Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial Plasmacytoma

Area of Science:

• Neurology
• Hematology
• Oncology

Background:

• Intracranial plasmacytomas are rare central nervous system tumors.
• Acquired von Willebrand syndrome (VWS) is an uncommon bleeding disorder.

Observation:

• A patient presented with symptoms necessitating a stereotaxic intracerebral biopsy for plasmacytoma diagnosis.
• Abnormal bleeding occurred post-biopsy during ventriculoperitoneal shunt placement.
• Hemostasis assessment identified acquired von Willebrand disease.

Findings:

• This is the first reported case of acquired VWS associated with intracranial plasmacytoma.
• The patient achieved a very good partial response with no neurological symptoms after 4 months of maintenance therapy.
• The patient remains progression-free 14 months post-presentation.

Implications:

• Highlights a rare but significant association between intracranial plasmacytoma and acquired VWS.
• Demonstrates the efficacy of a multi-modal treatment approach including VTD chemotherapy, stem cell transplantation, and maintenance therapy.
• Suggests the importance of hemostasis assessment in patients with central nervous system tumors presenting with bleeding complications.