କ୍ରନିକ ମଲ୍ଟିଫୋକାଲ ଲାଙ୍ଗରହାନ୍ସ ସେଲ ହିଷ୍ଟିଓସାଇଟୋସିସ

Hand–Schüller–Christian disease, multifocal eosinophilic granuloma
	A child with Hand-Schüller-Christian disease
ବିଭାଗ	Dermatology
ଲକ୍ଷଣ	Bulging eyes, breakdown of areas of bone, diabetes insipidus
ଆକ୍ରାନ୍ତ ସମୟ	2 to 6 years old
ଅବଧି	Long term
କାରଣ	Genetic mutation during early development
ରୋଗ ନିର୍ଣ୍ଣୟ ପଦ୍ଧତି	Tissue biopsy
ସଠିକ ରୋଗ ନିର୍ଣ୍ଣୟ	Letterer-Siwe disease, mastocytosis
ଚିକିତ୍ସା	Prednisone, vinblastine, cytarabine, mercaptopurine
Prognosis	Variable
ପୁନଃପୌନିକ	Rare
	[ଉଇକିଡାଟାରେ ବଦଳ କରନ୍ତୁ]

କ୍ରନିକ ମଲ୍ଟିଫୋକାଲ ଲାଙ୍ଗରହାନ୍ସ ସେଲ ହିଷ୍ଟିଓସାଇଟୋସିସ, (ଇଂରାଜୀ ଭାଷାରେ Chronic multifocal Langerhans cell histiocytosis) ଯାହା ହ୍ୟାଣ୍ଡ-ଶୁଲର-ଖ୍ରୀଷ୍ଟିଆନ ସିଣ୍ଡ୍ରୋମ ଭାବରେ ମଧ୍ୟ ଜଣାଶୁଣା, ଏହା ଏକ ପ୍ରକାର ଲାଙ୍ଗରହାନ୍ସ ସେଲ ହିଷ୍ଟିଓସାଇଟୋସିସ୍ (LCH) ଯାହା ଏକାଧିକ ଅଙ୍ଗ ଉପରେ ପ୍ରଭାବ ପକାଇପାରେ ।^[୧] ^[୪] ^[୫] ଏହାର ତିନୋଟି ମୁଖ୍ୟ ବୈଶିଷ୍ଟ୍ୟ ହେଉଛି ଆଖି ବାହାରକୁ ଆସିବା, ହାଡ଼ର ଅନେକ ଅଂଶ ଭାଙ୍ଗିବା (ପ୍ରାୟତ ଖପୁରୀରେ) ଏବଂ ଡାଏବେଟିସ ଇନସିପିଡସ (ଅତ୍ୟଧିକ ତୃଷା ଏବଂ ପତଳା ପରିସ୍ରା) ।^[୨] ତଥାପି ପ୍ରାୟ ୭୫% ମାମଲାରେ ତିନୋଟି ଯାକ ବୈଶିଷ୍ଟ୍ୟ ନାହିଁ ।^[୨] ଅନ୍ୟାନ୍ୟ ବୈଶିଷ୍ଟ୍ୟଗୁଡିକ ଯଥାକ୍ରମେ ଏକ ଅସମାନ ମୁଖ, କାନ ସଂକ୍ରମଣ ଏବଂ ଉନ୍ନତ ପେରିଓଡୋଣ୍ଟାଲ ରୋଗ ଅନ୍ତର୍ଭୁକ୍ତ କରିହୋଇପାରେ ।^[୨]

ଏହା ଏମଏପିକାଇନେଜ (MAPKinase) ପଥରେ ଏକ ଜେନେଟିକ୍ ପରିବର୍ତ୍ତନ ହେତୁ ପ୍ରାରମ୍ଭିକ ବିକାଶ ସମୟରେ ଘଟିଥାଏ ।^[୪] ଲକ୍ଷଣ ଏବଂ ଏମଆରଆଇ ଉପରେ ଆଧାର କରି ଏହି ରୋଗ ନିର୍ଣ୍ଣୟ କରାଯାଇପାରେ ଏବଂ ତନ୍ତୁ ବାୟୋପସି ଦ୍ୱାରା ନିଶ୍ଚିତ କରାଯାଇପାରେ । ^[୫] ^[୬] ରକ୍ତ ପରୀକ୍ଷାରେ ସ୍ୱଳ୍ପ ଲୋହିତ ରକ୍ତ କଣିକା, ଏବଂ ବେଳେବେଳେ କମ୍ ଶ୍ୱେତ ରକ୍ତ କଣିକା ଏବଂ ସ୍ୱଳ୍ପ ପ୍ଲାଟେଲେଟ୍ ଦେଖାଯାଏ ।^[୨]

