{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:23:49Z","timestamp":1760189029698},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"612","license":[{"start":{"date-parts":[[1976,10,1]],"date-time":"1976-10-01T00:00:00Z","timestamp":212976000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1976,10,1]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>Systemic mastocytosis is uncommon. A patient is reported with many of the characteristic lesions of this disorder but with two features not before described\u2014the occurrence of mixed pyramidal and neuromyopathic neurological signs and the demonstration of lymph nodes with abnormal architecture by lymphography.<\/jats:p>","DOI":"10.1136\/pgmj.52.612.659","type":"journal-article","created":{"date-parts":[[2008,12,29]],"date-time":"2008-12-29T20:31:39Z","timestamp":1230582699000},"page":"659-662","source":"Crossref","is-referenced-by-count":9,"title":["Systemic mastocytosis\u2014central nervous system features and lymphographic demonstration of lymph node involvement"],"prefix":"10.1093","volume":"52","author":[{"given":"B W","family":"Hancock","sequence":"first","affiliation":[{"name":"Academic Division of Medicine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P","family":"Dandona","sequence":"additional","affiliation":[{"name":"Academic Division of Medicine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D C","family":"Cumberland","sequence":"additional","affiliation":[{"name":"Department of Radiology, The Royal Hospital , Sheffield S1 3SR"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J A","family":"Jarratt","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurophysiology , Fulwood Annexe, Sheffield S10 3TD"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[1976,10,1]]},"reference":[{"key":"ref_1","first-page":"136","article-title":"Une affection nouvelle, sans doute congenitale, des voies biliaires; la dilatation kystique unilobaire des canaux hepatiques","volume":"14","author":"CAROLI, S.; COUINEAUD, C.","year":"1958","unstructured":"CAROLI, S. & COUINEAUD, C. (1958) Une affection nouvelle, sans doute congenitale, des voies biliaires; la dilatation kystique unilobaire des canaux hepatiques. Semaine des Hopitaux de Paris, 14, 136.","journal-title":"Semaine des Hopitaux de Paris"},{"key":"ref_2","first-page":"29","article-title":"Cirrhosis, Gram-negative sepsis and chloramphenicol toxicity","volume":"45","author":"LAZAR, A.J.","year":"1966","unstructured":"LAZAR, A.J. (1966) Cirrhosis, Gram-negative sepsis and chloramphenicol toxicity. American Journal of Gastroenterology, 45, 29.","journal-title":"American Journal of Gastroenterology"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"21","DOI":"10.2214\/ajr.113.1.21","article-title":"Communicating cavernous ectasia of the intrahepatic ducts (Caroli's disease)","volume":"113","author":"MUJETED, L.; GLENN, F.; EVANS, J.A.","year":"1971","unstructured":"MUJETED, L., GLENN, F. & EVANS, J.A. (1971) Communicating cavernous ectasia of the intrahepatic ducts (Caroli's disease). American Journal of Roentgenology, 113, 21.","journal-title":"American Journal of Roentgenology"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1093\/infdis\/125.2.170","article-title":"Treatment of Salmonella typhi carriers with intravenous ampicillin","volume":"125","author":"SCIOLI, C.; FIORENTINO, F.; SASSO, G.","year":"1972","unstructured":"SCIOLI, C., FIORENTINO, F. & SASSO, G. (1972) Treatment of Salmonella typhi carriers with intravenous ampicillin. Journal of Infectious Diseases, 125, 170.","journal-title":"Journal of Infectious Diseases"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1093\/infdis\/124.Supplement_1.S106","article-title":"Gentamicin and ampicillin in human bile","volume":"124","author":"SMITHIVES, T.; HYAMS, P.J.; PATIOL, J.J.","year":"1971","unstructured":"SMITHIVES, T., HYAMS, P.J. & PATIOL, J.J. (1971) Gentamicin and ampicillin in human bile. Journal ofInfectious Diseases. 124, 106.","journal-title":"Journal ofInfectious Diseases"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1016\/S0016-5085(75)80061-8","article-title":"Salmonella heidelberg infection in Caroli's syndrome","volume":"68","author":"WALDRAM, R.M.R.; VAHRMAN, J.; WILLIAMS, R.","year":"1975","unstructured":"WALDRAM, R.M.R., VAHRMAN, J. & WILLIAMS, R. (1975) Salmonella heidelberg infection in Caroli's syndrome. Gastroenterology. 