Prognosis of hyperviscosity syndrome in newly diagnosed multiple myeloma in modern-era therapy: A real-life study

Abstract

Hyperviscosity syndrome (HVS) is a rare complication of newly diagnosed multiple myeloma (NDMM) related to high tumour burden. Studies about the prognosis of HVS in modern-era therapy for NDMM are missing. We investigated a retrospective cohort study of NDMM with HVS between 2011-2021. Thirty-nine NDMM patients with HVS were included. HVS presentation was heterogeneous, with asymptomatic, mild, and neurological forms in 23%, 59%, and 18% of cases, respectively. No thrombosis or major bleeding was observed. Therapeutic plasma exchanges were used in 92% of patients, which were effective and well tolerated. No rebound effect was observed. All patients except one had at least one CRAB criterion. Most of the patients received bortezomib and high-dose steroids (95%) associated with an immunomodulatory drug (43%) or alkylating agents (42%). HVS in NDMM patients had dismal overall survival matched to multiple myeloma patient controls (without HVS) in our center (median: 3.6 vs. 7.7 years, p=0.01), as confirmed by multivariate analysis. Early deaths (in the first two months) occurred in 21% of older patients (>65 years). HVS in NDMM patients is a rare but life-threatening complication associated with high lethality in older patients and be a potential dismal prognosis factor in the modern treatment era.

Management of hematological patients requiring emergency chemotherapy in the intensive care unit

“…Due to their pentameric structure and their high molecular weight, IgMs are more likely to induce HVS, which explains the importance of Waldenström macroglobulinemia (WM) among etiologies [ 71 , 72 ]. At diagnosis, 40% of WM patients present with HVS, compared to 3.4% of multiple myeloma (MM) patients [ 72 , 73 ]. Notably, the presence of a cryoglobulin can trigger or worsen plasma hyperviscosity, due to the cold-induced polymerization of the immunoglobulin involved.…”

“…Clinical signs of plasma hyperviscosity are dominated by a triad [ 74 ], associating neurological abnormalities (headache, delirium, tinnitus, ataxia), visual disturbances (blurring, phosphenes, myodesopsia), and mucosal bleeding (epistaxis, gum). In a context of HVS suspicion or for asymptomatic patients with a high protein concentration, the diagnosis can be rapidly confirmed by ophthalmologic examination revealing delayed venule clearance and/or retinal hemorrhage [ 72 , 73 , 75 ]. Beyond major hyperproteinemia, serum protein electrophoresis reveals the monoclonal hypergammaglobulinemia, characterized by immunofixation, with levels of IgM, IgG, and IgA generally greater than 30, 40, and 60 g/L, respectively [ 71 ].…”

Multiple Myeloma with Concurrent Pericardial Effusion

“… 9 Hyperviscosity can also be an issue due to the altered makeup of the vascular environment with some patients ending up with pulmonary emboli or ischemic strokes. 10 Hypercalcemia symptoms, such as constipation, nausea, vomiting, psychosis, and increased thirst and urination, also can manifest after the lytic bone process has taken place. While the patient manifested some of the above symptomatology later in her hospital stay, her only complaint upon admission to the ED was radiating upper abdominal pain, which was an unusual disease presentation.…”

Changes of myocardial dysfunction and injury biomarkers over chemotherapy for multiple myeloma: difficulties in laboratory data interpretation

“…Наряду с электролитными нарушениями и анемией, парапротеинемия представляется одним из важных факторов повреждающего механизма ММ в отношении ССС. Циркуляция в сыворотке крови большого количества патологических иммуноглобулинов приводит не только к нарушению межбелковых взаимодействий, но и к синдрому повышенной вязкости, который характеризуется триадой признаков: геморрагический синдром, нарушения со стороны органа зрения и неврологические дефициты [7][8]. В отдельных случаях гипервискозный синдром может приводить к жизнеугрожающим событиям, например, тромбозам, в т.ч.…”