School of Medicine and Health Sciences Poster Presentations

Title

Fluctuating Monoclonal Gammopathy Protein levels in Idiopathic Systemic Capillary Leak Syndrome

Document Type

Poster

Keywords

MGUS; capillary leak; myeloma; SPEP; monoclonal gammopathy

Publication Date

Spring 2017

Abstract

Background: Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a rare condition characterized by hypotension, edema, and fluid extravasation with potentially life threatening complications including multi-organ failure1. It can be difficult to diagnose and differentiate from other causes of hypotension in severely ill patients. There is no well-defined treatment guidelines for ISCLS, but there is evidence that high dose monthly intravenous immunoglobulin (IVIG) can successfully be used to prevent recurrences2. Additionally, there are significant data suggesting an association between ISCLS and Monoclonal Gammopathy of Undetermined Significance (MGUS) 3-10.

Case Presentation: We present a case of a 39-year-old male with a history of several hospitalizations due to acute cardiogenic shock, with intermittent periods of complete recovery, eventually diagnosed with ISCLS after four years. After diagnosing ISCLS, further work up revealed IgG lambda free light chain elevation on serum electrophoresis (SPEP) consistent with MGUS. His first SPEP result showed 1 g/dl of protein, followed by 0.4 g/dl several months later, and then 1.91 g/dl several months after that. The most recent measurement was made 6 days after the patient received his monthly IVIG dose. Typically, a monoclonal protein level fluctuation to a higher value would signal MGUS progression, but in a patient with ISCLS who has received recent IVIG infusion, other differential diagnosis was sought.

The differential diagnosis at this time included false positive SPEP given recent IVIG treatment. The half-life of IVIG is 20-30 days, so in theory this might be possible, but, given that IVIG is polyclonal IgG obtained from a large group of donors, we concluded it is unlikely to cause monoclonal M spike on SPEP. Other possibilities included protein extravasation into interstitial tissues secondary to the ISCLS. And finally, the fluctuating SPEP reflected a true change in monoclonal IgG for which additional work up might be necessary.

Discussion: Data suggests that these patients progress to Multiple Myeloma at the same rates as non-ISCLS patients1. Therefore, hematologists must follow these patients’ SPEP values to monitor for progressive disease. Given the main stay of treatment for patients with ISCLS is monthly intravenous IVIG, this presents a challenge when interpreting the SPEP abnormal protein values. We would like to convey caution when interpreting the M protein and recommend conducting the SPEP testing prior to the monthly IVIG administration as this could possibly avoid unnecessary interventions in this population.

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Open Access

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Poster to be presented at GW Annual Research Days 2017.

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Fluctuating Monoclonal Gammopathy Protein levels in Idiopathic Systemic Capillary Leak Syndrome

Background: Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a rare condition characterized by hypotension, edema, and fluid extravasation with potentially life threatening complications including multi-organ failure1. It can be difficult to diagnose and differentiate from other causes of hypotension in severely ill patients. There is no well-defined treatment guidelines for ISCLS, but there is evidence that high dose monthly intravenous immunoglobulin (IVIG) can successfully be used to prevent recurrences2. Additionally, there are significant data suggesting an association between ISCLS and Monoclonal Gammopathy of Undetermined Significance (MGUS) 3-10.

Case Presentation: We present a case of a 39-year-old male with a history of several hospitalizations due to acute cardiogenic shock, with intermittent periods of complete recovery, eventually diagnosed with ISCLS after four years. After diagnosing ISCLS, further work up revealed IgG lambda free light chain elevation on serum electrophoresis (SPEP) consistent with MGUS. His first SPEP result showed 1 g/dl of protein, followed by 0.4 g/dl several months later, and then 1.91 g/dl several months after that. The most recent measurement was made 6 days after the patient received his monthly IVIG dose. Typically, a monoclonal protein level fluctuation to a higher value would signal MGUS progression, but in a patient with ISCLS who has received recent IVIG infusion, other differential diagnosis was sought.

The differential diagnosis at this time included false positive SPEP given recent IVIG treatment. The half-life of IVIG is 20-30 days, so in theory this might be possible, but, given that IVIG is polyclonal IgG obtained from a large group of donors, we concluded it is unlikely to cause monoclonal M spike on SPEP. Other possibilities included protein extravasation into interstitial tissues secondary to the ISCLS. And finally, the fluctuating SPEP reflected a true change in monoclonal IgG for which additional work up might be necessary.

Discussion: Data suggests that these patients progress to Multiple Myeloma at the same rates as non-ISCLS patients1. Therefore, hematologists must follow these patients’ SPEP values to monitor for progressive disease. Given the main stay of treatment for patients with ISCLS is monthly intravenous IVIG, this presents a challenge when interpreting the SPEP abnormal protein values. We would like to convey caution when interpreting the M protein and recommend conducting the SPEP testing prior to the monthly IVIG administration as this could possibly avoid unnecessary interventions in this population.