Short- and long-term outcomes of AL amyloidosis patients admitted into intensive care units

Br J Haematol. 2016 Sep;174(6):868-75. doi: 10.1111/bjh.14135. Epub 2016 Jun 12.

Abstract

Amyloidosis is a rare and threatening condition that may require intensive care because of amyloid deposit-related organ dysfunction or therapy-related adverse events. Although new multiple myeloma drugs have dramatically improved outcomes in AL amyloidosis, the outcomes of AL patients admitted into intensive care units (ICUs) remain largely unknown. Admission has been often restricted to patients with low Mayo Clinic staging and/or with a complete or very good immunological response at admission. In a retrospective multicentre cohort of 66 adult AL (n = 52) or AA (n = 14) amyloidosis patients, with similar causes of admission to an ICU, the 28-d and 6-month survival rates of AA patients were significantly higher compared to AL patients (93% vs. 60%, P = 0·03; 71% vs. 45%, P = 0·02, respectively). In AL patients, the simplified Index of Gravity Score (IGS2) was the only independent predictive factor for death by day 28, whereas the Mayo-Clinic classification stage had no influence. In Cox's multivariate regression model, only cardiac arrest and on-going chemotherapy at ICU admission significantly predicted death at 6 months. Short-term outcomes of AL patients admitted into an ICU were mainly related to the severity of the acute medical condition, whereas on-going chemotherapy for active amyloidosis impacted on long-term outcomes.

Keywords: amyloidosis; intensive care unit; monoclonal gammopathy; outcome.

MeSH terms

  • Adult
  • Aged
  • Amyloidosis / blood
  • Amyloidosis / diagnosis
  • Amyloidosis / epidemiology*
  • Critical Care*
  • Disease Management
  • Female
  • Hospital Mortality
  • Humans
  • Immunoglobulin Light Chains / blood
  • Intensive Care Units*
  • Male
  • Middle Aged
  • Patient Admission
  • Patient Outcome Assessment
  • Prognosis
  • Risk Assessment
  • Severity of Illness Index
  • Survival Analysis

Substances

  • Immunoglobulin Light Chains