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Waldenström Macroglobulinemia
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Extramedullary infiltration by LPL cells commonly results in lymphadenopathy and splenomegaly, which are seen in approximately 20%–25% of patients [57]. While LPL cells can infiltrate other organs, such as kidney and lung, such manifestations are unusual and are seen in less than 4% of patients. Unlike MM, osteolytic lesions are rare (<2%) and raise the suspicion for an alternative diagnosis, such as IgM MM [57]. Bing−Neel syndrome, a rare complication of WM, is caused by the direct invasion of the central nervous system by the LPL cells [63,68]. Schnitzler syndrome, an uncommon disease characterized by an IgM monoclonal gammopathy, chronic urticarial rash, and inflammatory symptoms, has been described in association with WM [69]. Upregulation of the IL-1 pathway plays a pivotal role in the pathophysiology of Schnitzler syndrome [70,71].
Rheology of Paraproteinemias and Leukemias
Published in Gordon D. O. Lowe, Clinical Blood Rheology, 2019
These are also common in HVS and include headaches, dizziness, vertigo, visual disturbances (e.g., diplopia), nystagmus, ataxia, deafness, tinnitus, psychiatric syndromes, pyramidal lesions, myelopathy, peripheral neuropathy (Bing-Neel syndrome), myopathy, drowsiness, seizures, and coma.8 Fatal cerebral hemorrhage may occur. As with retinopathy, RSV is usually greater than 4.0 in patients with neurological symptoms. Again, however, there is much interindividual variation in the “symptomatic threshold” of hyperviscosity.3,8,31 Blood viscosity, measured at a low shear rate, also correlated with neurological symptoms.24 One patient with WM had a fatal neurological syndrome with a relatively low RSV (3.6) but high calculated high-shear blood viscosity (13.2 mPa sec).32 Plasmapheresis again appears rapidly effective in treatment of neurological features.3,8,31
Zanubrutinib for the treatment of adults with Waldenstrom macroglobulinemia
Published in Expert Review of Anticancer Therapy, 2022
Shayna Sarosiek, David Sermer, Andrew R. Branagan, Steven P. Treon, Jorge J. Castillo
Future studies should focus on inducing durable deeper responses, and hopefully attain complete responses, with fixed-duration regimens maximizing efficacy while decreasing long-term toxicity and cost. Potential next steps could include the combination of zanubrutinib with proteasome inhibitors or alkylating agents with and without anti-CD20 monoclonal antibodies. As examples, prospective studies evaluating ibrutinib in combination with bortezomib and rituximab (NCT03620903), carfilzomib (NCT04263480), and ixazomib (NCT03506373) are underway in Europe and the United States. A Canadian study will evaluate the combination of six cycles of bendamustine and rituximab and 1-year course of acalabrutinib (NCT04624906). Other combinations of interest include ibrutinib and daratumumab (NCT03679624), ibrutinib and venetoclax (NCT04273139), and ibrutinib, venetoclax and rituximab (NCT04840602). There is also a dearth of data available for the treatment of patients with WM involving the central nervous system, known as Bing-Neel syndrome. The current standard of care in these patients is treatment with ibrutinib and other chemotherapy agents that penetrate the blood–brain barrier. Zanubrutinib may offer an effective therapeutic option in this setting with an improved side effect profile, but at this time only one published case report is available demonstrating the efficacy in this setting [36]. Future studies will hopefully determine the role of zanubrutinib in this patient population.
Zanubrutinib for the treatment of Waldenström Macroglobulinemia
Published in Expert Review of Hematology, 2020
Kenneth J. C. Lim, Constantine S. Tam
Bing Neel Syndrome (BNS) is a rare complication of WM affecting about 1% of patients. It occurs when WM is detected in the CNS causing a diverse range of neurological symptoms and diagnosed through the detection of a lymphoplasmacytic infiltrate on histological biopsy of the cerebrum or meninges. This is in conjunction with the detection of a monoclonal population of B-cells and the presence of MYD88L265P mutation in the cerebrospinal fluid and presence of radiological abnormalities on MRI of the brain and spinal cord as outlined by the guidelines by Minnema et al [42]. There is currently no consensus on the treatment of BNS, although the common treatment principle involves using agents with good CNS penetration such as systemic fludarabine, methotrexate, and cytarabine with intrathecal chemotherapy and Rituximab used as adjuncts. Subsequently, a retrospective study found that these agents yielded an ORR of 70% when used as first-line therapy [43]. However, these agents also carried an increased risk of immunosuppression and myelosuppression. Renal toxicity and mucositis was also seen with Methotrexate, cerebellar toxicity with cytarabine and secondary myeloid neoplasms and persistent cytopenias with Fludarabine. The prognosis of BNS remains undefined with a 5 year OS of 70% and a 3 years OS of 60% found in two separate retrospective studies [21,44,45]. However, it was noted that prognosis was significantly poorer in previously treated patients compared to patients who were treatment naïve (3 year OS 40% vs 100%).
Managing complications secondary to Waldenström’s macroglobulinemia
Published in Expert Review of Hematology, 2021
Ilias Pessach, Meletios A. Dimopoulos, Efstathios Kastritis
Bing–Neel syndrome (BNS) is a rare neurologic syndrome, seen in approximately 1% of WM patients, and is associated with substantial morbidity and mortality. BNS results from infiltration of the central nervous system (CNS) by lymphoplasmacytic cells. BNS may present with subtle symptoms from CNS or multiple cranial nerves, may develop in previously asymptomatic patients and may also occur without evidence of systemic disease recurrence and should be suspected in all WM patients presenting with unexplained and persistent neurologic symptoms [29,30].