Hyperviscosity syndrome: Difference between revisions

Latest revision as of 15:09, 12 February 2021

Background

Increased serum viscosity due to:
- Increased blood products (polycythemia, thrombocytosis, leukemia)
- Immunoglobulins (Waldenstrom's, IgA myeloma, multiple myeloma)
Symptoms arise from poor capillary flow and organ congestion

Clinical Features

General: fever, fatigue
CNS: headache, blurred vision, altered mental status, focal neurologic deficits
Pulm: dyspnea, hypoxia
Cardiac: chest pain, CHF
Heme: mucosal bleeding

Differential Diagnosis

Detailed differential can be found on each page

Oncologic Emergencies

Related to Local Tumor Effects

Malignant airway obstruction
Bone metastases and pathologic fractures
Malignant spinal cord compression
Malignant Pericardial Effusion and Tamponade
Superior vena cava syndrome

Related to Biochemical Derangement

Related to Hematologic Derangement

Related to Therapy

Chemotherapy-induced nausea and vomiting
Cytokine release syndrome
Chemotherapeutic drug extravasation
Differentiation syndrome (retinoic acid syndrome) in APML
Stem cell transplant complications
Catheter-related complications
- Tunnel infection
- Exit site infection
- CVC obstruction (intraluminal or catheter tip thrombosis)
- Catheter-related venous thrombosis
- Fracture of catheter lumen
- Oncologic therapy related adverse events

Evaluation

Workup

CBC, coags, type and screen, chem 10
Urinalysis
CT head if neuro symptoms
CXR
Consider initiating inpatient testing with: Total protein, albumin, SPEP, UPEP, IgA, IgG, IgM
Note: ABGs might have SaO2 vs. SpO2 discrepancies
- Causing spurious hypoxemia on ABG, with low PaO2 (as opposed to dyshemoglobinemia)
- Due to "leukocyte larceny," "platelet larceny"^[1]^[2]^[3]
- Excessive O2 consumption by WBCs or platelets following blood sample collection

Evaluation

Symptoms of hyperviscosity can appear when one of the following occur:

Red cell mass > 25% of normal^[4]
Platelets >600,000/µL^[5]
Leukocytosis >100,000
Serum viscosity >4-5cP (normal 1.4-1.8cP)^[6] or Globulin gap (Total protein - albumin) > 4

Management

ABCs
IVF, supportive care
Ig mediated/Dysproteinemia
- Plasmapheresis
Leukostasis
- Leukapheresis
Thrombocytosis
- Plateletpheresis
- Consider ASA
Polycythemia
- Phlebotomy
If pheresis is not unavailable, phlebotomize 2-3 units, give 2-3 L IVF

Disposition

Typically require admission if symptomatic
Hem/Onc consult

External Links

References

↑ Mehta A, Lichtin AE, Vigg A, Parambil JG. Platelet larceny: spurious hypoxaemia due to extreme thrombocytosis. Eur Respir J. 2008 Feb;31(2):469-72.
↑ Sacchetti A, Grynn J, Pope A, Vasso S. Leukocyte larceny: spurious hypoxemia confirmed with pulse oximetry. J Emerg Med. 1990 Sep-Oct;8(5):567-9.
↑ Lele AV, Mirski MA, Stevens RD. Spurious hypoxemia. Crit Care Med. 2005 Aug;33(8):1854-6.
↑ Pearson TC and Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1999; 22 (1):87-93.
↑ Murphy S et al. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol. 1999; 36(1):9-13.
↑ Mehta J and Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003; 29(5):467-471.

Authors:

[1] Mehta A, Lichtin AE, Vigg A, Parambil JG. Platelet larceny: spurious hypoxaemia due to extreme thrombocytosis. Eur Respir J. 2008 Feb;31(2):469-72.

[2] Sacchetti A, Grynn J, Pope A, Vasso S. Leukocyte larceny: spurious hypoxemia confirmed with pulse oximetry. J Emerg Med. 1990 Sep-Oct;8(5):567-9.

[3] Lele AV, Mirski MA, Stevens RD. Spurious hypoxemia. Crit Care Med. 2005 Aug;33(8):1854-6.

[4] Pearson TC and Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1999; 22 (1):87-93.

[5] Murphy S et al. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol. 1999; 36(1):9-13.

[6] Mehta J and Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003; 29(5):467-471.

