When Tissue Becomes the Issue: A Lesson on Atypical Complications of Waldenström Macroglobulinemia Kimberly E. Chesteen, MD, Alan J. Hunter, MD, FACP Oregon Health & Science University, Portland OR

[ ] Introduction Case Data Discussion Waldenström macroglobulinemia (WM) Pertinent Studies: • Unlike multiple myeloma, there is no frequentlyDevelopmentpresents of a Newwith Practicepancytopenia Model clear association between placing patients at risk of infection and Chemistry & Other Hematology Waldenström macroglobulinemia and bleeding. However, other less common Basic metabolic panel normal Leukocytes 2820/mL plasmacytoma. However infiltrative complications can occur with similar Total protein 13.2 g/dL Neutrophils 1480/mL extramedullary WM may present on presentations. This case highlights an Albumin 2.1 g/dL Hemoglobin 7.9 g/dL imaging as an infiltrative mass with apparent case of osteomyelitis and Platelets 119,000/mL pathology resembling bleeding diathesis, that with a systematic IgM >5850 mg/dL INR 1.17 plasmacytoma.3 diagnostic approach was identified as: vWF antigen low aPTT 56.7 sec. (1) plasmacytoma-like infiltrative vWF activity low (normalized with mixing study) • It is estimated extramedullary disease extramedullary WM and (2) acquired (ristocetin cofactor) Fibrinogen 95 mg/dL is only present in 4.4% of WM cases von Willebrand syndrome (aVWS) in a Factor VIII low ESR >120 mm/hr with the common locations involving patient with progressive WM. Viscosity 8.72 centipoises (high) the lungs, CSF, soft tissue, and bone.3 Cultures: Case Description • Blood & vertebral cultures: no growth • There is a clear association between References A [ 73] -year-old man with Waldenström Imaging (Lumbar MRI): Representative Pathology: 1. [ ] acquired von Willebrand syndrome macroglobulinemia, pancytopenia, and (aVWS) and monoclonal peripheral neuropathy presented with gammopathies. A retrospective study progressive dorsalgia, epistaxis, and 30- shows an association between pound weight loss with initial imaging increasing IgM levels and risk of concerning for lumbar osteomyelitis. He aVWS.1 reported no fever, headaches, vision changes, or weakness, and had no Teaching Points neurologic deficits on exam except for decreased peripheral pinprick sensation. 1. Importance of evaluating coagulation disorders in patients Studies demonstrated hyperviscosity and with mild thrombocytopenia & new an acquired von Willebrand syndrome bleeding

(shown on right). Repeat MRI (shown MRI T1 post-gadolinium of lumbar spine showing Example microscopy of a plasmacytoma in a 2. Benefits of adhering to a systematic on right) remained concerning for abnormal T1 hypo-intensity and post-contrast patient with multiple myeloma with plasma cell diagnostic approach and enhancement in L4-L5 vertebral bodies and L5 pedicles infiltration and osteolytic destruction2 osteomyelitis. Infection continued to be with abnormal thickening and enhancement of epidural maintaining a broad differential the most likely etiology, but a biopsy soft tissues at L5-S1, ventral more than dorsal. L4-5 high diagnosis grade stenosis. Compression fracture of L5 vertebral body. was pursued to guide antimicrobial Abnormal post-contrast enhancement involving medial 3. Reinforces the importance of tissue selection, and to provide diagnostic portion of bilateral psoas muscle without abscess. biopsy to not only guide antibiotic confirmation. Given the patient’s Pathology: selection, but assure the correct hyperviscocity syndrome and aVWS, his • L4-5 vertebral biopsy: hypo-cellular marrow with lambda light chain diagnosis. surgical biopsy was delayed one week producing plasma cells. References while he required plasmapheresis with 1. Castillo, J., et al. (2017). Acquired Von Willebrand cryoglobulin and VWF/FVIII Proposed Mechanisms for Acquired von-Willebrand Syndrome Disease in Patients with Waldenström concentrates (Humate-P®). Vertebral in Waldenström Macroglobulinemia1 Macroglobulinemia. Blood, 130(Supp 1), 1088. biopsy revealed clusters of plasma cells 2. Crane, G.M. Plasmacytoma. • Phagocytosis of von Willebrand factor by plasma cells PathologyOutlines.com website. indicative of infiltrative extramedullary http://www.pathologyoutlines.com/topic/lympho WM. He was initiated on therapy for • Plasma cells make antibodies against vWF maplasmacytoma.html. Accessed October 25th, WM with bortezomib and 2018 dexamethasone. Rituximab was held due 3. Banwait, R., et al. (2015). Extramedullary • Shear force from increased viscosity Waldenström macroglobulinemia. American to elevated IgM. Journal of Hematology, 90(2), 100-104.