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Hyperviscosity Syndrome- Myeloma
“Hyperviscosity syndrome (HVS) is a rare complication of newly diagnosed multiple myeloma (NDMM) related to high tumour burden…Among MM patients, 3–6% experienced this complication at diagnosis in cohorts prior to the 21st century (7, 8).”
Hyperviscosity syndrome is a rare symptom in newly diagnosed myeloma patients but if you, the patient is experiencing one of the symptoms below, this post may be helpful.
Pathophysiology
Hyper viscosity syndrome arises as a consequence of the overproduction of monoclonal immunoglobulins (M-proteins) by malignant plasma cells. In multiple myeloma, these cells proliferate uncontrollably within the bone marrow, crowding out normal hematopoietic cells. This excessive production of M-proteins, mainly immunoglobulin G (IgG) or immunoglobulin A (IgA), leads to a marked increase in serum viscosity. The large, irregularly-shaped M-proteins hinder the flow of blood through small vessels, particularly in high-shear areas like the retinal vessels and cerebral circulation. This ultimately results in compromised tissue perfusion, leading to the clinical manifestations of HVS.
Clinical Manifestations
The clinical presentation of hyperviscosity syndrome in multiple myeloma is diverse and can range from subtle symptoms to life-threatening complications. Common manifestations include:
- Neurological Symptoms: These are the most characteristic features of HVS. Patients may experience headaches, visual disturbances, dizziness, and altered mental status. Severe cases can lead to seizures, coma, or even stroke-like symptoms due to impaired cerebral blood flow.
- Ocular Symptoms: Retinal hemorrhages, dilated retinal veins, and papilledema may occur due to impaired blood flow in the retinal vessels.
- Bleeding Tendencies: Elevated viscosity can lead to impaired platelet function, resulting in epistaxis, gum bleeding, and easy bruising.
- Renal Dysfunction: Glomerular filtration may be compromised due to reduced perfusion, leading to acute kidney injury.
- Cardiac Symptoms: In rare cases, patients may present with congestive heart failure, angina, or arrhythmias due to increased afterload on the heart.
Diagnosis
The diagnosis of hyperviscosity syndrome in multiple myeloma relies on a combination of clinical suspicion, laboratory tests, and specialized assessments. Key diagnostic steps include:
- Serum Protein Electrophoresis (SPE): This test is crucial for identifying the presence and quantifying the monoclonal immunoglobulin (M-protein) responsible for the hyper viscosity.
- Viscosity Measurement: Serum viscosity levels should be measured, with a level >4 cP (centipoise) considered indicative of HVS.
- Complete Blood Count (CBC): Anemia, thrombocytopenia, and leukopenia may be present due to the crowded bone marrow.
- Peripheral Blood Smear: Examination of a blood smear may reveal rouleaux formation, a characteristic stacking of red blood cells, indicative of increased viscosity.
- Ophthalmologic Evaluation: Dilated fundus examination can identify retinal changes associated with HVS.
Management
The treatment of hyperviscosity syndrome in multiple myeloma necessitates a multi-faceted approach targeting both the underlying malignancy and the immediate symptoms:
- Plasmapheresis: This is the cornerstone of acute management. Plasmapheresis involves removing the excessive M-proteins from the blood, thereby reducing viscosity. It provides rapid relief of symptoms and can be life-saving.
- Chemotherapy and Immunomodulatory Agents: Targeted therapies such as proteasome inhibitors, immunomodulatory drugs, and monoclonal antibodies are employed to suppress the production of M-proteins, addressing the root cause.
- Supportive Care: Aggressive hydration, diuretics, and blood products may be necessary to maintain hemodynamic stability and correct any electrolyte imbalances.
- Ongoing Monitoring: Regular assessment of serum viscosity levels, complete blood counts, and serum protein electrophoresis is essential for tracking disease progression and response to treatment.
If you are a newly diagnosed myeloma patient, have you experienced headaches? Eye problems? Dizziness? Slurred speech? To learn more about various treatments feel free to reach out to me at David.PeopleBeatingCancer@gmail.com.
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Thanks,
David Emerson
- MM Survivor
- MM Cancer Coach
- Director PeopleBeatingCancer
Prognosis of hyperviscosity syndrome in newly diagnosed multiple myeloma in modern-era therapy: A real-life study
“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…
HVS results from a high blood viscosity due to an abnormal protein or cellular element increase, frequently secondary to plasma cell disorders with immunoglobulin (Ig) (4).
The clinical triad of HVS includes:
- mucosal bleeding (epistaxis, gum),
- visual abnormalities (blurring, papilledema, retinal bleeding, venule thrombosis), and
- neurological abnormalities (drowsiness, ataxia, cerebral bleeding) (4).
In a context of HVS suspicion or for asymptomatic patients with a high protein concentration, the diagnosis can be confirmed by ophthalmologic examination.
On one hand, the principal cause of HVS is pentameric IgM in the context of Waldenström macroglobulinemia (WM). At diagnosis, 40% of WM patients present with HVS, which is a criterion for starting specific treatment (5, 6). On the other hand, MM plasma cells produce mostly monomeric IgG or dimeric IgA M-protein, leading to fewer blood viscosity increased than pentameric IgM.
Among MM patients, 3–6% experienced this complication at diagnosis in cohorts prior to the 21st century (7, 8).
To avoid vital complications (thrombosis, gut, or cerebral bleeding), early management with therapeutic plasma exchange (TPE) for HVS is advised as well as management in the intensive care unit (ICU) for severe forms (neurological signs, bleeding, or thrombosis related to HVS) (9, 10). One TPE can be sufficient to resolve symptoms of HVS (10).”