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August 14, 1987, Vol 258, No. 6 >
ARTICLE | August 14, 1987

New Hope for Neurofibromatosis? The Mast Cell Connection FREE

Henry N. Claman, MD
JAMA. 1987;258(6):823-823. doi:10.1001/jama.1987.03400060099039
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References

Neurofibromatosis is a common and progressive illness.1,2 The spectrum of disease varies from the mildest of formes frustes to highly destructive and disfiguring syndromes. (It now seems that the most famous putative severe example, that of Joseph Merrick, "The Elephant Man," was not neurofibromatosis at all.3) The primary, if not the only, treatment has been surgery.

In the August issue of the American Medical Association's Archives of Dermatology,4 Riccardi presents his early experience in treating neurofibromatosis with ketotifen, a mast cell stabilizer.5 During a three-year period, 15 patients received oral ketotifen. The conditions of 11 patients improved in one or more of the following variables: pain, itching, or the general feeling of well-being. In addition, most of these patients also had an apparent decrease in neurofibroma size, or a slowing of tumor growth, and a decrease in the profuse bleeding that accompanies surgery in this illness.

REFERENCES

1 +
CroweFW, Schull WJ, Neel JV:A Clinical, Pathological and Genetic Study of Multiple Neurofibromatosis . Springfield, Ill, Charles C Thomas Publishers, 1956;.
2 +
Riccardi VM, Eichner JE:  Pathogenesis , in Neurofibromatosis: Phenotype, Natural History, and Pathogenesis . Baltimore, The Johns Hopkins University Press, 1986;, pp 209-213.
3 +
Tibbles JAR, Cohen MM Jr:  The Proteus syndrome: The Elephant Man diagnosed . Br Med J 1986;;293:683-685.
4 +
Riccardi VM:  Mast cell stabilization to decrease neurofibroma growth: Preliminary experience with ketotifen . Arch Dermatol 1987;;123:1011-1016.
5 +
MacDonald GF:  An overview of ketotifen . Chest 1982;;82( (suppl) ):30s-32s.
6 +
Issacson P:  Mast cells in benign nerve sheath tumors . J Pathol 1975;;119: 193-196.
7 +
Cawley EP, Hoch-Ligeti C:  Association of tissue mast cells and skin tumors . Arch Dermatol 1961;;83:92-96.
8 +
Sylven B:  Ester sulphuric acids of high molecular weight and mast cells in mesenchymal tumors . Acta Radiol Scand 1945;;59( (suppl) ):1-99.
9 +
Kawanami O, Ferrans VJ, Falmer JD, et al:  Ultrastructure of pulmonary mast cells in patients with fibrotic lung disorders . Lab Invest 1979;;40:717-734.
10 +
Watanabe S, Watanabe K, Ohishi T, et al:  Mast cells in the rat alveolar septa undergoing fibrosis after ionizing irradiation . Lab Invest 1974;;31: 555-567.
11 +
Goto T, Befus D, Low R, et al:  Mast cell heterogeneity and hyperplasia in bleomycin-induced pulmonary fibrosis in rats . Am Rev Respir Dis 1984;;130: 797-802.
12 +
Wichmann B-E:  The mast cell count during the process of wound healing . Acta Pathol Microbiol Immunol Scand Suppl 1955;;108:1-35.
13 +
Fitzpatrick DW, Fisher H:  Histamine synthesis, imidazole dipeptides, and wound healing . Surgery 1982;;91:430-434.
14 +
Topol BM, Lewis VL, Benveniste K:  The use of antihistamine to retard the growth of fibroblasts derived from human skin, scar and keloid . Plast Reconstr Surg 1981;;68:227-232.
15 +
Jaffee BD, Claman HN:  Chronic graft-versus-host disease (GVHD) as a model for scleroderma: I. Description of model systems . Cell Immunol 1983;;77:1-12.
16 +
Claman HN:  Mast cell depletion in murine chronic graft-versus-host disease . J Invest Dermatol 1985;;84:246-248.
17 +
