Pedigree and haplotypes of patient families. Squares and circles indicate males and females, respectively. Closed circles and squares with arrows with a P indicate probands. The half-closed circles and squares represent asymptomatic carriers. The cross indicates deceased. The ADAMTS13 activity is shown as a percentage of the normal control. ND indicates not determined. Mutations found in the ADAMTS13 gene are shown as one-letter amino acid abbreviations numbered from the initial Met or as nucleotides numbered from the A of the translation initiation Met codon.