PT  - JOURNAL ARTICLE
AU  - Isabel Dorn
AU  - Ulrich Budde
AU  - Michael C Frühwald
AU  - Monika Pöppelmann
AU  - Ulrike Nowak-Göttl
TI  - Acquired von Willebrand syndrome in a 10-year-old girl with acute lymphoblastic leukaemia
AID  - 10.1136/bcr.04.2009.1816
DP  - 2009 Jan 01
TA  - BMJ Case Reports
PG  - bcr0420091816
VI  - 2009
4099  - http://casereports.bmj.com/content/2009/bcr.04.2009.1816.short
4100  - http://casereports.bmj.com/content/2009/bcr.04.2009.1816.full
AB  - Following diagnosis of acute lymphoblastic leukaemia (ALL) in a 10-year-old girl, routine coagulation screening including von Willebrand factor antigen (VWF:Ag), ristocetin cofactor activity (VWF:RCo) and factor VIIIC (FVIII:C) detected no pathological findings. After the first HR2′ element of the high-risk group of the ALL-BFM 2000 protocol, the patient demonstrated extensive bleeding symptoms and acquired von Willebrand syndrome was diagnosed. VWF:Ag (13%), VWF:RCo (13%) and FVIII:C (27%) were decreased. Multimer analysis showed a loss of large multimers and a loss in triplet structures. The observed pattern was thought to be typical for monoclonal IgG gammopathy; however, in this case, unexpectedly, biclonal IgM gammopathy (κ and λ) was detected. After treatment with intravenous immunoglobulin over 5 days, coagulation factors increased to normal levels. Although this effect was assumed to be at best only temporary, especially in a case of IgM gammopathy, no further bleeding symptoms have been observed. Trial registration number: M208