ଚିକିତ୍ସାରେ ପ୍ରେଡନିସୋନ, ଭିନବ୍ଲାଷ୍ଟିନ ଓ ମରକାପ୍ଟୋପ୍ୟୁରିନ ମିଶ୍ରଣ ଅନ୍ତର୍ଭୁକ୍ତ ହୋଇପାରେ ।^[୪] କେତେ ଏବଂ କିପରି ଅଙ୍ଗ ପ୍ରଭାବିତ ହୁଏ ତାହା ଉପରେ ଦୃଷ୍ଟିକୋଣ ନିର୍ଭର କରେ ।^[୬] କେତେକ ଲୋକଙ୍କ ଅବସ୍ଥା ଜୀବନ ପ୍ରତି ବିପଦଜନକ ଅଟେ । ^[୬] ଏହି ରୋଗ ବିରଳ ଅଟେ । ^[୭] ଅଧିକାଂଶ ମାମଲା ୨ରୁ ୬ ବର୍ଷ ବୟସ ବର୍ଗରେ ହୋଇଥାଏ ।^[୩] ଝିଅମାନଙ୍କ ଅପେକ୍ଷା ପୁଅମାନେ ଅଧିକ ପ୍ରଭାବିତ ହୁଅନ୍ତି ।^[୨]

ଏହା ପୂର୍ବରୁ ଆମେରିକାର ଶିଶୁରୋଗ ବିଶେଷଜ୍ଞ ଆଲଫ୍ରେଡ୍ ହ୍ୟାଣ୍ଡ୍ ଜୁନିଅର, ଅଷ୍ଟ୍ରିଆର ଆର୍ଥର ସ୍କଲ୍ଲେର ଓ ଆମେରିକାର ହେନରି ଆସବେରି କ୍ରିଷ୍ଟିଆନ ଏହା ବିଷୟରେ ଯଥାକ୍ରମ ୧୮୯୩, ୧୯୧୫ ଓ ୧୯୧୯ରେ ବର୍ଣ୍ଣନା କରିଥିଲେ ।^[୮] ୧୯୮୦ ଦଶକରେ ହିଷ୍ଟୋସାଇଟ୍ ସୋସାଇଟି ଏହାକୁ ହିଷ୍ଟୋସାଇଟୋସେସ ଶ୍ରେଣୀଭୁକ୍ତ କରିବା ପୂର୍ବରୁ, LCH କୁ "ହିଷ୍ଟୋଓସାଇଟୋସିସ ଏକ୍ସ (X) " ଭାବରେ ଜଣାଶୁଣା ଥିଲା, ଯେଉଁଠାରେ "X" ସେହି ସମୟରେ ଅଜ୍ଞାତ କାରଣକୁ ଦର୍ଶାଉଥିଲା ।^[୯] ^[୧୦]

ଆଧାର

↑ ^୧.୦ ^୧.୧ "Orphanet: OBSOLETE: Hand Schüller Christian disease". www.orpha.net. Archived from the original on 15 November 2017. Retrieved 4 December 2020.
↑ ^୨.୦ ^୨.୧ ^୨.୨ ^୨.୩ ^୨.୪ ^୨.୫ ^୨.୬ ^୨.୭ Rajendran, R. (2009). "17. Diseases of bone and joint (non-neoplastic and non-infectious disorders of bone, skeletal dysplasias/dysostoses, constitutional bone disorders)". In Rajendran, R.; Sivapathasundharan, B. (eds.). Shafer's Textbook of Oral Pathology (6th ed.). Elsevier. p. 744. ISBN 978-81-312-1570-8. Archived from the original on 2021-08-29. Retrieved 2020-12-02.
↑ ^୩.୦ ^୩.୧ Stanway, Amy (2005). "Langerhans cell histiocytosis | DermNet NZ". dermnetnz.org. Archived from the original on 14 August 2021. Retrieved 4 December 2020.
↑ ^୪.୦ ^୪.୧ ^୪.୨ ^୪.୩ ^୪.୪ ^୪.୫ "Langerhans Cell Histiocytosis". NORD (National Organization for Rare Disorders). Archived from the original on 10 June 2021. Retrieved 4 December 2020.
↑ ^୫.୦ ^୫.୧ ^୫.୨ "Hand-Schüller-Christian Disease | American Journal of Neuroradiology". www.ajnr.org. Archived from the original on 29 August 2021. Retrieved 2 December 2020.
↑ ^୬.୦ ^୬.୧ ^୬.୨ ^୬.୩ ^୬.୪ "Langerhans cell histiocytosis | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Archived from the original on 10 January 2021. Retrieved 5 December 2020. Updated 2017
↑ ^୭.୦ ^୭.୧ Chang, Karen L.; Snyder, David S. (2007). "17. Langerhans Cell Histiocytosis". In Stephen M. Ansell (ed.). Rare Hematological Malignancies. Springer Science & Business Media. p. 383. ISBN 978-0-387-73743-0. Archived from the original on 2021-08-29. Retrieved 2020-12-04.
↑ Pluot, Etienne; Davies, Mark; James, Steven L. J. (2009). "Who was who in bone tumours". In Davies, A. Mark; Sundaram, Murali; James, Steven J. (eds.). Imaging of Bone Tumors and Tumor-Like Lesions: Techniques and Applications. Springer. p. 681. ISBN 978-3-540-77982-7. Archived from the original on 2021-08-28. Retrieved 2020-12-02.
↑ Broadbent, V.; Egeler, R. M.; Nesbit, M. E. (September 1994). "Langerhans cell histiocytosis--clinical and epidemiological aspects". The British Journal of Cancer. Supplement. 23: S11–S16. ISSN 0306-9443. PMC 2149699. PMID 8075001. Archived from the original on 2021-08-28. Retrieved 2020-12-04.
↑ The Writing Group of the Histiocyte Society (1987). "Histiocytosis syndromes in children. Writing Group of the Histiocyte Society". Lancet. 1 (8526): 208–9. doi:10.1016/S0140-6736(87)90016-X. PMID 2880029.

