Organ involvement in hypereosinophilic syndrome
Organ system . | Cumulative frequency from 3 studies, %* . | Examples of organ-specific manifestations . |
|---|---|---|
| Hematologic | 100 | Leukocytosis with eosinophilia; neutrophilia, basophilia, myeloid immaturity, immature and/or dysplastic eosinophils; anemia, thrombocytopenia or thrombocytosis, increased marrow blasts, myelofibrosis19,21 |
| Cardiovascular | 58 | Cardiomyopathy,28,29 constrictive pericarditis,30,31 endomyocarditis,32,33 mural thrombi,27,34 valvular dysfunction,27,35,36 endomyocardial fibrosis,37,38 myocardial infarction39 |
| Dermatologic | 56 | Angioedema,40 urticaria,40 papules/nodules,40 plaques,41 aquagenic pruritis,42 erythroderma,43 mucosal ulcers,44 vesicobullous lesions,45 microthrombi,46,47 vasculitis,48 Wells syndrome49 |
| Neurologic | 54 | Thromboembolism,50 peripheral neuropathy,50,51 encephalopathy,50,52 dementia,53,54 epilepsy,55 cerebellar disease,56 eosinophilic meningitis57 |
| Pulmonary | 49 | Pulmonary infiltrates,9,58 effusions,9,59 fibrosis,4 emboli,60 nodules/focal ground glass attenuation,61 acute respiratory distress syndrome (ARDS)62 |
| Splenic | 43 | Hypersplenism, infarct63 |
| Liver/gallbladder | 30 | Hepatomegaly,64 focal or diffuse hepatic lesions on imaging,64 chronic active hepatitis,65 hepatic necrosis,66 Budd-Chiari syndrome,67 sclerosing cholangitis,68 cholecystitis,69 cholestasis70 |
| Ocular | 23 | Microthrombi,71-73 choroidal infarcts,72 retinal arteritis,73 episcleritis,74 keratoconjunctivitis sicca,74 Adie syndrome (pupillotonia)75 |
| Gastrointestinal | 23 | Ascites,76 diarrhea,77 gastritis,78 colitis,69,78 pancreatitis77 |
| Musculoskeletal | N/A | Arthritis,79,80 effusions,80 bursitis,81 synovitis,82 Raynaud phenomena,83 digital necrosis,84 polymyositis/myopathy85,86 |
| Renal | N/A | Acute renal failure with Charcot-Leyden crystalluria,87 nephrotic syndrome,88 immunotactoid glomerulopathy,89 crescentic glomerulonephritis90 |
Organ system . | Cumulative frequency from 3 studies, %* . | Examples of organ-specific manifestations . |
|---|---|---|
| Hematologic | 100 | Leukocytosis with eosinophilia; neutrophilia, basophilia, myeloid immaturity, immature and/or dysplastic eosinophils; anemia, thrombocytopenia or thrombocytosis, increased marrow blasts, myelofibrosis19,21 |
| Cardiovascular | 58 | Cardiomyopathy,28,29 constrictive pericarditis,30,31 endomyocarditis,32,33 mural thrombi,27,34 valvular dysfunction,27,35,36 endomyocardial fibrosis,37,38 myocardial infarction39 |
| Dermatologic | 56 | Angioedema,40 urticaria,40 papules/nodules,40 plaques,41 aquagenic pruritis,42 erythroderma,43 mucosal ulcers,44 vesicobullous lesions,45 microthrombi,46,47 vasculitis,48 Wells syndrome49 |
| Neurologic | 54 | Thromboembolism,50 peripheral neuropathy,50,51 encephalopathy,50,52 dementia,53,54 epilepsy,55 cerebellar disease,56 eosinophilic meningitis57 |
| Pulmonary | 49 | Pulmonary infiltrates,9,58 effusions,9,59 fibrosis,4 emboli,60 nodules/focal ground glass attenuation,61 acute respiratory distress syndrome (ARDS)62 |
| Splenic | 43 | Hypersplenism, infarct63 |
| Liver/gallbladder | 30 | Hepatomegaly,64 focal or diffuse hepatic lesions on imaging,64 chronic active hepatitis,65 hepatic necrosis,66 Budd-Chiari syndrome,67 sclerosing cholangitis,68 cholecystitis,69 cholestasis70 |
| Ocular | 23 | Microthrombi,71-73 choroidal infarcts,72 retinal arteritis,73 episcleritis,74 keratoconjunctivitis sicca,74 Adie syndrome (pupillotonia)75 |
| Gastrointestinal | 23 | Ascites,76 diarrhea,77 gastritis,78 colitis,69,78 pancreatitis77 |
| Musculoskeletal | N/A | Arthritis,79,80 effusions,80 bursitis,81 synovitis,82 Raynaud phenomena,83 digital necrosis,84 polymyositis/myopathy85,86 |
| Renal | N/A | Acute renal failure with Charcot-Leyden crystalluria,87 nephrotic syndrome,88 immunotactoid glomerulopathy,89 crescentic glomerulonephritis90 |
Modified from Weller and Bubley21 and from Brito-Babapulle17 with permission from Elsevier.
N/A indicates not available.
Data are taken from Fauci et al,4 Spry et al,19 and Lefebure et al.20