Hyperviscosity Syndrome in Paraprotein Secreting Conditions Including Waldenstrom Macroglobulinemia

Weaver, Allison; Rubinstein, Samuel M.; Cornell, Robert F.

doi:10.3389/fonc.2020.00815

Cited by 27 publications

(20 citation statements)

References 34 publications

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“…There is a high degree of clinical overlap between BNS and other WM complications, and even experienced clinicians struggle to differentiate them. As an example, we discussed a patient (case 2) who presented with blurred vision and a headache, both of which could have been caused by hyperviscosity, a condition associated with WM ( 16 ). Notably, in one patient (case 1), the median and ulnar nerve impairments were the most prominent symptoms at BNS onset, indicating the presence of mononeuropathy or peripheral neuropathies that can be seen in WM.…”

Section: Discussionmentioning

confidence: 99%

Bing-Neel Syndrome: Real-Life Experience in Personalized Diagnostic Approach and Treatment

et al. 2022

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The involvement of the central nervous system (CNS) in Waldenström’s Macroglobulinemia (WM) is a rare extramedullary manifestation of the disease known as Bing-Neel syndrome (BNS). To expand our understanding of this disease manifestation, we conducted a retrospective analysis of the incidence of BNS in 86 consecutive patients with WM [70% male, median age 65 years (range 33-86)] seen in our center during a 30-year period. Six patients (7%) from this group were diagnosed with BNS. The median period of time between WM diagnosis and BNS diagnosis was 6.8 years (range 2.3-15). They demonstrated a range of neurological deficits, including transient expressive aphasia, impaired vision, resting hand tremor, foot drop, and headache. Between the onset of symptoms and the diagnosis of BNS, the median time interval was 12.5 months (range 1-30). The diagnosis was made not on the basis of neurological symptoms or radiological evidence, but on the basis of the presence of WM cells in cerebrospinal fluid (CSF). Intrathecal chemotherapy with methotrexate, cytarabine, and dexamethasone (IT MTX, ARA-C, DEX) was used as front-line treatment, followed by intensive immunochemotherapy with rituximab, high-dose MTX, and ARA-C (R-Hi MTX/ARA-C) in three patients who were fit enough to receive this type of cytotoxic regimen, and rituximab plus bendamustine (R-Benda) in two patients who simultaneously required treatment for WM. Ibrutinib was administered to five patients (three as consolidation and two for initial treatment). All patients responded to front-line treatment, with four (67%) achieving partial response (PR) and two (33%) achieving complete response (CR). This study provides insight into the clinical presentation, diagnostic and treatment options, as well as the outcome of patients who have BNS.

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Section: Discussionmentioning

confidence: 99%

Bing-Neel Syndrome: Real-Life Experience in Personalized Diagnostic Approach and Treatment

et al. 2022

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“…An increase in blood viscosity leads to the development of hyperviscosity syndrome. Of note, hyperviscosity syndrome is developed in different hematological disorders, including multiple myeloma, sickle cell anemia, Waldenstorm macroglobulinemia, polycythemia, and leukemia (21,22). Normal BV is usually between 1.4 and 1.8 centipoise (cp), and symptoms of hyperviscosity syndrome develop when blood viscosity exceeds 4.0 cp (23).…”

Section: Blood Viscosity and Hyperviscosity Syndromementioning

confidence: 99%

“…Normal BV is usually between 1.4 and 1.8 centipoise (cp), and symptoms of hyperviscosity syndrome develop when blood viscosity exceeds 4.0 cp (23). Patients with hyperviscosity syndrome are presented with diving symptoms due to impairment of blood flow, including headache, confusion, visual disturbances, vertigo, and thrombotic events with or without mucosal hemorrhage (21,22). Sloop and colleagues found that inflammation and hypergammaglobulinemia together with the fostering of erythrocyte aggregation in sepsis could be the potential mechanisms of increasing blood viscosity in different infectious diseases (24).…”

