Suspected paraprotein hyperviscosity
Adult with a known or suspected paraprotein (Waldenström, myeloma, cryoglobulinaemia) and suspected hyperviscosity: symptoms, retinal signs or a high paraprotein level
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Hematology & OncologyEmergency
Paraprotein Hyperviscosity Syndrome Management
Paraprotein Hyperviscosity Syndrome Management: Suspected paraprotein hyperviscosity → High white cell count or suspected APL: use the leukostasis pathw...
Pathway Overview
12 steps
Algorithm Steps
12 total
01Start 02Warning High white cell count or suspected APL: use the leukostasis pathway
This pathway covers paraprotein (serum) hyperviscosity only
- Leukostasis is a different emergency with different treatment
- Plasma exchange does not lower the white cell count
03Action Recognise hyperviscosity (clinical diagnosis)
Classic triad: mucosal bleeding, visual change, neurological symptoms
- Bleeding: epistaxis, gum bleeding
- Visual: blurred vision; retinal haemorrhages, dilated tortuous veins
- Neurological: headache, dizziness, confusion, drowsiness, coma
- Also: breathlessness, heart failure
- Uncommon with IgM below 40 g/L; IgG3 from about 40 g/L; IgA usually 60 to 70 g/L or more
04Action Urgent tests and eye examination
Send tests; do not delay treatment for results
- Fundoscopy in all patients, even without visual symptoms
- Serum viscosity: send, but do not wait for the result
- Paraprotein level and type; immunoglobulins
- FBC, coagulation, group and screen, creatinine, electrolytes, calcium
- Cryoglobulins: collect and transport the sample at 37 °C
05Decision Symptoms or retinal signs of hyperviscosity?
Bleeding, visual or neurological symptoms, or retinal changes of hyperviscosity (not explained by hypertension or diabetes)
- If Yes
06Warning Symptomatic: avoid red cells before plasma exchange
Red cell transfusion raises blood viscosity
- Stable patient: delay red cell transfusion until after plasma exchange
- Unstable or life-threatening bleeding: transfuse now; do not wait for plasma exchange; tell haematology
- Stable but severe anaemia: ask haematology before transfusing
07Action Symptomatic: urgent plasma exchange
Paraprotein hyperviscosity: first-line (ASFA Category I). Cryoglobulinaemia (Category II): warm the replacement fluid and lines.
- Call haematology and the apheresis team now
- No apheresis on site: arrange urgent transfer
- 1 to 1.5 plasma volumes per procedure
- Replacement: albumin (4% in Australia); consider plasma if bleeding or coagulopathy
- Daily or every other day until symptoms resolve: usually 1 to 3 procedures; IgG may need 4 to 5; cryoglobulinaemia 3 to 8
- IgG: watch for a fluid shift at the end of each procedure
08Action Supportive care (caution: heart failure)
Heart failure or older patient: give IV fluid with care and avoid overload
- IV fluid for dehydration, hypercalcaemia or kidney injury; reassess volume often
- Oxygen if hypoxic
- Bleeding: check coagulation and von Willebrand factor (acquired VWD with high IgM); treat with haematology advice
09Action Disease-directed therapy when indicated (haematology)
Symptomatic: start therapy; plasma exchange is only a bridge. No symptoms: treat if haematology finds an indication. Waldenström: delay rituximab until IgM is below 40 g/L (IgM flare).
- Waldenström: a regimen that lowers IgM quickly (for example bortezomib-based or a BTK inhibitor)
- If rituximab is used: plasma exchange first, or add rituximab in a later cycle
- Myeloma: bortezomib-based regimen
- Pregnant or cannot have systemic therapy: maintenance plasma exchange may be used; specialist plan
- Cryoglobulinaemia: treat the cause (for example hepatitis C, B-cell disorder)
- Doses: see local haematology protocol
10Action Monitor response
Confirm that symptoms and retinal signs improve
- Symptoms and fundoscopy; after each plasma exchange if given
- Repeat serum viscosity and paraprotein level
- Symptoms recur before therapy works: repeat plasma exchange; maintenance every 1 to 4 weeks may be needed
- Check calcium, fibrinogen and coagulation after repeated procedures
11Outcome Hyperviscosity controlled
Continue treatment of the underlying disease with haematology
If No12Action No symptoms: urgent haematology review
No emergency plasma exchange for a high paraprotein level alone
- Before rituximab, if IgM 40 g/L or more or serum viscosity above 3.5 cP: plasma exchange first, or delay rituximab (IgM flare)
- Tell the patient to return at once with bleeding, visual or neurological symptoms
- Path rejoins step 09Shared downstream outcome
Guideline Source
ASFA Guidelines on the Use of Therapeutic Apheresis in Clinical Practice, Tenth Special Issue (J Clin Apher 2026)
Clinical Safety Information
Clinical Decision Support — Not a Substitute for Clinical Judgment
Individual patient factors may require deviation from these recommendations.
Known Limitations
- Hyperviscosity is a clinical diagnosis; serum viscosity units and ranges vary by laboratory
- Apheresis availability varies; arrange urgent transfer if it is not on site
- Covers paraprotein hyperviscosity only; leukostasis has a separate pathway
- No drug doses: disease-directed therapy follows haematology protocols
Contraindicated Populations
Hyperleukocytosis or leukostasis (use the leukostasis pathway)Children
Applicable Regions
AUUSEU
AU: Albumin for plasma exchange is usually 4% (Albumex 4). Paraprotein is reported in g/L. Serum viscosity units and reference ranges differ by laboratory.
Version 2Next review: 2027-09-30
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Frequently Asked Questions
What is the Paraprotein Hyperviscosity Syndrome Management?
The Paraprotein Hyperviscosity Syndrome Management is a emergency clinical algorithm for Hematology & Oncology. It provides a structured decision tree to guide clinical decision-making, based on ASFA Guidelines on the Use of Therapeutic Apheresis in Clinical Practice, Tenth Special Issue (J Clin Apher 2026).
What guideline is the Paraprotein Hyperviscosity Syndrome Management based on?
This algorithm is based on ASFA Guidelines on the Use of Therapeutic Apheresis in Clinical Practice, Tenth Special Issue (J Clin Apher 2026) (DOI: 10.1002/jca.70141).
What are the limitations of the Paraprotein Hyperviscosity Syndrome Management?
Known limitations include: Hyperviscosity is a clinical diagnosis; serum viscosity units and ranges vary by laboratory; Apheresis availability varies; arrange urgent transfer if it is not on site; Covers paraprotein hyperviscosity only; leukostasis has a separate pathway; No drug doses: disease-directed therapy follows haematology protocols. Individual patient factors may require deviation from these recommendations.
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