JOURNAL OF PATHOLOGY, VOL.
166: 395-404 (1992)
DEGRANULATION OF CYSTIC FIBROSIS NASAL POLYP MAST CELLS WILLIAM R. HENDERSON, JR* AND EMIL Y. CHI?
Depurtments of *Medicine and tPuthology, University of Wushington School of Medicine, Seuttle, Wushington, U . S . A . Received 26 June 1991 Accepted27 August 1991
SUMMARY The ultrastructure of nasal polyps from cystic fibrosis (CF) patients was compared with non-CF nasal polyps in this study. Morphometric analysis showed that C F nasal polyps contained greater numbers of mast cells, endothelial cells, lymphocytes, and plasma cells compared with the non-CF specimens. Morphologic evidence of degranulation was seen in approximately 30 per cent of the C F mast cells but was not observed in the non-CF mast cells. Increased numbers of small granules, vacuolated granules, and lipid bodies were noted in the C F compared with the non-CF nasal polyp mast cells. Also observed was a decrease in collagen in the extracellular space of the C F nasal polyps compared with the nonC F specimens. Although eosinophils were observed in the non-CF nasal polyp tissue, these leukocytes were absent in the C F nasal polyps. These data indicate that striking morphologic differences exist between C F and non-CF nasal polyps with mast cell degranulation, a salient feature of C F specimens. KEY
WORDS-cystic
fibrosis, nasal polyps, mast cells, degranulation.
INTRODUCTION Cystic fibrosis (CF) is the most common lethal inherited disease in Caucasians. The C F gene has recently been identified and localized on chromosome 7. An autosomal recessive disorder, C F is of heterogeneous genotype. Pancreatic insufficiency is the most prominent clinical symptom of C F in infancy and early childhood, whereas pulmonary disease predominates as the principal cause of morbidity and mortality in older children and young adult^.^ Although nasal polyps rarely occur in children without CF, they are frequently observed in children with CF.'-'' Several studies have examined the histological features of C F nasal polyps by light microscopy.8," C F nasal polyps consist of oedematous submucosal nasal tissue containing hyperplastic mucus glands. These glands produce an acid mucin which differs from the neutral mucin produced by hyperplastic glands of non-CF nasal polyp^.^ In this study, we compared the ultrastructure of nasal polyps Addressee for correspondence: Dr W. R. Henderson, Jr, Department of Medicine, SJ-lo, University of Washington, Seattle, WA 98195, U.S.A.
0022-341 7/92/040395-10 $05.00 0 1992 by John Wiley & Sons, Ltd.
from C F and non-CF patients by transmission electron microscopy. As determined by morphometric analysis, we found an increased number of mast cells and a high rate of spontaneous degranulation of these cells in the C F compared with the non-CF Table I-Cell composition of nasal polyps from C F and non-CF patients* Cell type
CF
Non-CF
P
5.9k 1.1 2.8 i- 0.7 1.8 i- 0.3 1 1.9 0.8 4.9 & 0.7 16.0k 1.9 8 . 3 k 1.1 5.8f 1.1
166: 395-404 (1992)
DEGRANULATION OF CYSTIC FIBROSIS NASAL POLYP MAST CELLS WILLIAM R. HENDERSON, JR* AND EMIL Y. CHI?
Depurtments of *Medicine and tPuthology, University of Wushington School of Medicine, Seuttle, Wushington, U . S . A . Received 26 June 1991 Accepted27 August 1991
SUMMARY The ultrastructure of nasal polyps from cystic fibrosis (CF) patients was compared with non-CF nasal polyps in this study. Morphometric analysis showed that C F nasal polyps contained greater numbers of mast cells, endothelial cells, lymphocytes, and plasma cells compared with the non-CF specimens. Morphologic evidence of degranulation was seen in approximately 30 per cent of the C F mast cells but was not observed in the non-CF mast cells. Increased numbers of small granules, vacuolated granules, and lipid bodies were noted in the C F compared with the non-CF nasal polyp mast cells. Also observed was a decrease in collagen in the extracellular space of the C F nasal polyps compared with the nonC F specimens. Although eosinophils were observed in the non-CF nasal polyp tissue, these leukocytes were absent in the C F nasal polyps. These data indicate that striking morphologic differences exist between C F and non-CF nasal polyps with mast cell degranulation, a salient feature of C F specimens. KEY
WORDS-cystic
fibrosis, nasal polyps, mast cells, degranulation.
INTRODUCTION Cystic fibrosis (CF) is the most common lethal inherited disease in Caucasians. The C F gene has recently been identified and localized on chromosome 7. An autosomal recessive disorder, C F is of heterogeneous genotype. Pancreatic insufficiency is the most prominent clinical symptom of C F in infancy and early childhood, whereas pulmonary disease predominates as the principal cause of morbidity and mortality in older children and young adult^.^ Although nasal polyps rarely occur in children without CF, they are frequently observed in children with CF.'-'' Several studies have examined the histological features of C F nasal polyps by light microscopy.8," C F nasal polyps consist of oedematous submucosal nasal tissue containing hyperplastic mucus glands. These glands produce an acid mucin which differs from the neutral mucin produced by hyperplastic glands of non-CF nasal polyp^.^ In this study, we compared the ultrastructure of nasal polyps Addressee for correspondence: Dr W. R. Henderson, Jr, Department of Medicine, SJ-lo, University of Washington, Seattle, WA 98195, U.S.A.
0022-341 7/92/040395-10 $05.00 0 1992 by John Wiley & Sons, Ltd.
from C F and non-CF patients by transmission electron microscopy. As determined by morphometric analysis, we found an increased number of mast cells and a high rate of spontaneous degranulation of these cells in the C F compared with the non-CF Table I-Cell composition of nasal polyps from C F and non-CF patients* Cell type
CF
Non-CF
P
5.9k 1.1 2.8 i- 0.7 1.8 i- 0.3 1 1.9 0.8 4.9 & 0.7 16.0k 1.9 8 . 3 k 1.1 5.8f 1.1