Giorno R, Lee Choi K, Claman HN:  Simultaneous in situ detection of IgE receptors and cytoplasmic granules in murine cutaneous mast cells . J Immunol Methods 1987;;99:163-166.
18 +
Hawkins RA, Claman HN, Clark RAF, et al:  Increased dermal mast cell populations in progressive systemic sclerosis: A link in chronic fibrosis? Ann Intern Med 1985;;102:182-186.
19 +
Nishioka K, Kobayashi Y, Katayama I, et al:  Mast cell numbers in diffuse scleroderma . Arch Dermatol 1987;;123:205-208.
20 +
Claman HN:  Mast cells, T cells and abnormal fibrosis . Immunol Today 1985;;6:192-195.
21 +
Atkins FM, Clark RAF:  Mast cells and fibrosis . Arch Dermatol 1987;;123: 191-193.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
CroweFW, Schull WJ, Neel JV:A Clinical, Pathological and Genetic Study of Multiple Neurofibromatosis . Springfield, Ill, Charles C Thomas Publishers, 1956;.
2 +
Riccardi VM, Eichner JE:  Pathogenesis , in Neurofibromatosis: Phenotype, Natural History, and Pathogenesis . Baltimore, The Johns Hopkins University Press, 1986;, pp 209-213.
3 +
Tibbles JAR, Cohen MM Jr:  The Proteus syndrome: The Elephant Man diagnosed . Br Med J 1986;;293:683-685.
4 +
Riccardi VM:  Mast cell stabilization to decrease neurofibroma growth: Preliminary experience with ketotifen . Arch Dermatol 1987;;123:1011-1016.
5 +
MacDonald GF:  An overview of ketotifen . Chest 1982;;82( (suppl) ):30s-32s.
6 +
Issacson P:  Mast cells in benign nerve sheath tumors . J Pathol 1975;;119: 193-196.
7 +
Cawley EP, Hoch-Ligeti C:  Association of tissue mast cells and skin tumors . Arch Dermatol 1961;;83:92-96.
8 +
Sylven B:  Ester sulphuric acids of high molecular weight and mast cells in mesenchymal tumors . Acta Radiol Scand 1945;;59( (suppl) ):1-99.
9 +
Kawanami O, Ferrans VJ, Falmer JD, et al:  Ultrastructure of pulmonary mast cells in patients with fibrotic lung disorders . Lab Invest 1979;;40:717-734.
10 +
Watanabe S, Watanabe K, Ohishi T, et al:  Mast cells in the rat alveolar septa undergoing fibrosis after ionizing irradiation . Lab Invest 1974;;31: 555-567.
11 +
Goto T, Befus D, Low R, et al:  Mast cell heterogeneity and hyperplasia in bleomycin-induced pulmonary fibrosis in rats . Am Rev Respir Dis 1984;;130: 797-802.
12 +
Wichmann B-E:  The mast cell count during the process of wound healing . Acta Pathol Microbiol Immunol Scand Suppl 1955;;108:1-35.
13 +
Fitzpatrick DW, Fisher H:  Histamine synthesis, imidazole dipeptides, and wound healing . Surgery 1982;;91:430-434.
14 +
Topol BM, Lewis VL, Benveniste K:  The use of antihistamine to retard the growth of fibroblasts derived from human skin, scar and keloid . Plast Reconstr Surg 1981;;68:227-232.
15 +
Jaffee BD, Claman HN:  Chronic graft-versus-host disease (GVHD) as a model for scleroderma: I. Description of model systems . Cell Immunol 1983;;77:1-12.
16 +
Claman HN:  Mast cell depletion in murine chronic graft-versus-host disease . J Invest Dermatol 1985;;84:246-248.
17 +
Giorno R, Lee Choi K, Claman HN:  Simultaneous in situ detection of IgE receptors and cytoplasmic granules in murine cutaneous mast cells . J Immunol Methods 1987;;99:163-166.
18 +
Hawkins RA, Claman HN, Clark RAF, et al:  Increased dermal mast cell populations in progressive systemic sclerosis: A link in chronic fibrosis? Ann Intern Med 1985;;102:182-186.
19 +
Nishioka K, Kobayashi Y, Katayama I, et al:  Mast cell numbers in diffuse scleroderma . Arch Dermatol 1987;;123:205-208.
20 +
Claman HN:  Mast cells, T cells and abnormal fibrosis . Immunol Today 1985;;6:192-195.
21 +
Atkins FM, Clark RAF:  Mast cells and fibrosis . Arch Dermatol 1987;;123: 191-193.

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