Bone marrow involvement of a patient with Niemann-Pick disease concomitant with Kartagener’s syndrome: Report of a rare case
Niemann-Pick disease is a rare lipid storage disorder with autosomal recessive inheritance, which is characterized by accumulation of sphingomyelin and other sphingolipids in macrophages. In most cases, macrophage lipids show a non-spesific, ?ntense blue staining with Giemsa, so the term “sea blue h...
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2014-08-01
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| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
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| Online Access: | https://mjhid.org/index.php/mjhid/article/view/1860 |
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| author | Muzaffer Keklik serdar sivgin suleyman baldane afra yildirim olgun kontas fatih kardas cigdem pala ali unal |
| author_facet | Muzaffer Keklik serdar sivgin suleyman baldane afra yildirim olgun kontas fatih kardas cigdem pala ali unal |
| author_sort | Muzaffer Keklik |
| collection | DOAJ |
| description | Niemann-Pick disease is a rare lipid storage disorder with autosomal recessive inheritance, which is characterized by accumulation of sphingomyelin and other sphingolipids in macrophages. In most cases, macrophage lipids show a non-spesific, ?ntense blue staining with Giemsa, so the term “sea blue histiocytosis” has been employed to describe this feature. Kartagener’s syndrome is a rare syndrome with autosomal recessive inheritance consisting of chronic paranasal sinusitis, situs inversus and bronchiectasis. It is accounted from half of primary ciliary dyskinesia syndromes. The correct diagnosis of this rare congenital disorder in early life is important in the overall prognosis of the syndrome.
Here, we reported a case having Kartagener’s syndrome with concomitant Niemann-Pick disease, as there is no report of such case in literature. |
| format | Article |
| id | doaj-art-6790b7f0207b4522972b7dd7f3f901b6 |
| institution | DOAJ |
| issn | 2035-3006 |
| language | English |
| publishDate | 2014-08-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj-art-6790b7f0207b4522972b7dd7f3f901b62025-08-20T02:57:56ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062014-08-011Bone marrow involvement of a patient with Niemann-Pick disease concomitant with Kartagener’s syndrome: Report of a rare caseMuzaffer Keklik0serdar sivgin1suleyman baldane2afra yildirim3olgun kontas4fatih kardas5cigdem pala6ali unal7Erciyes Stem Cell Transplantation Hospital, Department of Hematology, Faculty of Medicine, Erciyes University, Kayseri, TurkeyErciyes Stem Cell Transplantation Hospital, Department of Hematology, Faculty of Medicine, Erciyes University, Kayseri, TurkeyDepartment of İnternal Medicene, Faculty of Medicine, Erciyes University, Kayseri, TurkeyDepartment of Radyology, Faculty of Medicine, Erciyes University, Kayseri, TurkeyDepartment of Pathology, Faculty of Medicine, Erciyes University, Kayseri, TurkeyDepartment of Pediatry, Faculty of Medicine, Erciyes University, Kayseri, TurkeyErciyes Stem Cell Transplantation Hospital, Department of Hematology, Faculty of Medicine, Erciyes University, Kayseri, TurkeyErciyes Stem Cell Transplantation Hospital, Department of Hematology, Faculty of Medicine, Erciyes University, Kayseri, TurkeyNiemann-Pick disease is a rare lipid storage disorder with autosomal recessive inheritance, which is characterized by accumulation of sphingomyelin and other sphingolipids in macrophages. In most cases, macrophage lipids show a non-spesific, ?ntense blue staining with Giemsa, so the term “sea blue histiocytosis” has been employed to describe this feature. Kartagener’s syndrome is a rare syndrome with autosomal recessive inheritance consisting of chronic paranasal sinusitis, situs inversus and bronchiectasis. It is accounted from half of primary ciliary dyskinesia syndromes. The correct diagnosis of this rare congenital disorder in early life is important in the overall prognosis of the syndrome. Here, we reported a case having Kartagener’s syndrome with concomitant Niemann-Pick disease, as there is no report of such case in literature.https://mjhid.org/index.php/mjhid/article/view/1860Kartagener’s syndromeNiemann-Pick disease |
| spellingShingle | Muzaffer Keklik serdar sivgin suleyman baldane afra yildirim olgun kontas fatih kardas cigdem pala ali unal Bone marrow involvement of a patient with Niemann-Pick disease concomitant with Kartagener’s syndrome: Report of a rare case Mediterranean Journal of Hematology and Infectious Diseases Kartagener’s syndrome Niemann-Pick disease |
| title | Bone marrow involvement of a patient with Niemann-Pick disease concomitant with Kartagener’s syndrome: Report of a rare case |
| title_full | Bone marrow involvement of a patient with Niemann-Pick disease concomitant with Kartagener’s syndrome: Report of a rare case |
| title_fullStr | Bone marrow involvement of a patient with Niemann-Pick disease concomitant with Kartagener’s syndrome: Report of a rare case |
| title_full_unstemmed | Bone marrow involvement of a patient with Niemann-Pick disease concomitant with Kartagener’s syndrome: Report of a rare case |
| title_short | Bone marrow involvement of a patient with Niemann-Pick disease concomitant with Kartagener’s syndrome: Report of a rare case |
| title_sort | bone marrow involvement of a patient with niemann pick disease concomitant with kartagener s syndrome report of a rare case |
| topic | Kartagener’s syndrome Niemann-Pick disease |
| url | https://mjhid.org/index.php/mjhid/article/view/1860 |
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