68, 151.","journal-title":"Gastroenterology"},{"key":"ref_7","first-page":"659","article-title":"Postgraduate Medical Journal","volume":"52","year":"1976","unstructured":"Postgraduate Medical Journal (October 1976) 52, 659-662."},{"key":"ref_8","article-title":"Case report A female patient (Fig","unstructured":"Case report A female patient (Fig. 1) presented in 1967 aged"},{"key":"ref_9","article-title":"Histological examination of a muscle biopsy specimen showed generalized atrophy of muscle fibres. In the oxidative enzyme stains the type II fibres particularly were atrophied and many had a mottled appearance. There were also a few groups of atrophied fibres with a motor-unit type of distribution. The appearances were of a non-specific myopathy with evidence of a neurogenic component. Occasional mast cells were found between muscle fibres but there was no evidence of any other inflammatory reaction. In January 1975 she developed gross abdominal ascites. No abnormal cells were found in the ascitic fluid (sp.g. 1P3; protein content 55 g\/l). Repeated paracentesis and administration of frusemide and spironolactone have given symptomatic improvement. High dose prednisolone therapy has been restarted, again with reduction of spleen size and partial remission of disease progress","unstructured":"Histological examination of a muscle biopsy specimen showed generalized atrophy of muscle fibres. In the oxidative enzyme stains the type II fibres particularly were atrophied and many had a mottled appearance. There were also a few groups of atrophied fibres with a motor-unit type of distribution. The appearances were of a non-specific myopathy with evidence of a neurogenic component. Occasional mast cells were found between muscle fibres but there was no evidence of any other inflammatory reaction. In January 1975 she developed gross abdominal ascites. No abnormal cells were found in the ascitic fluid (sp.g. 1P3; protein content 55 g\/l). Repeated paracentesis and administration of frusemide and spironolactone have given symptomatic improvement. High dose prednisolone therapy has been restarted, again with reduction of spleen size and partial remission of disease progress."},{"key":"ref_10","article-title":"Discussion Mastocytosis, an abnormal proliferation of tissue mast cells, may involve skin, lymph nodes, viscera, bones and the entire reticulo-endothelial system","year":"1967","unstructured":"Discussion Mastocytosis, an abnormal proliferation of tissue mast cells, may involve skin, lymph nodes, viscera, bones and the entire reticulo-endothelial system (Gonella, 1967)."},{"key":"ref_11","article-title":"The unusual feature about the patient was the presence of widespread neurological abnormalities which have not before been described in systemic mastocytosis. Clinically she showed evidence of proximal myopathy, bilateral pyramidal tract involvement, sensory loss and some loss of sphincteric control. Electrophysiological tests confirmed a mixed neuromyopathic picture. These features may be attributed either to diffuse, patchy intra-medullary infiltration of spinal cord and brain stem with mast cells or to an indirect effect similar","volume-title":"She was treated with prednisolone and although this may cause myopathy (Afifi, Bergman and Harvey, 1968) such disseminated neurological findings have not been described","year":"1971","unstructured":"The unusual feature about the patient was the presence of widespread neurological abnormalities which have not before been described in systemic mastocytosis. Clinically she showed evidence of proximal myopathy, bilateral pyramidal tract involvement, sensory loss and some loss of sphincteric control. Electrophysiological tests confirmed a mixed neuromyopathic picture. These features may be attributed either to diffuse, patchy intra-medullary infiltration of spinal cord and brain stem with mast cells or to an indirect effect similar to that observed in association with malignant disease (Wilkinson, Croft and Urich, 1971). She was treated with prednisolone and although this may cause myopathy (Afifi, Bergman and Harvey, 1968) such disseminated neurological findings have not been described."