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Multiorgan dysfunction syndrome in sickle cell disease.

Sujata Hiran1

  • 1Department of Medicine, JLN Hospital and Research Centre, Bhilai.

The Journal of the Association of Physicians of India
|April 29, 2005
PubMed
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Multiorgan dysfunction syndrome (MODS) in sickle cell disease (SCD) is a rare but serious complication. Prompt and aggressive blood transfusions can reverse MODS, even without prior organ damage.

Area of Science:

  • Hematology
  • Critical Care Medicine
  • Genetics

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder.
  • Concurrent failure of multiple organs (MODS) is infrequently reported in SCD patients.

Purpose of the Study:

  • To investigate the occurrence and characteristics of multiorgan dysfunction syndrome (MODS) in patients experiencing sickle cell crisis.
  • To highlight the clinical presentation and outcomes of MODS in SCD.

Main Methods:

  • Retrospective analysis of ten episodes of MODS in SCD patients.
  • Organ failure defined using Acute Physiological and Chronic Health Evaluation - II (APACHE-II) criteria for at least two organs (lung, liver, or renal).

Main Results:

  • Most MODS episodes occurred during vaso-occlusive crisis, associated with severe pain, fever, and hematological changes.

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  • Respiratory, hepatic, and renal failure occurred concurrently in three patients; one patient died despite intensive support.
  • Aggressive blood transfusions led to improvement in most patients, except for two cases with severe complications.

    • Conclusions:

    • Acute MODS is a life-threatening complication of SCD.
    • MODS can manifest without pre-existing chronic organ damage.
    • Prompt and aggressive transfusion therapy is crucial for reversing MODS in SCD.