Fatal systemic angiomatosis with widespread sclerotic skeletal changes, diagnosed with the aid of a bone marrow biopsy: the lymphatics enter the bone marrow
A 56-year-old female presented with popliteal venous thrombosis, splenomegaly, and sclerotic bone lesions. Bone marrow biopsy showed fibrosis, proliferation of abnormal blood vessels and lymphatics, bone remodelling, and no significant changes in haematopoietic elements. Following a relatively indol...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Termedia Publishing House
2018-11-01
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| Series: | Polish Journal of Pathology |
| Subjects: | |
| Online Access: | https://www.termedia.pl/Fatal-systemic-angiomatosis-with-widespread-sclerotic-skeletal-changes-diagnosed-with-the-aid-of-a-bone-marrow-biopsy-the-lymphatics-enter-the-bone-marrow,55,34122,1,1.html |
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| author | Zbigniew Rudzki Richard Lovell Ben Miller Vidhya Murthy Shankara Paneesha Guy Pratt Neil Smith |
| author_facet | Zbigniew Rudzki Richard Lovell Ben Miller Vidhya Murthy Shankara Paneesha Guy Pratt Neil Smith |
| author_sort | Zbigniew Rudzki |
| collection | DOAJ |
| description | A 56-year-old female presented with popliteal venous thrombosis, splenomegaly, and sclerotic bone lesions. Bone marrow biopsy showed fibrosis, proliferation of abnormal blood vessels and lymphatics, bone remodelling, and no significant changes in haematopoietic elements. Following a relatively indolent initial clinical course, one year later she rapidly deteriorated and died of respiratory failure associated with widespread disease. Ingrowth of podoplanin+ lymphatics mixed with CD34+/podoplanin– blood vessels into the bone marrow is a new finding and may be a unique feature of this disease. |
| first_indexed | 2024-12-20T06:47:01Z |
| format | Article |
| id | doaj.art-57769672c640444aa92c7e034174c19d |
| institution | Directory Open Access Journal |
| issn | 1233-9687 2084-9869 |
| language | English |
| last_indexed | 2024-12-20T06:47:01Z |
| publishDate | 2018-11-01 |
| publisher | Termedia Publishing House |
| record_format | Article |
| series | Polish Journal of Pathology |
| spelling | doaj.art-57769672c640444aa92c7e034174c19d2022-12-21T19:49:41ZengTermedia Publishing HousePolish Journal of Pathology1233-96872084-98692018-11-0169331431810.5114/pjp.2018.7955234122Fatal systemic angiomatosis with widespread sclerotic skeletal changes, diagnosed with the aid of a bone marrow biopsy: the lymphatics enter the bone marrowZbigniew RudzkiRichard LovellBen MillerVidhya MurthyShankara PaneeshaGuy PrattNeil SmithA 56-year-old female presented with popliteal venous thrombosis, splenomegaly, and sclerotic bone lesions. Bone marrow biopsy showed fibrosis, proliferation of abnormal blood vessels and lymphatics, bone remodelling, and no significant changes in haematopoietic elements. Following a relatively indolent initial clinical course, one year later she rapidly deteriorated and died of respiratory failure associated with widespread disease. Ingrowth of podoplanin+ lymphatics mixed with CD34+/podoplanin– blood vessels into the bone marrow is a new finding and may be a unique feature of this disease.https://www.termedia.pl/Fatal-systemic-angiomatosis-with-widespread-sclerotic-skeletal-changes-diagnosed-with-the-aid-of-a-bone-marrow-biopsy-the-lymphatics-enter-the-bone-marrow,55,34122,1,1.htmlangiomatosis angiogenesis bone marrow trephine bone marrow biopsy |
| spellingShingle | Zbigniew Rudzki Richard Lovell Ben Miller Vidhya Murthy Shankara Paneesha Guy Pratt Neil Smith Fatal systemic angiomatosis with widespread sclerotic skeletal changes, diagnosed with the aid of a bone marrow biopsy: the lymphatics enter the bone marrow Polish Journal of Pathology angiomatosis angiogenesis bone marrow trephine bone marrow biopsy |
| title | Fatal systemic angiomatosis with widespread sclerotic skeletal changes, diagnosed with the aid of a bone marrow biopsy: the lymphatics enter the bone marrow |
| title_full | Fatal systemic angiomatosis with widespread sclerotic skeletal changes, diagnosed with the aid of a bone marrow biopsy: the lymphatics enter the bone marrow |
| title_fullStr | Fatal systemic angiomatosis with widespread sclerotic skeletal changes, diagnosed with the aid of a bone marrow biopsy: the lymphatics enter the bone marrow |
| title_full_unstemmed | Fatal systemic angiomatosis with widespread sclerotic skeletal changes, diagnosed with the aid of a bone marrow biopsy: the lymphatics enter the bone marrow |
| title_short | Fatal systemic angiomatosis with widespread sclerotic skeletal changes, diagnosed with the aid of a bone marrow biopsy: the lymphatics enter the bone marrow |
| title_sort | fatal systemic angiomatosis with widespread sclerotic skeletal changes diagnosed with the aid of a bone marrow biopsy the lymphatics enter the bone marrow |
| topic | angiomatosis angiogenesis bone marrow trephine bone marrow biopsy |
| url | https://www.termedia.pl/Fatal-systemic-angiomatosis-with-widespread-sclerotic-skeletal-changes-diagnosed-with-the-aid-of-a-bone-marrow-biopsy-the-lymphatics-enter-the-bone-marrow,55,34122,1,1.html |
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