Hyperviscosity syndromeMANAGEMENT

HEMATOLOGIC AND ONCOLOGIC PRESENTATIONS · 12 SECTIONS

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494 items

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Cardiovascular Presentations66▾

Respiratory Presentations47▾

Neurological Presentations77▾

Gastrointestinal Presentations63▾

Genitourinary and Reproductive Presentations44▾

Infectious Disease Presentations146▾

Trauma Presentations88▾

Toxicologic Presentations67▾

Psychiatric Presentations25▾

Musculoskeletal and Rheumatologic Presentations89▾

Dermatology Presentations21▾

Environmental and Exposure Presentations26▾

Hematologic and Oncologic Presentations30▾

Acute chest syndrome Acute Hemolytic Transfusion Reaction Aplastic Anemia (Crisis)Autoimmune Hemolytic Anemia Coagulopathy Delayed Hemolytic Transfusion Reaction Disseminated Intravascular Coagulation (DIC)Essential Thrombocythemia Febrile Non-hemolytic Transfusion Reaction Graft Versus Host Disease (Acute)Hemolytic Uremic Syndrome (HUS)Hemophilia A (Bleeding Crisis)Heparin-Induced Thrombocytopenia (HIT)Hyperviscosity syndrome Immune Thrombocytopenic Purpura (ITP)Leukemoid Reaction Myelofibrosis Paroxysmal Nocturnal Hemoglobinuria Polycythemia Vera (Crisis)Pure Red Cell Aplasia Sickle Cell (Acute Chest Syndrome)Sickle Cell (Splenic Sequestration)Sickle cell crisis (vaso-occlusive)Symptomatic anemia Thrombocytopenia (severe)Thrombotic Thrombocytopenic Purpura (TTP)Transfusion-Associated Circulatory Overload (TACO)Transfusion-Related Acute Lung Injury (TRALI)Tumor lysis syndrome Von Willebrand Disease (Bleeding)