},{"key":"ref_12","article-title":"Lymph node involvement in systemic mastocytosis may be regional or generalized (Havard and Bodley Scott, 1959). Bipedal lymphography in the patient demonstrated dilated lymphatics (Fig. 4a) some of which still contained contrast after 24 hr (Fig. 4b) indicating obstruction. The iliac and paraaortic glands to the level of L4 were normal in size but showed multiple marginal filling defects of varying size, giving the glands an irregular outline (Fig. 4b). The changes do not resemble those seen in the lymphomas or in secondary carcinoma but similar appearances are sometimes present in patients with collagen-vascular disease","unstructured":"Lymph node involvement in systemic mastocytosis may be regional or generalized (Havard and Bodley Scott, 1959). Bipedal lymphography in the patient demonstrated dilated lymphatics (Fig. 4a) some of which still contained contrast after 24 hr (Fig. 4b) indicating obstruction. The iliac and paraaortic glands to the level of L4 were normal in size but showed multiple marginal filling defects of varying size, giving the glands an irregular outline (Fig. 4b). The changes do not resemble those seen in the lymphomas or in secondary carcinoma but similar appearances are sometimes present in patients with collagen-vascular disease."},{"key":"ref_13","article-title":"Only one other report has been found of lymphography in mastocytosis (Munro, Craig and Feiwel, 1969). The changes described are quite different from those seen in the present patient with 'symmetrically enlarged glands of normal architecture but a greatly thickened cortex and medulla'. The typical lymphographic changes of mastocytosis are therefore not known and future reports will be of great interest","unstructured":"Only one other report has been found of lymphography in mastocytosis (Munro, Craig and Feiwel, 1969). The changes described are quite different from those seen in the present patient with 'symmetrically enlarged glands of normal architecture but a greatly thickened cortex and medulla'. The typical lymphographic changes of mastocytosis are therefore not known and future reports will be of great interest."},{"key":"ref_14","article-title":"Haematological disturbance is common-anaemia, leucopenia, monocytosis, thrombocytopenia and bleeding diathesis","volume-title":"Mast infiltration of bone marrow, a marrow depressant action of mast cell released substances (e.g. heparin) and hypersplenism have all been incriminated in these blood abnormalities. Circulating heparin was detected in the patient, bone marrow infiltration was marked and red cell survival significantly reduced. Marrow involvement and hypersplenism would adequately explain her anaemia and thrombocytopenia. There were no haematological changes of tissue mast cell leukaemia","author":"(Efrati, Klajman and Spitz,","year":"1967","unstructured":"Haematological disturbance is common-anaemia, leucopenia, monocytosis, thrombocytopenia and bleeding diathesis have all been described (Gonella, 1967). Mast infiltration of bone marrow, a marrow depressant action of mast cell released substances (e.g. heparin) and hypersplenism have all been incriminated in these blood abnormalities. Circulating heparin was detected in the patient, bone marrow infiltration was marked and red cell survival significantly reduced. Marrow involvement and hypersplenism would adequately explain her anaemia and thrombocytopenia. There were no haematological changes of tissue mast cell leukaemia (Efrati, Klajman and Spitz, 1957)."},{"key":"ref_15","article-title":"The skin (Nettleship, 1869) and bone (Sagher, Cohen and Schorr, 1952) changes of mastocytosis are well recognized. The present patient had characteristic involvement of both tissues","unstructured":"The skin (Nettleship, 1869) and bone (Sagher, Cohen and Schorr, 1952) changes of mastocytosis are well recognized. The present patient had characteristic involvement of both tissues."},{"key":"ref_16","article-title":"Systemic mastocytosis, particularly with reticuloendothelial involvement may be progressive and lethal","volume-title":"Antimitotic drugs and radiotherapy have not proved helpful in treatment and whilst corticosteroid therapy may give temporary benefit this is not curative (Sagher and Even-Pas, 1960). Certainly, the condition of the patient described here continues to deteriorate even though there has been partial response to steroid therapy","year":"1967","unstructured":"Systemic mastocytosis, particularly with reticuloendothelial involvement may be progressive and lethal (Gonella, 1967). Antimitotic drugs and radiotherapy have not proved helpful in treatment and whilst corticosteroid therapy may give temporary benefit this is not curative (Sagher and Even-Pas, 1960). Certainly, the condition of the patient described here continues to deteriorate even though there has been partial response to steroid therapy."