Unusual association of diseases/symptoms
Acquired von Willebrand syndrome in a 10-year-old girl with acute lymphoblastic leukaemia
1 University Childrens’s Hospital Münster, General Pediatrics, Albert-Schweitzer-Strasse 33, Muenster, 48149, Germany
2 Aesculabor Hamburg, Haferweg 36, Hamburg, 22769, Germany
3 University Children’s Hospital Münster, Pediatric Hematology and Oncology, Albert-Schweitzer-Strasse 33, Muenster, 48149, Germany
Correspondence to:
Isabel Dorn, Isabel.Dorn{at}ukmuenster.de
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 (
and 
) 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
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Haematology (incl blood transfusion)
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