Pediatric-Specific Presentations13▾

Endocrine and Metabolic Presentations42▾

ENT and Maxillofacial Presentations21▾

Ophthalmologic Presentations15▾

Obstetric Presentations17▾

Systemic and Miscellaneous Presentations15▾

01Approach to the Critical Patient/attcp62

Immediate stabilization

›Resuscitation priorities

›Airway compromise from decreased consciousness

›If unable to protect airway, rapid sequence intubation

›Breathing failure from aspiration or pulmonary edema

›If hypoxemia, supplemental oxygen to target SpO2 92-96%

›Circulatory instability

›If hypotension, isotonic crystalloid 500-1000 mL IV bolus

›Active bleeding

›If life threatening hemorrhage, massive transfusion protocol consideration

Hyperviscosity recognition triggers

›Time critical pattern recognition

›Neurologic symptoms

›Headache

›Confusion

›Somnolence

›Seizure

›Focal neurologic deficit

›Visual symptoms

›Blurred vision

›Diplopia

›Visual field loss

›Mucosal bleeding

›Epistaxis

›Gingival bleeding

›Easy bruising

›Cardiopulmonary symptoms

›Dyspnea

›Chest discomfort

›Heart failure signs

›High risk contexts

›Known Waldenström macroglobulinemia

›Known multiple myeloma

›Hyperleukocytosis with acute leukemia

›Polycythemia with neurologic or visual symptoms

Monitoring and access

›Early monitoring setup

›Continuous cardiac monitoring

›Dysrhythmia surveillance during electrolyte shifts

›Frequent neurologic reassessment

›Interval mental status trend

›Two large bore IV access

›Apheresis compatible access planning

›If severe symptoms, arterial line consideration

›Frequent blood sampling support

Immediate consultation and activation

›Team activation

›Hematology urgent consult

›Symptomatic suspected paraprotein hyperviscosity

›Apheresis service urgent consult

›Neurologic symptoms plus suspected paraproteinemia

›Visual symptoms plus suspected paraproteinemia

›Mucosal bleeding plus suspected paraproteinemia

›Oncology consult

›Suspected plasma cell dyscrasia or lymphoplasmacytic lymphoma

›If hyperleukocytosis, leukemia team consult

›Cytoreduction pathway

Early pitfalls to avoid

›High risk errors

›Red cell transfusion before viscosity control

›Potential viscosity increase and neurologic worsening

›Diuretics without intravascular assessment

›Potential hemoconcentration and viscosity increase

›Rituximab initiation before plasmapheresis in Waldenström

›IgM flare risk with symptom worsening

›Delay in apheresis for classic triad presentation

›Time dependent neurologic and visual injury risk

02History/hx57

Focused symptom pattern

›Presenting features

›Symptom timing

›Rapid progression over hours to days

›Neurologic symptoms

›Headache

›Dizziness

›Confusion

›Ataxia

›Seizure

›Visual symptoms

›Blurred vision

›Transient vision loss

›Bleeding symptoms

›Epistaxis

›Gingival bleeding

›Menorrhagia

›Constitutional symptoms

›Weight loss

›Night sweats

›Fatigue

Disease specific risk context

›Underlying disorder context

›Waldenström macroglobulinemia features

›Prior IgM paraprotein diagnosis

›Peripheral neuropathy

›Cryoglobulinemia symptoms

›Multiple myeloma features

›Bone pain

›Recurrent infections

›Prior monoclonal gammopathy

›Acute leukemia features

›Fever

›Easy bruising

›Dyspnea

›Prior blasts or leukocytosis

›Polycythemia features

›Pruritus after hot shower

›Thrombosis history

Medication and exposure factors

›Contributing factors

›Dehydration

›Reduced oral intake

›Recent transfusion

›Red cell transfusion within days

›Prothrombotic medications

›Estrogen exposure

›Renal impairment

›Reduced clearance of paraprotein associated complications

Coding and terminology anchors

›Standardized terms

›Hyperviscosity syndrome

›ICD-10 D75.89 other specified diseases of blood and blood-forming organs

›Waldenström macroglobulinemia

›ICD-10 C88.0

›Multiple myeloma

›ICD-10 C90.0

›Leukostasis

›SNOMED CT concept term leukostasis

›Plasma hyperviscosity

›SNOMED CT concept term hyperviscosity syndrome

03Physical Exam/exam42

General and hemodynamics

›Initial exam priorities

›Mental status

›Agitation

›Somnolence

›Delirium

›Vital signs

›Hypotension

›Tachycardia

›Hypoxemia

›Volume status

›Dry mucous membranes

›Poor skin turgor

Bleeding and mucosal findings

›Hemorrhage pattern

›Epistaxis

›Ongoing bleeding

›Gingival bleeding

›Oozing without trauma

›Skin findings

›Petechiae

›Purpura

Neurologic exam

›Neurologic localization

›Cranial nerves

›Visual acuity change

›Dysarthria

›Cerebellar function

›Ataxia

›Motor and sensory

›Focal deficit

›Seizure activity

›Postictal state

Ophthalmologic exam

›Funduscopic clues

›Retinal hemorrhages

›Flame shaped hemorrhages

›Venous engorgement

›Sausage link segmentation

›Papilledema

›Optic disc swelling

Pitfalls and mimics on exam

›Examination traps

›Normal early fundus exam despite symptoms

›Need for urgent ophthalmology if visual complaints persist

›Focal deficits mimicking stroke

›Hyperviscosity related microcirculatory sludging

