Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and Osteosclerosis
Primary myelofibrosis (PMF), per WHO criteria, is a clonal myeloproliferative neoplasm that usually presents with a proliferation of granulocytic and megakaryocytic lineages with an associated fibrous deposition and extramedullary hematopoiesis. The bone marrow histologic findings of this disorder a...
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| Format: | Article |
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MDPI AG
2015-12-01
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| Series: | Cancers |
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| Online Access: | http://www.mdpi.com/2072-6694/7/4/0903 |
| _version_ | 1797706251569725440 |
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| author | Adam Stelling Brian A. Jonas Hooman H. Rashidi Mehrdad Abedi Mingyi Chen |
| author_facet | Adam Stelling Brian A. Jonas Hooman H. Rashidi Mehrdad Abedi Mingyi Chen |
| author_sort | Adam Stelling |
| collection | DOAJ |
| description | Primary myelofibrosis (PMF), per WHO criteria, is a clonal myeloproliferative neoplasm that usually presents with a proliferation of granulocytic and megakaryocytic lineages with an associated fibrous deposition and extramedullary hematopoiesis. The bone marrow histologic findings of this disorder are typically characterized by the presence of myeloid metaplasia with an associated reactive fibrosis, angiogenesis, and osteosclerosis. However, marked myelofibrosis is not solely confined to PMF and may also be associated with other conditions including but not limited to acute megakaryoblastic leukemias (FAB AML-M7). Here, we describe a rare case of a non-megakaryoblastic acute myeloid leukemia with marked myelofibrosis with osteosclerosis and an isolated trisomy 19. A 19-year-old male presented with severe bone pain of one week duration with a complete blood cell count and peripheral smear showing a mild anemia and occasional circulating blasts. A follow up computed tomography (CT) scan showed diffuse osteosclerosis with no evidence of hepatosplenomegaly or lymphadenopathy. Subsequently, the bone marrow biopsy showed markedly sclerotic bony trabeculae and a hypercellular marrow with marked fibrosis and intervening sheets of immature myeloid cells consistent with myeloblasts with monocytic differentiation. Importantly, these myeloblasts were negative for megakaryocytic markers (CD61 and vWF), erythroid markers (hemoglobin and E-cadherin), and lymphoid markers (CD3, CD19, and TdT). Metaphase cytogenetics showed an isolated triosomy 19 with no JAK2 V617F mutation. The patient was treated with induction chemotherapy followed by allogenic hematopoietic stem cell transplantation which subsequently resulted in a rapid resolution of bone marrow fibrosis, suggesting graft-anti-fibrosis effect. This is a rare case of a non-megakaryoblastic acute myeloid leukemia with myelofibrosis and osteosclerosis with trisomy 19 that may provide insights into the prognosis and therapeutic options of future cases. |
| first_indexed | 2024-03-12T05:49:28Z |
| format | Article |
| id | doaj.art-58cd9e5739a34656849c0ef0e2b5fb22 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-12T05:49:28Z |
| publishDate | 2015-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-58cd9e5739a34656849c0ef0e2b5fb222023-09-03T05:18:45ZengMDPI AGCancers2072-66942015-12-01742459246510.3390/cancers7040903cancers7040903Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and OsteosclerosisAdam Stelling0Brian A. Jonas1Hooman H. Rashidi2Mehrdad Abedi3Mingyi Chen4Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, PATH Bldg. 4400 V Street, Sacramento, CA 95817, USADivision of Hematology and Oncology, University of California Davis Medical Center, Sacramento, CA 95817, USADepartment of Pathology and Laboratory Medicine, University of California Davis Medical Center, PATH Bldg. 4400 V Street, Sacramento, CA 95817, USADivision of Hematology and Oncology, University of California Davis Medical Center, Sacramento, CA 95817, USADepartment of Pathology and Laboratory Medicine, University of California Davis Medical Center, PATH Bldg. 4400 V Street, Sacramento, CA 95817, USAPrimary myelofibrosis (PMF), per WHO criteria, is a clonal myeloproliferative neoplasm that usually presents with a proliferation of granulocytic and megakaryocytic lineages with an associated fibrous deposition and extramedullary hematopoiesis. The bone marrow histologic findings of this disorder are typically characterized by the presence of myeloid metaplasia with an associated reactive fibrosis, angiogenesis, and osteosclerosis. However, marked myelofibrosis is not solely confined to PMF and may also be associated with other conditions including but not limited to acute megakaryoblastic leukemias (FAB AML-M7). Here, we describe a rare case of a non-megakaryoblastic acute myeloid leukemia with marked myelofibrosis with osteosclerosis and an isolated trisomy 19. A 19-year-old male presented with severe bone pain of one week duration with a complete blood cell count and peripheral smear showing a mild anemia and occasional circulating blasts. A follow up computed tomography (CT) scan showed diffuse osteosclerosis with no evidence of hepatosplenomegaly or lymphadenopathy. Subsequently, the bone marrow biopsy showed markedly sclerotic bony trabeculae and a hypercellular marrow with marked fibrosis and intervening sheets of immature myeloid cells consistent with myeloblasts with monocytic differentiation. Importantly, these myeloblasts were negative for megakaryocytic markers (CD61 and vWF), erythroid markers (hemoglobin and E-cadherin), and lymphoid markers (CD3, CD19, and TdT). Metaphase cytogenetics showed an isolated triosomy 19 with no JAK2 V617F mutation. The patient was treated with induction chemotherapy followed by allogenic hematopoietic stem cell transplantation which subsequently resulted in a rapid resolution of bone marrow fibrosis, suggesting graft-anti-fibrosis effect. This is a rare case of a non-megakaryoblastic acute myeloid leukemia with myelofibrosis and osteosclerosis with trisomy 19 that may provide insights into the prognosis and therapeutic options of future cases.http://www.mdpi.com/2072-6694/7/4/0903acute myeloid leukemiatrisomy 19myelofibrosisosteosclerosis |
| spellingShingle | Adam Stelling Brian A. Jonas Hooman H. Rashidi Mehrdad Abedi Mingyi Chen Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and Osteosclerosis Cancers acute myeloid leukemia trisomy 19 myelofibrosis osteosclerosis |
| title | Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and Osteosclerosis |
| title_full | Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and Osteosclerosis |
| title_fullStr | Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and Osteosclerosis |
| title_full_unstemmed | Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and Osteosclerosis |
| title_short | Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and Osteosclerosis |
| title_sort | acute myeloid leukemia with isolated trisomy 19 associated with diffuse myelofibrosis and osteosclerosis |
| topic | acute myeloid leukemia trisomy 19 myelofibrosis osteosclerosis |
| url | http://www.mdpi.com/2072-6694/7/4/0903 |
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