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Table 1 Severity scores for vascular anomalies

From: The impact of sirolimus therapy on lesion size, clinical symptoms, and quality of life of patients with lymphatic anomalies

  Score of 0 Score of 1 Score of 2 Score of 3 Score of 4 Score of 5 Score of 6
Bleeding/hemorrhage Low-risk organs (skin and mucosa) None Mild without treatment Moderate without transfusion Severe, transfusion indicated Very severe with hemodynamic instability   Fatal bleeding or death
High-risk organs (gastrointestinal tract and lungs) None   Mild without treatment   Moderate without transfusion Severe, transfusion indicated Very severe or death
Thoracic lesions Respiratory None   Mild symptoms without treatment (PaO2 ≥ 80 Torr)   Chronic continuous symptoms, oxygen administration required (PaO2 ≥ 70 and < 80 Torr) Severe symptoms, continuous drainage and surgery required (PaO2 ≥ 60 and < 70 Torr) Very severe (PaO2 < 60 Torr, spO2 < 90%) or death
r Cardiac None Very mildly symptomatic Mildly symptomatic without treatment Moderate, treatment required Moderate or severe, oxygen administration required (EF < 50%) Severe, with continuous intervention or surgery required, or heart failure (EF < 40%) Very severe, fatal, or death
Abdominal lesions   None Very mildly symptomatic Moderately symptomatic without treatment Severe, treatment required Very severe with continuous intervention or surgery   Fatal or death
Bone lesions   None Very mildly symptomatic Moderately symptomatic without treatment Severe, treatment required Very severe with continuous intervention or surgery   Fatal or death
Cutaneous lesions   None Very mildly symptomatic Moderately symptomatic without treatment Severe, treatment required Very severe with continuous intervention or surgery   Fatal or death
Neurological symptoms   None Very mildly symptomatic Moderately symptomatic without treatment Severe, treatment required Very severe with continuous intervention or surgery   Fatal or death
Coagulation disorder and thrombocytopenia   Normal Mild coagulation disorder or thrombocytopenia (Plt ≤100 × 103/μl) Moderate coagulation disorder, low fibrinogen levels (≤100 mg/dl), or thrombocytopenia (Plt ≤50 × 103/μl) Severe coagulation disorder, low fibrinogen levels (≤100 mg/dl), and thrombocytopenia (Plt ≤50 × 103/μl); treatment required Very severe coagulation disorder, low fibrinogen levels (≤100 mg/dl) and thrombocytopenia (Plt ≤50 × 103/μl) with continuous intervention   Fatal or death
Nutritional status   Normal Decreased oral intake or mild body weight loss (≤3%) Moderately decreased oral intake or body weight loss (3–5%) Poor oral intake or body weight loss (≥5%), required treatment Very severe with continuous intervention   Fatal or death