Section: Blood Viscosity and Hyperviscosity Syndromementioning

confidence: 99%

Changes in the Blood Viscosity in Patients With SARS-CoV-2 Infection

et al. 2022

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Coronavirus disease 2019 (COVID-19) is caused by a novel virus known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). SARS-CoV-2-induced hyperinflammation together with alteration of plasma proteins, erythrocyte deformability, and platelet activation, may affect blood viscosity. Thus, this review aimed to study the link between SARS-CoV-2 infection and alteration of blood viscosity in COVID-19 patients. In order to review findings related to hyperviscosity in COVID-19, we suggested a protocol for narrative review of related published COVID-19 articles. Hyperviscosity syndrome is developed in different hematological disorders including multiple myeloma, sickle cell anemia, Waldenstorm macroglobulinemia, polycythemia, and leukemia. In COVID-19, SARS-CoV-2 may affect erythrocyte morphology via binding of membrane cluster of differentiation 147 (CD147) receptors, and B and 3 proteins on the erythrocyte membrane. Variations in erythrocyte fragility and deformability with endothelial dysfunction and oxidative stress in SARS-CoV-2 infection may cause hyperviscosity syndrome in COVID-19. Of interest, hyperviscosity syndrome in COVID-19 may cause poor tissue perfusion, peripheral vascular resistance, and thrombosis. Most of the COVID-19 patients with a blood viscosity more than 3.5 cp may develop coagulation disorders. Of interest, hyperviscosity syndrome is more commonly developed in vaccine recipients who had formerly received the COVID-19 vaccine due to higher underlying immunoglobulin concentrations, and only infrequently in those who have not received the COVID-19 vaccine. Taken together, these observations are untimely too early to give a final connotation between COVID-19 vaccination and the risk for development of hyperviscosity syndrome, consequently prospective and retrospective studies are necessary in this regard.

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“…Higher IgM levels (>4000 mg/dl) and/or serum viscosity >4 centipoise (normal value-1.5) are usually associated with these symptoms. 20 A classic fundoscopic finding in WM associated with hyperviscosity is the presence of dilated, segmented, and tortuous retinal veins. Hemorrhages, exudates, and papilledema can also be seen.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Differential Diagnosis of Waldenström’s Macroglobulinemia and Early Management: Perspectives from Clinical Practice

Cingam¹,

Sidana²

2022

BLCTT

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Waldenström’s Macroglobulinemia (WM) is a clonal B-lymphocyte neoplasm characterized by the presence of IgM monoclonal protein and ≥10% bone marrow involvement with lymphoplasmacytic cells. Several mature B-cell and plasma cell disorders can potentially produce monoclonal IgM immunoglobulin and hence, careful consideration of the differential diagnosis is vital. Clinico-pathological features, immunophenotype, and MYD88 mutation status help distinguish WM from other plasma cell and lymphoproliferative disorders. Treatment is only indicated in patients symptomatic from adenopathy or organomegaly, neuropathy, hyper viscosity, cryoglobulinemia, cold agglutinin disease, cytopenia’s or amyloidosis. Alkylators (cyclophosphamide, bendamustine) in combination with anti-CD20 antibodies and novel targeted agents including Bruton tyrosine kinase (BTK) inhibitors like ibrutinib are the mainstay of frontline treatment in symptomatic WM.

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Hyperviscosity Syndrome in Paraprotein Secreting Conditions Including Waldenstrom Macroglobulinemia

Cited by 27 publications

References 34 publications

Bing-Neel Syndrome: Real-Life Experience in Personalized Diagnostic Approach and Treatment

Bing-Neel Syndrome: Real-Life Experience in Personalized Diagnostic Approach and Treatment

Changes in the Blood Viscosity in Patients With SARS-CoV-2 Infection

Differential Diagnosis of Waldenström’s Macroglobulinemia and Early Management: Perspectives from Clinical Practice

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