Diagnostic studies
| Test . | Comment . |
|---|---|
| All patients with HES | |
| Complete blood count* | |
| Routine chemistries, including liver function tests* | |
| Quantitative serum immunoglobulin levels, including IgE | |
| Serum troponin*, echocardiogram | If abnormal, cardiac MRI should be considered as this may show characteristic features of eosinophilic involvement; tissue involvement may be patchy limiting the utility of biopsy |
| Pulmonary function tests* | |
| Chest/abdomen/pelvis CT* | To assess for splenomegaly, lymphadenopathy, and occult neoplasms |
| Bone marrow biopsy, including cytogenetics* | Recommended in all patients with AEC > 5.0 × 109/L and features of M-HES or L-HES. Should be considered in other patients |
| Biopsies of affected tissues (if possible)* | |
| Other testing as indicated by history, signs, and symptoms | Including parasitic serologies, anti-neutrophil cytoplasmic antibodies, and HIV |
| Serum tryptase and B12 levels | |
| FIP1L1/PDGFRA analysis by FISH or RT-PCR | Testing of peripheral blood is sufficient |
| T- and B-cell receptor rearrangement studies | |
| Lymphocyte phenotyping by flow cytometry* | At a minimum CD3, CD4, and CD8 and CD19 or 20 staining should be performed to assess for aberrant CD3−CD4+, CD3+CD4+CD8+, and CD3+CD4−CD8− populations and B-cell lymphoproliferative disorders |
| Patients with features of M-HES | |
| Additional testing for BCR-ABL1, PDGFRB, JAK2, FGFR1, and KIT mutations by PCR, FISH, or other methods, as appropriate | Testing should be guided by bone marrow findings |
| Patients with evidence of L-HES | |
| Consider PET scan,* lymph node biopsy* | |
| EBV viral load |
| Test . | Comment . |
|---|---|
| All patients with HES | |
| Complete blood count* | |
| Routine chemistries, including liver function tests* | |
| Quantitative serum immunoglobulin levels, including IgE | |
| Serum troponin*, echocardiogram | If abnormal, cardiac MRI should be considered as this may show characteristic features of eosinophilic involvement; tissue involvement may be patchy limiting the utility of biopsy |
| Pulmonary function tests* | |
| Chest/abdomen/pelvis CT* | To assess for splenomegaly, lymphadenopathy, and occult neoplasms |
| Bone marrow biopsy, including cytogenetics* | Recommended in all patients with AEC > 5.0 × 109/L and features of M-HES or L-HES. Should be considered in other patients |
| Biopsies of affected tissues (if possible)* | |
| Other testing as indicated by history, signs, and symptoms | Including parasitic serologies, anti-neutrophil cytoplasmic antibodies, and HIV |
| Serum tryptase and B12 levels | |
| FIP1L1/PDGFRA analysis by FISH or RT-PCR | Testing of peripheral blood is sufficient |
| T- and B-cell receptor rearrangement studies | |
| Lymphocyte phenotyping by flow cytometry* | At a minimum CD3, CD4, and CD8 and CD19 or 20 staining should be performed to assess for aberrant CD3−CD4+, CD3+CD4+CD8+, and CD3+CD4−CD8− populations and B-cell lymphoproliferative disorders |
| Patients with features of M-HES | |
| Additional testing for BCR-ABL1, PDGFRB, JAK2, FGFR1, and KIT mutations by PCR, FISH, or other methods, as appropriate | Testing should be guided by bone marrow findings |
| Patients with evidence of L-HES | |
| Consider PET scan,* lymph node biopsy* | |
| EBV viral load |
Substantially affected by corticosteroid therapy.