Mast Cell Leukemia: Review of a Rare Disease and Case Report of Prolonged Survival after Allogeneic Stem Cell Transplant
Mast cell leukemia is a rare and aggressive form of mastocytosis characterized by >20% mast cells found in the bone marrow aspirates of patients with signs of systemic mastocytosis-related organ damage. The prognosis for patients with mast cell leukemia is extremely poor, with resistance to both...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2017-11-01
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| Series: | Human Pathology: Case Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2214330016301390 |
| _version_ | 1811322314448437248 |
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| author | James Bauer, MD, PhD Walter Longo, MD David Yang, MD |
| author_facet | James Bauer, MD, PhD Walter Longo, MD David Yang, MD |
| author_sort | James Bauer, MD, PhD |
| collection | DOAJ |
| description | Mast cell leukemia is a rare and aggressive form of mastocytosis characterized by >20% mast cells found in the bone marrow aspirates of patients with signs of systemic mastocytosis-related organ damage. The prognosis for patients with mast cell leukemia is extremely poor, with resistance to both cytoreductive therapies and tyrosine kinase inhibitors being relatively common. While allogeneic hematopoietic stem cell transplantation has been associated with long-term survival in patients with advanced systemic mastocytosis, reports regarding its effectiveness in mast cell leukemia are limited to fewer than 20 cases described in the literature. Here, we report a patient with mast cell leukemia who remains in complete remission 24 months after allogeneic HSCT at the time of this writing, and briefly review the clinical, diagnostic, and therapeutic approaches to this rare disease. Keywords: Mast cell leukemia, Allogeneic hematopoietic stem cell transplant, Mastocytosis |
| first_indexed | 2024-04-13T13:33:03Z |
| format | Article |
| id | doaj.art-cb0b1584d5344aaf9e0c033ae76fb379 |
| institution | Directory Open Access Journal |
| issn | 2214-3300 |
| language | English |
| last_indexed | 2024-04-13T13:33:03Z |
| publishDate | 2017-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Human Pathology: Case Reports |
| spelling | doaj.art-cb0b1584d5344aaf9e0c033ae76fb3792022-12-22T02:44:53ZengElsevierHuman Pathology: Case Reports2214-33002017-11-01104649Mast Cell Leukemia: Review of a Rare Disease and Case Report of Prolonged Survival after Allogeneic Stem Cell TransplantJames Bauer, MD, PhD0Walter Longo, MD1David Yang, MD2University of Wisconsin Hospital and Clinics, Department of Pathology and Laboratory Medicine, UWHC-Rm A4/204-3224, 600 Highland Avenue, Madison, WI 53792University of Wisconsin Hospital and Clinics, Department of Hematology and Oncology, UWHC-MEDICINE K6/568 CSC, MC 5669, 600 Highland Avenue, Madison, WI 53792University of Wisconsin Hospital and Clinics, Department of Pathology and Laboratory Medicine, K4/446 CSC-8550, 600 Highland Avenue, Madison, WI 53792; Corresponding author. Tel.: +1 608 263 5965.Mast cell leukemia is a rare and aggressive form of mastocytosis characterized by >20% mast cells found in the bone marrow aspirates of patients with signs of systemic mastocytosis-related organ damage. The prognosis for patients with mast cell leukemia is extremely poor, with resistance to both cytoreductive therapies and tyrosine kinase inhibitors being relatively common. While allogeneic hematopoietic stem cell transplantation has been associated with long-term survival in patients with advanced systemic mastocytosis, reports regarding its effectiveness in mast cell leukemia are limited to fewer than 20 cases described in the literature. Here, we report a patient with mast cell leukemia who remains in complete remission 24 months after allogeneic HSCT at the time of this writing, and briefly review the clinical, diagnostic, and therapeutic approaches to this rare disease. Keywords: Mast cell leukemia, Allogeneic hematopoietic stem cell transplant, Mastocytosishttp://www.sciencedirect.com/science/article/pii/S2214330016301390 |
| spellingShingle | James Bauer, MD, PhD Walter Longo, MD David Yang, MD Mast Cell Leukemia: Review of a Rare Disease and Case Report of Prolonged Survival after Allogeneic Stem Cell Transplant Human Pathology: Case Reports |
| title | Mast Cell Leukemia: Review of a Rare Disease and Case Report of Prolonged Survival after Allogeneic Stem Cell Transplant |
| title_full | Mast Cell Leukemia: Review of a Rare Disease and Case Report of Prolonged Survival after Allogeneic Stem Cell Transplant |
| title_fullStr | Mast Cell Leukemia: Review of a Rare Disease and Case Report of Prolonged Survival after Allogeneic Stem Cell Transplant |
| title_full_unstemmed | Mast Cell Leukemia: Review of a Rare Disease and Case Report of Prolonged Survival after Allogeneic Stem Cell Transplant |
| title_short | Mast Cell Leukemia: Review of a Rare Disease and Case Report of Prolonged Survival after Allogeneic Stem Cell Transplant |
| title_sort | mast cell leukemia review of a rare disease and case report of prolonged survival after allogeneic stem cell transplant |
| url | http://www.sciencedirect.com/science/article/pii/S2214330016301390 |
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