04Differential Diagnosis/ddx33

Life threatening mimics

›Immediate threats

›Acute ischemic stroke

›Sudden focal deficit

›Intracranial hemorrhage

›Severe headache

›Meningitis or encephalitis

›Fever

›Status epilepticus

›Persistent altered consciousness

›Thrombotic thrombocytopenic purpura

›MAHA plus thrombocytopenia

Hematologic causes of hyperviscosity phenotype

›Viscosity mechanisms

›IgM paraproteinemia

›Waldenström macroglobulinemia C88.0

›IgA paraproteinemia

›Multiple myeloma C90.0

›IgG paraproteinemia

›Multiple myeloma C90.0

›Hyperleukocytosis with leukostasis

›Acute myeloid leukemia

›Acute lymphoblastic leukemia

›Erythrocytosis

›Polycythemia vera D45

›Secondary polycythemia D75.1

›Cryoglobulinemia

›Cold induced symptoms

Clinical clues to differentiate

›Differentiation hints

›Mucosal bleeding with retinal findings

›Paraprotein hyperviscosity pattern

›Dyspnea with very high WBC

›Leukostasis pattern

›Pruritus and thrombosis history

›Polycythemia pattern

05Laboratory Tests/lt52

Immediate core labs

›Initial laboratory bundle

›Complete blood count with differential

›Hemoglobin and hematocrit for erythrocytosis

›Platelet count for bleeding risk modifiers

›White blood cell count for hyperleukocytosis

›Peripheral blood smear

›Rouleaux formation

›Circulating blasts

›Schistocytes for MAHA screen

›Basic metabolic panel

›Sodium mmol/L

›Potassium mmol/L

›Creatinine for renal impairment

›Liver panel

›Synthetic dysfunction contribution to bleeding

›Coagulation studies

›INR

›aPTT

›Fibrinogen

›Consumptive coagulopathy assessment

Hyperviscosity targeted labs

›Etiology confirmation studies

›Serum viscosity

›Centipoise value trending with symptoms

›Quantitative immunoglobulins

›IgM g/L

›IgG g/L

›IgA g/L

›Serum protein electrophoresis

›Monoclonal spike identification

›Immunofixation

›Isotype confirmation

›Serum free light chains

›Kappa and lambda ratio

›Serum albumin and total protein

›Protein gap screening

Supportive and complication labs

›Complication assessment

›Venous blood gas if respiratory compromise

›pH

›pCO2 mmHg

›Lactate mmol/L if shock physiology

›Tissue hypoperfusion marker

›Type and screen

›Transfusion readiness if hemorrhage

›Beta hCG in pregnancy risk

›Imaging and medication planning

Interpretation pitfalls

›Common laboratory pitfalls

›Pseudohyponatremia with marked hyperproteinemia

›Indirect ISE sodium artifact

›Serum viscosity result delay

›Clinical diagnosis supported by symptoms and context

›Coagulation testing interference

›Paraprotein assay artifact consideration

06Diagnostic Tests/dt41

Scoring Systems

›Decision and threshold frameworks

›Symptomatic hyperviscosity definition

›Neurologic symptoms attributable to impaired microcirculation

›Visual symptoms attributable to retinal venous congestion

›Mucosal bleeding attributable to paraprotein related platelet dysfunction

›Serum viscosity thresholds

›Normal serum viscosity approximately 1.4-1.8 centipoise

›Symptoms often when viscosity exceeds 4 centipoise

›Threshold variability by paraprotein type and patient factors

›Paraprotein level risk markers

›IgM levels above 40 g/L associated with higher hyperviscosity risk

›IgA paraprotein can cause symptoms at lower viscosity than IgG in some patients

›Leukostasis risk markers

›WBC above 100 x10^9/L high risk for leukostasis

›AML leukostasis can occur at lower WBC than ALL

MRI

›MRI applications

›Brain MRI for persistent neurologic deficit after stabilization

›Diffusion restriction for ischemia assessment

›Contraindications

›Unstable airway or hemodynamics

›Interpretation pearls

›Multifocal small vessel ischemia pattern support microcirculatory sludging

›CT applications

›Noncontrast CT head for acute severe headache or focal deficit

›Intracranial hemorrhage exclusion

›CT angiography head and neck if stroke pathway indicated

›Large vessel occlusion assessment

›Chest CT if cardiopulmonary symptoms with alternative diagnosis concern

›Pulmonary embolism assessment

›Pitfalls

›Hyperviscosity symptoms can mimic stroke without imaging correlates early

Ultrasound (or US)

›Point of care ultrasound uses

›Cardiac POCUS for shock

›Left ventricular function

›Pericardial effusion

›Lung ultrasound for dyspnea

›B lines for pulmonary edema

›Ocular ultrasound if acute visual loss and limited fundoscopy

›Vitreous hemorrhage pattern

›Vascular ultrasound for suspected thrombosis

›Deep vein thrombosis assessment

07Disposition/dispo22

Level of care selection

›Disposition targets

›ICU admission criteria

›Any neurologic symptoms attributed to hyperviscosity

›Any visual symptoms attributed to hyperviscosity

›Active bleeding requiring ongoing intervention

›Hemodynamic instability

›Hyperleukocytosis with respiratory or neurologic symptoms

›Stepdown admission criteria

›Mild symptoms resolved after initial therapy with close monitoring need

›Transfer criteria

›No on site apheresis capability with symptomatic suspected paraprotein hyperviscosity

