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Pure White Cell Aplasia and Necrotizing Myositis

Peter Geon Kim1, Joome Suh1, Max W Adelman1

  • 1Department of Medicine, Massachusetts General Hospital, Boston, MA 02114, USA.

Case Reports in Hematology
|April 14, 2016
PubMed

Insights

Pure white cell aplasia (PWCA), a rare blood disorder, can be triggered by medications. This case highlights a patient whose PWCA improved with specific therapies after drug exposure.

Area of Science:

  • Hematology
  • Internal Medicine
  • Pharmacology

Background:

  • Pure white cell aplasia (PWCA) is a rare hematologic disorder.
  • It is characterized by absent neutrophil lineages in bone marrow.
  • PWCA is linked to autoimmune, drug-induced, and viral causes.

Purpose of the Study:

  • To report a case of PWCA.
  • To investigate the association with drug exposure and myositis.
  • To describe treatment outcomes.

Main Methods:

  • Case report of a 74-year-old female.
  • Biopsies revealed PWCA, necrotizing myositis, and acute liver injury.
  • Treatment included prednisone, intravenous immunoglobulin, and G-CSF.

Main Results:

  • The patient presented with proximal weakness.
  • Findings were associated with azithromycin and statin use.
  • Myositis resolved with prednisone, but PWCA persisted.
  • Intravenous immunoglobulin and G-CSF improved neutrophil counts and symptoms.

Conclusions:

  • PWCA can be associated with drug exposure, myositis, and liver injury.
  • Combination therapy with IVIG and G-CSF can be effective for drug-induced PWCA.
  • This case expands understanding of PWCA triggers and management.

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