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Article Figures & Data

Figures

  • Figure 1.

    Pedigree of the QPD family. Men (squares) and women (circles) in 7 generations are shown. Solid symbols indicate affected participants (n = 23). Symbols containing an X indicate additional individuals known or presumed to have QPD on the basis of results of previous testing (n = 6), a history of typical QPD bleeding problems, or documented QPD transmission to offspring. Open symbols containing dots indicate unaffected individuals who participated in the study (n = 104). Open symbols without dots indicate other known or presumed unaffected family members. Partners are shown for women who had children with several individuals. Children of unknown sex are shown as diamonds (representing known or unknown numbers of individuals, as indicated).

Tables

  • Table 1.

    Bleeding symptoms in members of the family with QPD

    % (proportion) responding with the symptom
    SymptomAUOR95% CIScore
    Blood relatives with bleeding problems 100 (23/23) 24 (24/98) 137 13-1000 Excluded
    Had bleeding problems that led to a change in lifestyle 60 (12/20) 0 (0/9) α 1.9-α 1
    Abundant bruises or bleeding 57 (13/23) 26 (27/104) 3.7 1.3-11 1
    Bruising
        Without reason 55 (12/22) 34 (34/99) 2.0 0.8-6.5
        Disproportionate to trauma 39 (9/23) 12 (12/100) 4.7 1.5-15 1
        Noticed by other people 4 (1/23) 1 (1/99) 4.5 0.1-170
        Painful 22 (5/23) 6 (6/99) 4.3 1.0-18
        That lasts a long time 22 (5/23) 10 (10/99) 2.5 0.6-9.2
        That spreads toward feet or size of an orange or larger 32 (7/23) 0 (0/99) α 4.6-α 1
    Experienced deep cut(s) 70 (16/23) 59 (61/104) 1.6 0.6-4.8
        Bleeding with this cut continued for days 56 (9/16) 3 (2/60) 37 5.6-320 1
    Get nosebleeds 57 (13/23) 24 (25/103) 4.0 1.4-12 1
    Have had nosebleeds
        Lasting longer than 15 min 77 (10/13) 56 (14/25) 2.6 0.5-16
        That required nasal packing 15 (2/13) 12 (3/25) 1.3 0.1-12
        That required cautery 24 (3/13) 24 (6/25) 1.0 0.2-6.6
        That required transfusion 8 (1/13) 0 (0/25) α 0.03-α
        That required hospitalization 17 (2/12) 0 (0/25) α 0.3-α
    Hematuria 50 (11/22) 12 (12/104) 7.7 2.4-25 1
    Joint bleeds 43 (10/23) 0 (0/104) α 7.4-α 1
    Had dental extractions 74 (17/23) 74 (77/104) 1.0 0.3-3.1
        With abnormal bleeding 94 (16/17) 35 (26/75) 30 3.8-644 1
        Bleeding for more than 24 h afterward 94 (16/17) 8 (6/72) 176 18-4250 1
    Gum problems 14 (3/21) 12 (13/104) 1.2 0.2-5.1
    Bleeding problems with surgery(ies) 50 (9/18) 9 (7/79) 10 2.7-41 1
        That required transfusions 38 (5/13) 3 (2/70) 21 2.9-195 *
        That required other treatments for bleeding 38 (5/13) 3 (2/71) 22 2.9-198 1
    Had a serious accident 65 (15/23) 38 (39/104) 3.1 1.0-9.0
        Bled excessively afterward 86 (12/14) 39 (15/38) 9.2 1.6-70 1
        Bleeding right away 79 (11/14) 57 (17/30) 2.8 0.5-16
        Bleeding started 12 h later 8 (1/12) 0 (0/30) α 0.04-α
        Needed to be hospitalized 67 (10/15) 26 (10/39) 5.8 1.4-26 1
        Became anemic after accident 15 (2/13) 7 (2/30) 3.0 0.2-30
        Needed transfusion after accident 31 (4/13) 7 (2/30) 6.0 0.8-60
        Developed wound infection 8 (1/13) 0 (0/30) α 0.03-α
    Problems healing after an injury 26 (6/23) 7 (7/104) 4.9 1.3-19 1
    History of transfusions 52 (12/23) 10 (10/100) 9.8 3.1-32 1*
    Other treatments recommended for bleeding 87 (20/23) 2 (2/90) 293 38-3370 1
    • Odds ratios (ORs) and 95% confidence intervals (CIs) compare affected (A) and unaffected (U) family members.

      — indicates items not included in bleeding scores; α, OR estimated as infinite.

    • * Overlapping queries, scored once as indicated.

  • Table 2.

    Sex-specific bleeding symptoms in the family with QPD

    % (proportion) responding with the symptom
    SymptomAUOR95% CIScore
    Men
        Circumcised 25 (4/16) 28 (10/36) 0.9 0.2-3.9
        Abnormal bleeding when circumcised 33 (1/3) 0 (0/8) α 0.03-α
    Women
        Abundant menstrual bleeding 50 (3/6) 53 (33/62) 1.0 0.1-6.0
        Menses lasting longer than 7 d 50 (3/6) 7 (4/61) 14 1.6-147 1
        Blood-soiled garments with menses 50 (3/6) 45 (28/62) 1.0 0.2-8.3
        Double protection used with menses 50 (3/6) 18 (11/61) 5.0 0.6-34
        Hysterectomy to control menses 17 (1/6) 5 (3/63) 4.0 0.1-63
        Been pregnant 71 (5/7) 60 (38/63) 2.0 0.2-13
        Transfused when gave birth 40 (2/5) 5 (2/39) 12 0.8-225
    All subjects
        Mother had/has abundant menstrual bleeding 50 (7/14) 32 (20/63) 2.0 0.6-8.1
        Sister(s) had/has abundant menstrual bleeding 57 (4/7) 51 (22/43) 1.0 0.2-8.4
    • OR and 95% CI compare affected (A) to unaffected (U) family members. — indicates items not included in bleeding scores.

  • Table 3.

    Cardiac disease, strokes, and thrombotic problems in the family with QPD

    % (proportion) responding with the problem
    ExperiencedUAOR95% CI
    Heart problems, heart attacks and/or angina 5 (5/104) 0 (0/23) α 0.1-α
    Thrombosis: DVT, PE, or both 2 (2/104) 4 (1/23) 0.4 0.03-13
    Stroke 1 (1/104) 4 (1/23) 0.2 0.01-8
    • OR and 95% CI compare unaffected (U) to affected (A) family members. DVT indicates deep vein thrombosis; and PE, pulmonary embolism.