Prognostic significance of pulmonary lymphangioleiomyomatosis histologic score.
Correlations were made between clinical and follow-up data and histopathologic findings in 105 women (mean age +/- standard deviation, 38.3 +/- 9.0 years) with pulmonary lymphangioleiomyomatosis (LAM). The actuarial survival (to pulmonary transplantation or death) of the patients from the time of lung biopsy was 85.1% and 71.0% after 5 and 10 years respectively. The histologic severity of LAM, graded as a LAM histologic score (LHS), was determined on the basis of semiquantitative estimation of the percentage of tissue involvement by the two major features of LAM: the cystic lesions and the infiltration by abnormal smooth muscle cells (LAM cells) in each case: LHS-1, <25%; LHS-2, 25% to 50%; and LHS-3, >50%. Analysis using the Kaplan-Meier method revealed significant differences in survival for patients with LHS-1, -2, and -3 (p = 0.01). The 5-and 10-year survivals were 100% and 100% for LHS-1, 81.2% and 74.4% for LHS-2, and 62.8% and 52.4% for LHS-3. Increased degrees of accumulation of hemosiderin in macrophages also were associated with higher LHS scores (p = 0.029) and a worse prognosis (p = 0.0012). Thus, the current study suggests that the LHS may provide a basis for determining the prognosis of LAM.
Matsui
K
K
Pathology Section, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland, USA.
Beasley
M B
MB
Nelson
W K
WK
Barnes
P M
PM
Bechtle
J
J
Falk
R
R
Ferrans
V J
VJ
Moss
J
J
Travis
W D
WD
D000293:Adolescent; D000328:Adult; D000368:Aged; D003560:Cysts / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006485:Hemosiderin / Q000378:metabolism; D006486:Hemosiderosis / Q000473:pathology; D006801:Humans; D008175:Lung Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D008203:Lymphangiomyoma / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D008264:Macrophages / Q000378:metabolism / Q000473:pathology; D008875:Middle Aged; D009130:Muscle, Smooth / Q000473:pathology; D011379:Prognosis; D015996:Survival Rate
D006485:Hemosiderin