Disease Information for Capillary leak syndrome

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Clinical Manifestations
Signs & Symptoms
Low Blood Pressure
Signs of Poor Perfusion
Abdominal Pain
Edema of Lower Extremities
Swelling all extremities
Cough
Polyuria
Weight gain
Disease Progression
Course/Potentially lethal/untreated
Course/Recurrent
Course/Recurrent illness pattern
Lethal Potential
Demographics & Risk Factors
Established Disease Population
Patient/Recent URI/LRI
Population Group
Middle Age Adult
Sex & Age Groups
Population/Fifties adult
Population/Middle-aged adult
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Monoclonal M protein spike/electrophoresis (Lab)
Abnormal Lab Findings - Decreased
Albumin, serum (Lab)
C1-esterase inhibitor titer (Lab)
Complement 1 Inhibitor (LAB)
Abnormal Lab Findings - Increased
Hematocrit (Lab)
Hemoglobin (Lab)
RBC/Red Blood Count (Lab)
Associated Diseases & Rule outs
Rule Outs
Anaphylaxis, generalized
Associated Disease & Complications
Acute Renal Failure
Anasarca/Generalized Edema
Capillary leak syndrome
Congestive heart failure
Death Outcome
Edema
Hemoconcentration effect
Hypoalbuminemia Hypoproteinemia
Hypotension
Pleural Effusion
Pulmonary edema
Rhabdomyolysis
Shock
Shock, refractory
Multiple organ System Failure
Disease Mechanism & Classification
Class
CLASS/Capillary/microcirculation disorder (ex)
CLASS/Cardiovascular (category)
CLASS/Vascular disorder (ex).
Pathophysiology
Pathophysiology/Blood volume/decrease/low
Pathophysiology/High extracellular fluid volume
Pathophysiology/Low flow states/Systemic ischemia
Pathophysiology/Reperfusion of Ischemia status
Pathophysiology/Third spacing fluid shift
Pathophysiology/Volume redistribution
Pathophysiology/IL2- Cytokine effect permeability
Treatment
Drug Therapy - Indication
RX/Danazol (Danocrine)
RX/Terbutaline (Brethair)
RX/Theophylline (Theodur)
Definition

plasma cell leukaemia with systemic capillary leak syndrome, a rare disorder often associated with monoclonal gammopathy; antedated the diagnosis of plasma cell leukaemia by 5-6 months; During that time, he was repeatedly admitted to the hospital with weight gain, congestive cardiac failure, cough and anasarca in the presence of normal renal function, liver function and normal echocardiography; On presentation, a serum protein electrophoresis showed monoclonal IgG; the blood smear showed 60% plasma cells with a total count of 4_4 × 109/l; A bone marrow aspirate showed replacement of the normal marrow by sheets of immature plasma cells; His systemic capillary leak syndrome initially responded to decongestive therapy with terbutaline and aminophylline but later on he became refractory to them and responded to vincristine, doxorubicin and dexamethasone (VAD) combination therapy only transiently; Danocrine and oxypentiphylline, added during VAD chemotherapy, did not produce a durable response in capillary leak syndrome, which finally responded to autologous peripheral blood stem cell transplantation (PBSCT); After PBSCT, he remained free of capillary leak for 10 months; It is characterized by recurrent episodes of hypotension with hemoconcentration, hypoalbuminemia without albuminuria and generalized edema; These episodes are due to capillary hyperpermeability with massive extravasation of plasma containing macromolecules; Fifty-seven cases have been reported so far, patients" mean age is 46 years (ranging from 9 to 67 years); Both sexes are equally affected; Monoclonal gammopathy, generally an IgG class, is associated in 82% of cases; For some authors this gammopathy may play a role in pathogenesis, without evidence of amyloidosis; Each episode is often preceded by infection; Pathophysiology is largely misunderstood but cytokines (IL2), leukotrienes and endothelial apoptosis have been suggested to be involved in capillary hyperpermeability; Therefore, some cases secondary to treatment by recombinant IL2 have been reported; Each episode consists of two phases:

- The initial phase is the capillary leak phase, lasting from 1 to 4 days; Clinical features are abdominal pain, nausea, generalized edema (sometimes responsible for rhabdomyolysis) and hypotension that may result in cardiopulmonary collapse; Acute renal failure is due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis; - The second phase results in the recruitment of the initially extravasated fluid; Intravascular overload with polyuria and pulmonary edema often occur; Edema may be more severe due to massive fluid supply in the initial phase; Therefore, monitoring those patients is essential to timely switch to depletion treatment (including diuretics or hemofiltration); Mortality is reported in 21% of the 57 cases described; However, better management of this condition has recently led to lower mortality; A few prophylactic treatments have been tried with variable results, principally terbutaline and theophyllin; Patients need to be monitored for monoclonal gammopathy since it may turn into multiple myeloma----------[orphanet 2006]---------

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External Links Related to Capillary leak syndrome
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NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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