Brigham and Women’s urgent care clinic

Wendy Turr - Attending physician
David Finkle - 2nd-year resident

Lucy Evergreen

62

unknown

- Persistent bleeding (5 hours) despite pressure and ice after periodontal scaling of her lower-right second molar
- Usual state of good health
- No easy bruising, epistaxis, rectal bleeding, hematuria, weakness, fatigue, light-headedness, fever, arthralgia, dyspnea, jaundice

- Similar episode 6 months previously after a periodontal procedure: bleeding only stopped after firm pressure for 6 hours
- Hypertension
- Deep-vein thrombosis in the legs 20 years before
- Cosmetic blepharoplasty without bleeding complications in her 20s
- Arthroscopic repair of meniscal tear in the left knee without bleeding complications one year before 
- Uncomplicated spontaneous vaginal delivery
- Quit smoking 30 years before
- Infrequent alcohol consumption
- No family history of bleeding diathesis
- Mother - 88 in good health
- Father died of lung cancer at 62
- Brother diagnosed with colorectal cancer at 57
- No allergies
- Treatment: thiazide diuretic
- Took aspirin 2 hours before the dental procedure

- Heart rate 80
- BP 128/76
- Slow oozing of blood near lower-right second molar
- No visible mucosal laceration, oral petechiae, bullae or ulcers
- Rest of the examination unremarkable.

- acquired von Willebrand’s disease
- liver disease
- thrombocytopenia
- uremia
- factor VIII inhibitor

- Blood tests
- VIII activity assay
- aPTT after mixing of the patient’s plasma and normal plasma in a 1:1 ratio
- Von Willebrand factor antigen level
- vWF multimer gel electrophoresis.
- ristocetin cofactor activity
- bone marrow biopsy
- quantitative immunoglobulin assays
- serum protein electrophoresis

- monocytes 13%
- hematocrit percentage at 31.8
- creatinine levels 1.4
- total protein levels 8.6
- activated partial-thromboplastin time at 49.6
- BUN, albumin, thyrotropin, platelet count, white-cell count, prothrombin time and MCV = normal
- reduced activity of factor VIII at 20%
- reduced ristocetin cofactor activity at 14%
- vWF at 22%
- loss of high-molecular-weight forms of vWF
- IgG kappa monoclonal gammopathy
- >10% monoclonal plasma cells (bone-marrow biopsy specimen)

Acquired von Willebrand’s disease due to IgG multiple myeloma

- chemotherapy with thalidomide and dexamethasone
- thalidomide as maintenance therapy

- IgG level, activated partial-thromboplastin time, factor VIII activity, level of vWF antigen, and ristocetin cofactor activity  normal within 4 months
- Slowly progressive increase in IgG levels over next 2 years
- No apparent recurrence of acquired vWD
- Additional periodontal work without bleeding complications