},{"key":"ref_17","first-page":"158","article-title":"Steroid myopathy","volume":"123","author":"AFIFI, A.K.; BERGMAN, R.A.; HARVEY, J.C.","year":"1968","unstructured":"AFIFI, A.K., BERGMAN, R.A. & HARVEY, J.C. (1968) Steroid myopathy. Johns Hopkins Medical Journal, 123, 158.","journal-title":"Johns Hopkins Medical Journal"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1182\/blood.V12.10.869.869","article-title":"Mast cell leukaemia-malignant mastocytosis and blood basophilia","volume":"12","author":"EFRATI, P.; KLAJMAN, A.; SPITZ, H.","year":"1957","unstructured":"EFRATI, P., KLAJMAN, A. & SPITZ, H. (1957) Mast cell leukaemia-malignant mastocytosis and blood basophilia. Blood, 12, 869.","journal-title":"Blood"},{"key":"ref_19","first-page":"281","article-title":"Mast cell disease","volume":"3","author":"GONELLA, J.S.","year":"1967","unstructured":"GONELLA, J.S. (1967) Mast cell disease. Progress in Clinical Cancer, 3, 281.","journal-title":"Progress in Clinical Cancer"},{"key":"ref_20","first-page":"459","article-title":"Urticaria pigmentosa with visceral and skeletal lesions","volume":"28","author":"HAVARD, C.W.H.; SCOTT, B.O.D.L.E.Y.; R.","year":"1959","unstructured":"HAVARD, C.W.H. & BODLEY SCOTT, R. (1959) Urticaria pigmentosa with visceral and skeletal lesions. Quarterly Journal of Medicine, 28, 459.","journal-title":"Quarterly Journal of Medicine"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1111\/j.1365-2133.1969.tb16203.x","article-title":"Lymphangiography in dermatology","volume":"81","year":"1969","unstructured":"MUNRO, D.D., CRAIG, 0. & FEIWEL, M. (1969) Lymphangiography in dermatology. British Journal of Dermatology, 81, 652.","journal-title":"British Journal of Dermatology"},{"key":"ref_22","first-page":"323","article-title":"Rare forms of urticaria","volume":"2","author":"NETTLESHIP, E.","year":"1869","unstructured":"NETTLESHIP, E. (1869) Rare forms of urticaria. British Medical Journal, 2, 323.","journal-title":"British Medical Journal"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1038\/jid.1952.51","article-title":"Concomitant bone changes in urticaria pigmentosa","volume":"18","author":"SAGHER, F.; COHEN, C.; SCHORR, S.","year":"1952","unstructured":"SAGHER, F., COHEN, C. & SCHORR, S. (1952) Concomitant bone changes in urticaria pigmentosa. Journal of Investigative Dermatology, 18, 425.","journal-title":"Journal of Investigative Dermatology"},{"key":"ref_24","first-page":"268","article-title":"Mastocytosis. In: Cutaneous Manifestations of Reticulo-Endothelial Granulomas","author":"SAGHER, F.; EVEN-PAZ, Z.","year":"1960","unstructured":"SAGHER, F. & EVEN-PAZ, Z. (1960) Mastocytosis. In: Cutaneous Manifestations of Reticulo-Endothelial Granulomas (Ed. by S. M. Bluefarb), p. 268. Thomas, Illinois."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1177\/003591576706000722","article-title":"The remote effects of cancer on the nervous system","volume":"60","author":"WILKINSON, M.; CROFT, P.B.; URICH, H.","year":"1967","unstructured":"WILKINSON, M., CROFT, P.B. & URICH, H. (1967) The remote effects of cancer on the nervous system. Proceedings of Royal Society of Medicine, 60, 683.","journal-title":"Proceedings of Royal Society of Medicine"}],"container-title":["Postgraduate Medical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/52\/612\/659\/50185176\/postgradmedj-52-659.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/52\/612\/659\/50185176\/postgradmedj-52-659.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,8]],"date-time":"2023-05-08T06:47:37Z","timestamp":1683528457000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/pmj\/article\/52\/612\/659\/7038078"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1976,10,1]]},"references-count":25,"journal-issue":{"issue":"612","published-online":{"date-parts":[[1976,10,1]]},"published-print":{"date-parts":[[1976,10,1]]}},"URL":"https:\/\/doi.org\/10.1136\/pgmj.52.612.659","relation":{},"ISSN":["0032-5473","1469-0756"],"issn-type":[{"value":"0032-5473","type":"print"},{"value":"1469-0756","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[1976,10]]},"published":{"date-parts":[[1976,10,1]]}}}