Table 1. ECD main organs involvement in... | American Society of Hematology

Table 1.

ECD main organs involvement in the current series from Pitié-Salpêtrière Hospital (n = 261) and the National Institutes of Health cohort (n = 60) compared with the review from the literature

Organ infiltration, clinical and mutational findings	Pitié-Salpêtrière (current series; n = 261)	Estrada-Veras et al (National Institutes of Health; n = 60)¹²	Cives et al (review of literature; n = 448)¹⁵
Long bone (metabolic, bone scintigraphy or PET), %	79	95	74
Bone pain, %	38	—	—
Cardiac involvement, %	51	—	19
Right atrium pseudotumor, %	36	37	11
Coronary infiltration, %	23	—	—
Pericardial involvement, %	29	8	9
Vascular involvement, %	61	—	16
Coated aorta, %	40	62	—
Xanthelasma, %	22	33	27
Diabetes insipidus, %	25	47	48
CNS involvement, %	37	38	25-50
Cerebellar involvement, %	16	—	—
Retro-orbital involvement, %	20	27	35
Lung involvement, %	36	52	18
Retroperitoneal involvement (hairy kidney aspect), %	63	65	35
Hydronephrosis, %	27	—	6

Organ infiltration, clinical and mutational findings	Pitié-Salpêtrière (current series; n = 261)	Estrada-Veras et al (National Institutes of Health; n = 60)¹²	Cives et al (review of literature; n = 448)¹⁵
Long bone (metabolic, bone scintigraphy or PET), %	79	95	74
Bone pain, %	38	—	—
Cardiac involvement, %	51	—	19
Right atrium pseudotumor, %	36	37	11
Coronary infiltration, %	23	—	—
Pericardial involvement, %	29	8	9
Vascular involvement, %	61	—	16
Coated aorta, %	40	62	—
Xanthelasma, %	22	33	27
Diabetes insipidus, %	25	47	48
CNS involvement, %	37	38	25-50
Cerebellar involvement, %	16	—	—
Retro-orbital involvement, %	20	27	35
Lung involvement, %	36	52	18
Retroperitoneal involvement (hairy kidney aspect), %	63	65	35
Hydronephrosis, %	27	—	6