June 2007, Volume 11, Number 3

 

CJON book excerpt series

Capillary Leak Syndrome

 

 

 

Susan A. Ezzone, MS, RN, CNP

 

 

 

This excerpt, chapter 27 from the book Clinical Manual for the Oncology Advanced Practice Nurse (2nd ed.), edited by Dawn Camp-Sorrell, MSN, FNP, AOCN, and Rebecca A. Hawkins, MSN, ANP, AOCN, is a part of a series of clinically relevant reprints that appear regularly in the Clinical Journal of Oncology Nursing.

 

VIII. Definition: Shift of intravascular fluid and plasma into the extravascular space (Fardet et al., 2004)

VIII. Physiology/Pathophysiology (Fishel, Are, & Barbul, 2003; Marx, 2003)

A. Normal: Small blood vessels carrying blood and forming the capillary system. Capillaries connect the smallest arteries (arterioles) with the smallest veins (venules).

B. Pathophysiology

11. Generalized capillary endothelial cell injury in multiple organs is responsible for the development of capillary leak syndrome (CLS).

12. Endothelial cell damage may occur because of endotoxin exposure, ischemia, vessel injury with platelet deposition, or mechanical injury.

13. Cytokines such as interleukin (IL)-2, tumor necrosis factor (TNF)-alpha, anti IL-1 B, and CD8-positive lymphocytes are present and may have a role in triggering CLS.

14. Platelet-activating factor and vascular endothelial growth factor increase vascular permeability.

15. Inflammatory reactions occur and cause microvascular permeability, capillary leak, loss of protein, tissue edema, and hypoalbuminemia.

16. A shift of fluid and albumin into body tissues
occurs.

17. An associated decreased peripheral vascular resistance, hypotension, and intravascular volume compound the fluid shift.

VIII. Clinical features (Cahill, Spitzer, & Mazumder, 1996; Nurnberger, Willers, Burdach, & Gobel, 1997)

A. Risk factors

11. Blood and marrow stem cell transplant

a) During preparative regimen

b) During time of engraftment along with abnormalities in liver and renal function

c) During rapid steroid tapers

d) During infection or graft-versus-host disease

e) During infusion of donor white blood cells (WBCs)

f) During infusion of marrow/blood stem cells

g) Human leukocyte antigen mismatched bone marrow transplant recipient

h) Oxygen toxicity

12. Kidney transplant

13. Liver transplant

14. Biotherapy (especially IL and TNF)

15. Chemotherapy

B. History

11. History of cancer and cancer treatment

12. Current medications: Prescribed and over-the-counter

13. History of presenting symptom(s): Precipitating factors, onset, location, and duration

14. Changes in activities of daily living

C. Signs and symptoms

11. Ascites

12. Weight gain

13. Edema and/or anasarca (generalized total-body edema)

14. Chest pain

15. Shortness of breath

16. Productive or nonproductive cough

17. Tachypnea

18. Decreased urine output

19. Fever

10. Lethargy, malaise, or obtundation

11. Confusion and restlessness

12. Cyanosis and pallor of skin, lips, and nail beds

D. Physical exam

11. Vital signs: Weight (signs of gain), blood pressure (hypotension), pulse (tachycardia)

12. Pulmonary exam: Presence of rales and rhonchi on auscultation; dullness on percussion over consolidated areas

13. Cardiac exam: Presence of S3, S4, murmur, or gallop; tachycardia; peripheral edema

14. Abdomen exam: Ascites, tenderness, distention, softness or firmness, hepatomegaly, splenomegaly, presence or absence of bowel sounds

15. Dermatologic: Presence of purpuric lesions, flesh-colored or erythematous lesions (Fardet et al., 2004)

IIIV. Diagnostic tests

A. Laboratory

11. Complete blood count with differential

a) Elevated WBC count may increase suspicion of infection.

b) Hemoconcentration may occur with an increased WBC count and hematocrit.

12. Urinalysis

a) Presence of leukocytes may be caused by
infection.

b) Presence of protein or casts may indicate renal failure or disease.

13. Liver function tests, including total and direct bilirubin, to rule out hepatobiliary disease

14. Renal function tests, including urea and creatinine, to evaluate renal function

15. Serum albumin: May be decreased, leading to decreased oncotic pressure and edema

B. Radiology: Chest x-ray: To rule out noncardiogenic pulmonary edema, pleural effusion, pulmonary venous hypertension, interstitial infiltrates, and pericardial effusions

C. Other

11. Bronchoscopy, with or without lung biopsy, can rule out infection, hemorrhage, or other causes of respiratory distress.

