A case of myelodysplastic syndrome complicated by pulmonary alveolar proteinosis with a high serum KL-6 level.
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.
Department of Pediatrics, Kawasaki Medical School, Okayama, Japan.
T
T
Kajiume
S
S
Yoshimi
A
A
Nagita
K
K
Kobayashi
N
N
Kataoka
M
M
Nakajima
T
T
Matsushima
D000941:Antigens; D000951:Antigens, Neoplasm; D002648:Child; D005260:Female; D006023:Glycoproteins; D006801:Humans; D018396:Mucin-1; D009077:Mucins; D009190:Myelodysplastic Syndromes / Q000150:complications*; D010446:Peptide Fragments / Q000097:blood; D011347:Procollagen / Q000097:blood; D011649:Pulmonary Alveolar Proteinosis / Q000097:blood / Q000209:etiology*
D000941:Antigens; D000951:Antigens, Neoplasm; D006023:Glycoproteins; C060192:MUC1 protein, human; D018396:Mucin-1; D009077:Mucins; D010446:Peptide Fragments; D011347:Procollagen; C041876:procollagen Type III-N-terminal peptide