›No on site leukemia specialty support with leukostasis suspicion

Disposition planning details

›System coordination

›Early interfacility transfer activation

›Apheresis availability confirmation

›Hematology accepting service communication

›Planned cytoreduction and disease directed therapy timing

›Ophthalmology involvement

›Retinal hemorrhage or severe visual symptoms

Discharge rarity

›Discharge considerations

›Asymptomatic hyperproteinemia without hyperviscosity features

›Expedited outpatient hematology follow up within 72 hours

08Treatment/t86

Supportive care

›Immediate supportive measures

›Intravascular volume optimization

›Isotonic crystalloid 500-1000 mL IV bolus

›Repeat bolus based on perfusion and pulmonary status

›Stop boluses if pulmonary edema develops

›Oxygen support

›Nasal cannula oxygen for hypoxemia

›Escalate to high flow nasal cannula if persistent work of breathing

›Intubation if refractory hypoxemia or inability to protect airway

›Seizure management if present

›Lorazepam 0.1 mg/kg IV

›Maximum 4 mg per dose

›Repeat once after 5 minutes if ongoing seizure

›Levetiracetam 60 mg/kg IV loading

›Maximum 4500 mg

›Maintenance 20-30 mg/kg IV every 12 hours

Therapeutic plasma exchange for paraprotein hyperviscosity

›Plasma exchange pathway

›Indications

›Symptomatic hyperviscosity with suspected or confirmed monoclonal gammopathy

›Neurologic symptoms

›Class I recommendation expert consensus for urgent plasma exchange

›Visual symptoms

›Class I recommendation expert consensus for urgent plasma exchange

›Clinically significant mucosal bleeding

›Class I recommendation expert consensus for urgent plasma exchange

›Procedure parameters

›Exchange volume 1-1.5 plasma volumes per session

›Daily sessions until symptom resolution

›Goal viscosity reduction with clinical improvement

›Replacement fluid 5% albumin

›Consider plasma if significant coagulopathy or bleeding

›Monitor fibrinogen during repeated exchanges

›Timing relative to disease directed therapy

›Waldenström macroglobulinemia

›Plasma exchange before rituximab initiation

›IgM flare risk mitigation

›Multiple myeloma

›Plasma exchange plus prompt cytoreductive therapy

›Hematology directed regimen planning

›Transfusion precautions

›If severe symptomatic anemia and unstable

›Red cell transfusion after plasma exchange when feasible

›If transfusion before exchange unavoidable, slow infusion with close neurologic monitoring

Leukostasis and hyperleukocytosis treatment

›Leukostasis pathway

›Cytoreduction

›Hydroxyurea 50 mg/kg/day PO

›Divide every 6-12 hours

›Titrate daily based on WBC trend and toxicity

›Definitive leukemia therapy

›Urgent induction planning with hematology

›Tumor lysis prophylaxis initiation based on risk

›Leukapheresis

›Severe symptoms with hyperleukocytosis

›Respiratory distress

›Class IIa recommendation expert consensus for leukapheresis as adjunct

›Neurologic symptoms

›Class IIa recommendation expert consensus for leukapheresis as adjunct

›Limitations

›Temporary WBC reduction without disease control

›Not a substitute for chemotherapy

Erythrocytosis driven hyperviscosity

›Phlebotomy pathway

›Symptomatic polycythemia

›Therapeutic phlebotomy 250-500 mL

›Repeat to symptom improvement and target hematocrit

›Hematology individualized target based on etiology

›Volume replacement

›Isotonic crystalloid after phlebotomy

›Avoid hypotension and hemoconcentration cycles

Bleeding management

›Hemorrhage control

›Local measures

›Topical vasoconstrictor for epistaxis

›Tranexamic acid topical or soaked pledget consideration

›Avoid if contraindicated by thrombosis concern assessment

›Platelet dysfunction from paraprotein

›Plasma exchange priority over empiric platelet transfusion when platelets adequate