[Orpha2020-1] ୧.୦ ^୧.୧ "Orphanet: OBSOLETE: Hand Schüller Christian disease". www.orpha.net. Archived from the original on 15 November 2017. Retrieved 4 December 2020.

[Raj2009-2] ୨.୦ ^୨.୧ ^୨.୨ ^୨.୩ ^୨.୪ ^୨.୫ ^୨.୬ ^୨.୭ Rajendran, R. (2009). "17. Diseases of bone and joint (non-neoplastic and non-infectious disorders of bone, skeletal dysplasias/dysostoses, constitutional bone disorders)". In Rajendran, R.; Sivapathasundharan, B. (eds.). Shafer's Textbook of Oral Pathology (6th ed.). Elsevier. p. 744. ISBN 978-81-312-1570-8. Archived from the original on 2021-08-29. Retrieved 2020-12-02.

[DermnetNZ2005-3] ୩.୦ ^୩.୧ Stanway, Amy (2005). "Langerhans cell histiocytosis | DermNet NZ". dermnetnz.org. Archived from the original on 14 August 2021. Retrieved 4 December 2020.

[NORD2017-4] ୪.୦ ^୪.୧ ^୪.୨ ^୪.୩ ^୪.୪ ^୪.୫ "Langerhans Cell Histiocytosis". NORD (National Organization for Rare Disorders). Archived from the original on 10 June 2021. Retrieved 4 December 2020.

[AJNR2010-5] ୫.୦ ^୫.୧ ^୫.୨ "Hand-Schüller-Christian Disease | American Journal of Neuroradiology". www.ajnr.org. Archived from the original on 29 August 2021. Retrieved 2 December 2020.

[GARD2017-6] ୬.୦ ^୬.୧ ^୬.୨ ^୬.୩ ^୬.୪ "Langerhans cell histiocytosis | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Archived from the original on 10 January 2021. Retrieved 5 December 2020. Updated 2017

[Ans2007-7] ୭.୦ ^୭.୧ Chang, Karen L.; Snyder, David S. (2007). "17. Langerhans Cell Histiocytosis". In Stephen M. Ansell (ed.). Rare Hematological Malignancies. Springer Science & Business Media. p. 383. ISBN 978-0-387-73743-0. Archived from the original on 2021-08-29. Retrieved 2020-12-04.

[Tum2009-8] Pluot, Etienne; Davies, Mark; James, Steven L. J. (2009). "Who was who in bone tumours". In Davies, A. Mark; Sundaram, Murali; James, Steven J. (eds.). Imaging of Bone Tumors and Tumor-Like Lesions: Techniques and Applications. Springer. p. 681. ISBN 978-3-540-77982-7. Archived from the original on 2021-08-28. Retrieved 2020-12-02.

[Broad1994-9] Broadbent, V.; Egeler, R. M.; Nesbit, M. E. (September 1994). "Langerhans cell histiocytosis--clinical and epidemiological aspects". The British Journal of Cancer. Supplement. 23: S11–S16. ISSN 0306-9443. PMC 2149699. PMID 8075001. Archived from the original on 2021-08-28. Retrieved 2020-12-04.

[10] The Writing Group of the Histiocyte Society (1987). "Histiocytosis syndromes in children. Writing Group of the Histiocyte Society". Lancet. 1 (8526): 208–9. doi:10.1016/S0140-6736(87)90016-X. PMID 2880029.

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