elevated, HFE gene analysis
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Imaging:
PET-CT preferred over CT chest/abdomen/pelvis
MRI brain, possibly spine
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Biopsies (as clinically indicated to identify hemophagocytosis or diagnose/stage underlying tumor):
Bone marrow aspiration and biopsies
Lymph node aspiration by FNAC if palpable nodes, consider excisional biopsy
Skin biopsies particularly if rash, livedo, skin lesions, normal vascular changes/red moles
Liver biopsy (if HLH liver involvement features)
Lumbar puncture (diagnostic and therapeutic) as soon as bleeding risk declines
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Hypomorphic mutation genetic testing: to be ordered when HLH diagnosis confirmed (if available locally or as a send-out)