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Bone marrow necrosis successfully treated with corticosteroid.

Alfredo N C Santana1, Renata G Ramos, Eduardo F Zanandrea

  • 1Pulmonary Division, University of São Paulo Medical School, São Paulo, Brazil. alfredonicodemos@hotmail.com

European Journal of Haematology
|December 23, 2004
PubMed
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This study reports the first case of bone marrow necrosis (BMN) in a patient with CREST syndrome. Intravenous pulse corticosteroids effectively treated severe anemia and pancytopenia, resolving BMN symptoms.

Area of Science:

  • Hematology
  • Rheumatology
  • Immunology

Background:

  • CREST syndrome is a rare autoimmune disorder affecting connective tissues.
  • Idiopathic portal hypertension and esophageal varices can complicate CREST syndrome.
  • Bone marrow necrosis (BMN) is a rare hematologic condition with significant morbidity.

Observation:

  • A 60-year-old woman with CREST syndrome presented with severe anemia and pancytopenia.
  • Bone marrow biopsy revealed extensive bone marrow necrosis.
  • The patient required frequent erythrocyte transfusions due to severe anemia.

Findings:

  • Treatment with intravenous pulse methylprednisolone followed by oral prednisone led to rapid hematologic recovery.
  • Hemoglobin levels normalized, and platelet and white blood cell counts significantly improved.

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  • This is the first reported case of BMN in a patient with CREST syndrome.

    • Implications:

    • Corticosteroid therapy appears effective in managing BMN associated with CREST syndrome.
    • The findings suggest a potential immune-mediated mechanism underlying BMN in this context.
    • This case highlights the importance of considering BMN in patients with CREST syndrome presenting with cytopenias.