12. Arterial blood gases may show hypoxia and CO2 retention.

13. Pulmonary function tests reveal decreased pulmonary compliance.

14. Hemodynamic monitoring including pulmonary capillary wedge pressure (PCWP) and cardiac output to measure fluid status (normal PCWP = 612 mm Hg; normal cardiac output = 48 liters/minute)

a) Decreased PCWP may indicate hypovolemia.

b) Increased PCWP may indicate left ventricular failure or cardiac insufficiency.

c) Cardiac output may be increased early in CLS, then decreased later in the syndrome.

15. Skin biopsy: Mild perivascular, nonspecific dermal mononuclear infiltrates; mucinous deposits; mild lymphocytic infiltration (Fardet et al., 2004)

IIIV. Differential diagnosis (Fardet et al., 2004)

A. Paraproteinemias or diseases/conditions with low protein levels

B. Lymphoma

C. Psoriasis

D. Drug-induced, such as IV cyclosporine or amphotericin-B, biotherapy

E. Viral syndrome, such as cytomegalovirus

F. Pneumonitis

G. Sepsis (see Chapter 140)

H. Disseminated intravascular coagulation (see Chapter 118)

I. Cytokine reaction

IIVI. Treatment: Supportive care until CLS resolves (Amoura et al., 1997; Fardet et al., 2004; Fishel et al., 2003; Marx, 2003)

A. Treat the underlying cause.

B. Administer glucocorticoids at high doses, then taper quickly as tolerated.

C. Provide IV fluid replacement.

11. Infusion of colloids rather than crystalloids, such as blood or albumin

12. Infusion of albumin

D. Administration of diuretics is controversial because intravascular hypovolemia is present and acute renal failure may develop.

E. Restrict oral fluids to 5001,000 ml per day. Gradually increase fluids as condition improves.

F. Hemodialysis may be necessary if acute renal failure occurs.

G. Mechanical ventilation may be indicated if respiratory distress or failure occurs.

H. Prophylactic antibiotics may promote growth of organisms and are not recommended.

I. Administer vasopressors as needed for management of hypotension.

J. Provide nutritional support, such as enteral feedings or total parenteral nutrition, to maintain high caloric intake because of increased energy expenditure.

K. Clinical studies using antibodies to IL-1, IL-6, TNF-alpha, angiopoietin-1, and endothelin A receptor antagonist blocks or reduces capillary leak in animal models (Fishel et al., 2003).

IVII. Follow-up

A. Inpatient hospitalization is necessary to manage the signs, symptoms, and complications of CLS.

B. Perform daily monitoring of intake, output, weight, and renal and liver function.

C. Perform frequent chest radiographs to monitor pulmonary edema.

VIII. Referrals

A. Nephrologist: To evaluate acute renal failure and recommend management

B. Pulmonologist: To evaluate lung function, perform bronchoscopy, and recommend management

 

 

References

 

Amoura, Z., Papo, T., Ninet, J., Hatron, P.Y., Guillaumie, J., Piette, A.M., et al. (1997). Systemic capillary leak syndrome: Report on 13 patients with special focus on course and treatment. American Journal of Medicine, 103, 514519.

 

Cahill, R.A., Spitzer, T.R., & Mazumder, A. (1996). Marrow engraftment and clinical manifestations of capillary leak syndrome. Bone Marrow Transplantation, 18, 177184.

 

Fardet, L., Kerob, D., Rybojad, M., Vignon-Pennamen, M.D., Schlemmer, B., Guermazi, A., et al. (2004). Idiopathic systemic capillary leak syndrome: Cutaneous involvement can be misleading. Dermatology, 209, 291295.

 

Fishel, R.S., Are, C., & Barbul, A. (2003). Vessel injury and capillary leak. Critical Care Medicine, 31(8 Suppl.), S502S511.

 

Marx, G. (2003). Fluid therapy in sepsis with capillary leakage. European Journal of Anaesthesiology, 20, 429442.

 

Nurnberger, W., Willers, R., Burdach, S., & Gobel, U. (1997). Risk factors for capillary leakage syndrome after bone marrow transplantation. Annals of Hematology, 74, 221224.

 

From Clinical Manual for the Oncology Advanced Practice Nurse (2nd ed., pp. 177180), edited by Dawn Camp-Sorrell, MSN, FNP, AOCN, and Rebecca A. Hawkins, MSN, ANP, AOCN, Pittsburgh, PA: Oncology Nursing Society. Reprinted with permission.

Digital Object Identifier: 10.1188/07.CJON.455-456