A case of myelodysplastic syndrome complicated by pulmonary alveolar proteinosis with a high serum KL-6 level

Pediatr Hematol Oncol. Jul-Aug 1999;16(4):367-71. doi: 10.1080/088800199277218.

Abstract

Serious hematological diseases often cause respiratory disorders. Because these are related to the prognoses of patients with hematological diseases, their early diagnosis is necessary. This study describes a 6-year-old girl with myelodysplastic syndrome complicated by pulmonary alveolar proteinosis who showed a remarkable increase in her serum KL-6 level. Three years and 2 months after the end of therapy for neonatal melanoma, a diagnosis of myelodysplastic syndrome with leukemic change was made. Ten months after the onset of leukemia, she had respiratory distress with an increased serum KL-6 level of 75,000 U/mL (reference range; < 500 U/mL). Despite various treatments for pulmonary complications, she died 3 months after developing respiratory distress. A diagnosis of pulmonary alveolar proteinosis was made at autopsy. Earlier treatment of respiratory distress could be achieved if serum KL-6 levels were examined earlier.

Publication types

  • Case Reports

MeSH terms

  • Antigens
  • Antigens, Neoplasm
  • Child
  • Female
  • Glycoproteins
  • Humans
  • Mucin-1
  • Mucins
  • Myelodysplastic Syndromes / complications*
  • Peptide Fragments / blood
  • Procollagen / blood
  • Pulmonary Alveolar Proteinosis / blood
  • Pulmonary Alveolar Proteinosis / etiology*

Substances

  • Antigens
  • Antigens, Neoplasm
  • Glycoproteins
  • MUC1 protein, human
  • Mucin-1
  • Mucins
  • Peptide Fragments
  • Procollagen
  • procollagen Type III-N-terminal peptide