Mast Cell Leukemia: Review of a Rare Disease and Case Report of Prolonged Survival after Allogeneic Stem Cell Transplant

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...

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Main Authors: James Bauer, MD, PhD, Walter Longo, MD, David Yang, MD
Format: Article
Language:English
Published: Elsevier 2017-11-01
Series:Human Pathology: Case Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2214330016301390
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spelling doaj-cb0b1584d5344aaf9e0c033ae76fb3792020-11-25T00:56:10ZengElsevierHuman 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
collection DOAJ
language English
format Article
sources DOAJ
author James Bauer, MD, PhD
Walter Longo, MD
David Yang, MD
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
author_facet James Bauer, MD, PhD
Walter Longo, MD
David Yang, MD
author_sort James Bauer, MD, PhD
title 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_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_sort mast cell leukemia: review of a rare disease and case report of prolonged survival after allogeneic stem cell transplant
publisher Elsevier
series Human Pathology: Case Reports
issn 2214-3300
publishDate 2017-11-01
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
url http://www.sciencedirect.com/science/article/pii/S2214330016301390
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AT davidyangmd mastcellleukemiareviewofararediseaseandcasereportofprolongedsurvivalafterallogeneicstemcelltransplant
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