Demographic and Clinical Characteristics of Patients with KHE and
TA
Investigators from 17 institutions submitted data on 179 patients and
159 met full eligibility criteria after central review (Table 1). Of the
159 patients, 138 (86.8%) carried a diagnosis of KHE, 18 (11.3%) TA,
and 3 (1.9%) diagnosed with a combined KHE/TA spectrum. Gender, race,
and ethnicity were equivalent across groups. Sixty-four patients
(40.3%) had KMP at presentation. Patients with KMP were diagnosed (49.5
versus 293 days, p<0.001) and started treatment (48 versus 300
days, p<0.001) at a younger age than those without KMP. Biopsy
for pathologic diagnosis was more common in those without KMP compared
to those with KMP (91.6% versus 71.9%, p=0.002). Non-truncal lesions
were the most common location in all groups, but more common in those
without KMP (65.3% versus 50%, p=0.029). Tumor size was larger in the
individuals with KMP, and tumors associated with KMP were more likely to
involve more than one anatomic body area. Only 2 patients (1.3%) were
described to have multifocal tumors. Some patients without KMP were
determined to have coagulopathy (fibrinogen < 150mg/dL and/or
D-dimer > 2 times the upper limit of normal) at diagnosis,
but this was more common in the those with KMP (79.7% versus 14.7%,
p<0.001). Number of hospitalizations, hospital length of stay,
and ICU length of stay were all higher for individual with KMP (all
p<0.001). Only 2 patients (1.3%) in the cohort were deceased
and both had KMP, large tumors (≥ 5cm in size), and had tumor extension
across more than one body region. As expected, hematologic parameters
were more abnormal in patients with KMP, with lower hemoglobin nadir,
lower platelet count nadir, lower fibrinogen nadir, and higher maximum
prothrombin time and partial thromboplastin time (all p<0.001,
Supplemental Table 1).