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Kidney Week

Abstract: TH-PO0049

AKI from Capillary Leak Syndrome in a Patient with a Solitary Kidney

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Plick, Natalie, Icahn School of Medicine at Mount Sinai Department of Medicine, New York, New York, United States
  • Natarajan, Hariharasudan, Icahn School of Medicine at Mount Sinai, New York, New York, United States
  • Abramson, Matthew, Icahn School of Medicine at Mount Sinai, New York, New York, United States
Introduction

Systemic capillary leak syndrome (SCLS) is a vasodilatory state wherein increased capillary permeability results in edema, hemoconcentration, and low effective circulating volume. SCLS is a toxic effect of Tagraxofusp (Tag), a CD123-directed cytotoxin used in the treatment of a rare and aggressive acute leukemia called blastic plasmacytoid dendritic cell neoplasm (BPDCN). SCLS can cause organ failure including acute kidney injury (AKI) that is usually hemodynamic in etiology. Volume status and resuscitation must be managed judiciously in SCLS due to the propensity for third spacing and pulmonary edema. There is mixed guidance on fluid administration in SCLS and no reports of Tag-associated AKI to guide management. Here we present a case of Tag-associated SCLS and AKI that is poorly responsive to albumin in a patient with a solitary kidney.

Case Description

A 68-year old male with CKD3aA1 from a left nephrectomy (for donation in 2004) and recently diagnosed BPDCN was admitted for Tag induction therapy. After the first dose of Tag he developed KDIGO Stage 1 AKI, weight gain, edema, and hemoconcentration consistent with SCLS. Tag was held and albumin administered as per drug protocol, but his AKI was refractory to albumin. Diagnostic testing suggested a sodium-avid prerenal state and kidney function promptly recovered with crystalloid administration.

Discussion

Given its rarity and poorly understood pathophysiology, there is minimal evidence to guide management of Tag-associated AKI. Consensus exists that patients with SCLS should not be aggressively resuscitated as one would in sepsis, but evidence-based strategies for fluid management are lacking. Declining serum albumin levels predict Tag-associated SCLS, and albumin resuscitation remains a cornerstone of management. However as shown in this case there is likely a role for isotonic fluid if AKI is refractory to albumin. As new oncological therapies emerge nephrologists will be called upon to care for nephrotoxicities that may have limited evidence-base to guide management. This case illustrates how returning to physiological principles can inform decision-making.

Digital Object Identifier (DOI)