Renal Extramedullary Hematopoiesis in Mast Cell Leukemia with Bone Marrow Fibrosis
Systemic mastocytosis is defined by the clonal proliferation of abnormal mast cells. The clinical course can range from indolent forms with normal life expectancy to advanced mast cell leukemia with dismal prognosis. An association with other diseases, including myeloproliferative neoplasia, has bee...
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| Format: | Article |
| Language: | English |
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Wiley
2024-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2024/3502887 |
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| author | Damian T. Rieke Laura K. Schmalbrock Jana Ihlow Karsten Kleo Ann-Christin von Brünneck Florian Nolte Ulrich Keller Sebastian Ochsenreither |
| author_facet | Damian T. Rieke Laura K. Schmalbrock Jana Ihlow Karsten Kleo Ann-Christin von Brünneck Florian Nolte Ulrich Keller Sebastian Ochsenreither |
| author_sort | Damian T. Rieke |
| collection | DOAJ |
| description | Systemic mastocytosis is defined by the clonal proliferation of abnormal mast cells. The clinical course can range from indolent forms with normal life expectancy to advanced mast cell leukemia with dismal prognosis. An association with other diseases, including myeloproliferative neoplasia, has been described. We present a case of a 75-year patient with a history of cutaneous mastocytosis who was diagnosed with mast cell leukemia more than 9 years ago and did not receive treatment. The patient presented to our clinic with acute kidney failure because of renal extramedullary hematopoiesis. Bone marrow histopathology revealed extensive fibrosis and 50% infiltration by mast cells with a c-KIT D816V mutation. No mutations supporting primary myelofibrosis were identified. Treatment with midostaurin was started, and the patient was discharged after improvement of renal function. Here, we discuss diagnostic challenges between different forms of mast cell leukemia and overlaps with other hematological malignancies. |
| format | Article |
| id | doaj-art-0aa11c6021ca4c8b9cfa4cb9e3b68211 |
| institution | DOAJ |
| issn | 2090-6579 |
| language | English |
| publishDate | 2024-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-0aa11c6021ca4c8b9cfa4cb9e3b682112025-08-20T03:17:27ZengWileyCase Reports in Hematology2090-65792024-01-01202410.1155/2024/3502887Renal Extramedullary Hematopoiesis in Mast Cell Leukemia with Bone Marrow FibrosisDamian T. Rieke0Laura K. Schmalbrock1Jana Ihlow2Karsten Kleo3Ann-Christin von Brünneck4Florian Nolte5Ulrich Keller6Sebastian Ochsenreither7Department of HematologyDepartment of HematologyBerlin Institute of Health (BIH) at CharitéInstitute of PathologyInstitute of PathologyPraxis Onkologie SeestrasseDepartment of HematologyDepartment of HematologySystemic mastocytosis is defined by the clonal proliferation of abnormal mast cells. The clinical course can range from indolent forms with normal life expectancy to advanced mast cell leukemia with dismal prognosis. An association with other diseases, including myeloproliferative neoplasia, has been described. We present a case of a 75-year patient with a history of cutaneous mastocytosis who was diagnosed with mast cell leukemia more than 9 years ago and did not receive treatment. The patient presented to our clinic with acute kidney failure because of renal extramedullary hematopoiesis. Bone marrow histopathology revealed extensive fibrosis and 50% infiltration by mast cells with a c-KIT D816V mutation. No mutations supporting primary myelofibrosis were identified. Treatment with midostaurin was started, and the patient was discharged after improvement of renal function. Here, we discuss diagnostic challenges between different forms of mast cell leukemia and overlaps with other hematological malignancies.http://dx.doi.org/10.1155/2024/3502887 |
| spellingShingle | Damian T. Rieke Laura K. Schmalbrock Jana Ihlow Karsten Kleo Ann-Christin von Brünneck Florian Nolte Ulrich Keller Sebastian Ochsenreither Renal Extramedullary Hematopoiesis in Mast Cell Leukemia with Bone Marrow Fibrosis Case Reports in Hematology |
| title | Renal Extramedullary Hematopoiesis in Mast Cell Leukemia with Bone Marrow Fibrosis |
| title_full | Renal Extramedullary Hematopoiesis in Mast Cell Leukemia with Bone Marrow Fibrosis |
| title_fullStr | Renal Extramedullary Hematopoiesis in Mast Cell Leukemia with Bone Marrow Fibrosis |
| title_full_unstemmed | Renal Extramedullary Hematopoiesis in Mast Cell Leukemia with Bone Marrow Fibrosis |
| title_short | Renal Extramedullary Hematopoiesis in Mast Cell Leukemia with Bone Marrow Fibrosis |
| title_sort | renal extramedullary hematopoiesis in mast cell leukemia with bone marrow fibrosis |
| url | http://dx.doi.org/10.1155/2024/3502887 |
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