›Platelet transfusion reserved for thrombocytopenia or procedural need

›Coagulopathy correction

›If fibrinogen low, cryoprecipitate guided by institutional thresholds

›Recheck fibrinogen after replacement

Medication cautions

›High risk medication decisions

›Anticoagulation

›Individualized based on thrombosis versus bleeding balance

›Hematology input prior to initiation when possible

›Diuretics

›Avoid early unless clear volume overload

›Risk of hemoconcentration and symptom worsening

09Special Populations/sp36

Pregnancy

›Pregnancy specific considerations

›Maternal physiology effects

›Baseline hypercoagulability

›Thrombosis risk amplification with hyperviscosity states

›Plasma volume expansion

›Symptom expression variability

›Diagnostic approach

›Beta hCG confirmation

›Imaging selection with fetal risk awareness

›MRI preferred over CT for neurologic evaluation when stable

›Avoid gadolinium unless essential

›Treatment considerations

›Plasma exchange compatibility with pregnancy

›Multidisciplinary coordination with obstetrics

›Thromboprophylaxis decisions

›Individualized maternal bleeding risk assessment

Geriatric

›Older adult considerations

›Comorbidity burden

›Heart failure vulnerability with aggressive fluids

›Frequent lung exam and POCUS reassessment

›Medication sensitivity

›Sedation risk with benzodiazepines

›Lowest effective dose for seizures

›Atypical presentations

›Delirium as primary manifestation

›Lower threshold for hyperviscosity evaluation with paraproteinemia history

Pediatrics

›Pediatric considerations

›Etiology differences

›Leukemia related hyperleukocytosis more common than paraprotein syndromes

›Early leukostasis recognition in acute leukemia

›Weight based dosing

›Hydroxyurea dosing mg/kg/day with hematology oversight

›Close monitoring for myelosuppression

›Apheresis logistics

›Vascular access planning with pediatric anesthesia

›Hemodynamic monitoring during exchange

10Background/b33

Epidemiology

›Epidemiologic patterns

›Waldenström macroglobulinemia

›Hyperviscosity more common with IgM paraprotein than IgG

›Multiple myeloma

›Hyperviscosity less common overall but occurs with IgA and high IgG

›Acute leukemia

›Leukostasis risk increases with hyperleukocytosis

Pathophysiology

›Mechanisms of viscosity related injury

›Increased plasma viscosity

›Paraprotein concentration and molecular size contribution

›IgM pentamer high viscosity effect

›Impaired microcirculatory flow

›Reduced tissue oxygen delivery

›Neurologic dysfunction and retinal ischemia

›Bleeding tendency

›Paraprotein related platelet dysfunction

›Mucosal bleeding pattern

›Leukostasis

›Blast rigidity and endothelial interaction

›Pulmonary and CNS microvascular obstruction

Therapeutic Considerations

›Treatment rationale

›Plasma exchange effectiveness

›Rapid reduction in circulating paraprotein

›Symptom improvement often within hours

›Need for disease directed therapy

›Paraprotein production continues without cytoreduction

›Recurrence risk without definitive treatment

›Transfusion effects

›Red cell transfusion increases whole blood viscosity

›Timing after viscosity reduction preferred when feasible

›Evidence framing

›Apheresis recommendations primarily based on expert consensus and observational data

›Class I and IIa labels used to reflect consensus strength rather than trial class

11Patient Discharge Instructions/pdi19

copy discharge instructions

›Discharge instruction set

›Return immediately to emergency care for any new neurologic symptom

›Confusion

›Weakness

›Seizure

›Return immediately for any new or worsening vision change

›Blurred vision

›Vision loss

›Return immediately for any uncontrolled bleeding

›Nosebleed lasting longer than 20 minutes

›Vomiting blood

›Black stools

›Hydration guidance

›Adequate oral fluids unless fluid restricted by clinician

›Medication safety

›Avoid NSAIDs if bleeding tendency present unless clinician approved

›Follow up

›Hematology appointment within 72 hours if discharged

›Same day return if unable to obtain urgent follow up

12References/r11

Clinical guidelines and evidence sources

›Reference set

›American Society for Apheresis guidelines for therapeutic apheresis indications

›Therapeutic plasma exchange for symptomatic paraprotein hyperviscosity

›NCCN guidelines Waldenström macroglobulinemia

›Rituximab associated IgM flare considerations

›International Myeloma Working Group guidance

›Acute complications management including hyperviscosity

›Acute leukemia supportive care guidance

›Hyperleukocytosis and leukostasis cytoreduction strategies

›Internal instruction source

›Clinical management system formatting and nesting requirements

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Reviewed by SymptomDx Medical Team · Last reviewed June 16, 2026

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