[1]

[2]

[3]

[4]

[5]

[6]

@@ Line 1: / Line 1: @@
 ==Background==
 *Increased serum viscosity due to:
-*#Increased blood products (polycythemia, thrombocytosis. leukemia)
+**Increased blood products ([[polycythemia]], thrombocytosis, [[leukemia]])
-*#Immunoglobulins (Waldenstrom's, IgA myeloma, multiple myeloma)
+**Immunoglobulins (Waldenstrom's, IgA myeloma, [[multiple myeloma]])
 *Symptoms arise from poor capillary flow and organ congestion
 ==Clinical Features==
-*Symptoms of hyperviscosity can appear when one of the following occur:
+*General: [[fever]], [[fatigue]]
-**Red cell mass > 25% of normal<ref>Pearson TC and Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1999; 22 (1):87-93.</ref>
+*CNS: [[headache]], [[blurred vision]], [[altered mental status]], [[focal neurologic deficits]]
-**Plt >600,000/µL<ref>Murphy S et al. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol. 1999; 36(1):9-13.</ref>
+*Pulm: [[dyspnea]], [[hypoxia]]
-**Leukocytosis >100,000
+*Cardiac: [[chest pain]], [[CHF]]
-**Serum viscosity >4-5cP (normal 1.4-1.8cP)<ref>Mehta J and Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003; 29(5):467-471.</ref> or Globulin gap (Total protein - albumin) > 4
+*Heme: mucosal bleeding
-===Symptoms===
-*Gen: fever, fatigue
-*CNS: headache, blurred vision, AMS, focal neuro deficits
-*Pulm: dyspnea, hypoxia
-*Cardiac: chest pain, CHF
-*Hem: mucosal bleeding
 ==Differential Diagnosis==
-''Detailed DDx can be found on each page''
+''Detailed differential can be found on each page''
 *[[Stroke]]
 *[[Altered mental status]]
@@ Line 27: / Line 20: @@
 *[[Dyspnea]]
-==Diagnosis==
+{{Oncologic emergencies DDX}}
-*CBC
-*Chem 10
+==Evaluation==
-*Coags
+===Workup===
-*Type and screen
+*CBC, coags, type and screen, chem 10
-*UA
+*[[Urinalysis]]
-*CT brain if neuro symptoms
+*[[CT head]] if neuro symptoms
-*CXR
+*[[CXR]]
 *Consider initiating inpatient testing with: Total protein, albumin, SPEP, UPEP, IgA, IgG, IgM
-*Note: ABGs might have SaO2 vs. SpO2 discrepancies
+*Note: [[ABG]]s might have SaO2 vs. SpO2 discrepancies
 **Causing spurious hypoxemia on ABG, with low PaO2 (as opposed to [[dyshemoglobinemia]])
 **Due to "leukocyte larceny," "platelet larceny"<ref>Mehta A, Lichtin AE, Vigg A, Parambil JG. Platelet larceny: spurious hypoxaemia due to extreme thrombocytosis. Eur Respir J. 2008 Feb;31(2):469-72.</ref><ref>Sacchetti A, Grynn J, Pope A, Vasso S. Leukocyte larceny: spurious hypoxemia confirmed with pulse oximetry. J Emerg Med. 1990 Sep-Oct;8(5):567-9.</ref><ref>Lele AV, Mirski MA, Stevens RD. Spurious hypoxemia. Crit Care Med. 2005 Aug;33(8):1854-6.</ref>
-**Excessive O2 consumption by WBCs or plts following blood sample collection
+**Excessive O2 consumption by WBCs or platelets following blood sample collection
+===Evaluation===
+Symptoms of hyperviscosity can appear when one of the following occur:
+*Red cell mass > 25% of normal<ref>Pearson TC and Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1999; 22 (1):87-93.</ref>
+*Platelets >600,000/µL<ref>Murphy S et al. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol. 1999; 36(1):9-13.</ref>
+*Leukocytosis >100,000
+*Serum viscosity >4-5cP (normal 1.4-1.8cP)<ref>Mehta J and Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003; 29(5):467-471.</ref> or Globulin gap (Total protein - albumin) > 4
 ==Management==
 *ABCs
-*IVF and supportive care
+*[[IVF]], supportive care
-*Ig mediated
+*Ig mediated/Dysproteinemia
-**Plasmapharesis
+**[[Plasmapheresis]]
 *Leukostasis
-**Leukopheresis
+**Leukapheresis
 *Thrombocytosis
 **Plateletpheresis
-**Consider ASA
+**Consider [[ASA]]
-*Polycythemia
+*[[Polycythemia vera|Polycythemia]]
 **Phlebotomy
 *If pheresis is not unavailable, phlebotomize 2-3 units, give 2-3 L IVF
@@ Line 60: / Line 60: @@
 ==See Also==
+*[[Oncologic emergencies]]
 ==External Links==