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BCR-ABL Negative Atypical Chronic Myeloid Leukemia (Leukemia, Myeloid, Philadelphia Negative)

Subscribe to New Research on BCR-ABL Negative Atypical Chronic Myeloid Leukemia

A myelodysplastic/myeloproliferative disorder characterized by myelodysplasia associated with bone marrow and peripheral blood patterns similar to CHRONIC MYELOID LEUKEMIA, but cytogenetically lacking a PHILADELPHIA CHROMOSOME or bcr/abl fusion gene (GENES, ABL).

Also Known As:

Leukemia, Myeloid, Philadelphia Negative; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative; Leukemia, Myelogenous, Ph1 Negative; Leukemia, Myelogenous, Ph1-Negative; Leukemia, Myeloid, Chronic, Atypical; Leukemia, Myeloid, Ph1 Negative; Leukemia, Myeloid, Ph1-Negative; Myelogenous Leukemia, Ph1-Negative; Myeloid Leukemia, Ph1-Negative; Leukemia, Ph1-Negative Myelogenous; Leukemia, Ph1-Negative Myeloid; Leukemia, Philadelphia-Negative Myeloid; Leukemias, Ph1-Negative Myelogenous; Leukemias, Ph1-Negative Myeloid; Leukemias, Philadelphia-Negative Myeloid; Myelogenous Leukemia, Ph1 Negative; Myelogenous Leukemias, Ph1-Negative; Myeloid Leukemia, Ph1 Negative; Myeloid Leukemia, Philadelphia Negative; Myeloid Leukemias, Ph1-Negative; Myeloid Leukemias, Philadelphia-Negative; Ph1-Negative Myelogenous Leukemia; Ph1-Negative Myelogenous Leukemias; Ph1-Negative Myeloid Leukemia; Ph1-Negative Myeloid Leukemias; Philadelphia-Negative Myeloid Leukemia; Philadelphia-Negative Myeloid Leukemias; Atypical Chronic Myeloid Leukemia; Chronic Myeloid Leukemia, Atypical; Leukemia, Myeloid, Philadelphia-Negative; Myeloid Leukemia, Philadelphia-Negative

Networked: 31 relevant articles (0 outcomes, 2 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Primary Myelofibrosis (Myelosclerosis)
3.	Precursor Cell Lymphoblastic Leukemia-Lymphoma (Acute Lymphoblastic Leukemia)
4.	Hematologic Neoplasms (Hematological Malignancy)
5.	Chronic Myelomonocytic Leukemia (Leukemia, Chronic Myelomonocytic)

Experts

1.	Cross, Nicholas C P: 5 articles (09/2010 - 10/2004)
2.	Chase, Andrew: 3 articles (09/2010 - 04/2005)
3.	Reiter, Andreas: 3 articles (09/2010 - 04/2005)
4.	Grand, Francis H: 3 articles (06/2009 - 10/2004)
5.	Kavelaars, François G: 2 articles (06/2015 - 05/2015)
6.	Valk, Peter J M: 2 articles (06/2015 - 05/2015)
7.	Ammatuna, Emanuele: 2 articles (06/2015 - 05/2015)
8.	Touw, Ivo P: 2 articles (06/2015 - 05/2015)
9.	van Lom, Kirsten: 2 articles (06/2015 - 05/2015)
10.	Eefting, Matthias: 2 articles (06/2015 - 05/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to BCR-ABL Negative Atypical Chronic Myeloid Leukemia:

1.	Carrier Proteins (Binding Protein)IBA 09/01/2013 - "Recent studies identified mutations in SET-binding protein 1 (SETBP1) as novel marker in myeloid malignancies, especially in atypical chronic myeloid leukemia (aCML) and related diseases. " 03/01/2015 - "Centromeric fragment of chromosome 7 in atypical chronic myeloid leukemia with the SET binding protein 1 gene mutation." 01/01/2015 - "Recurrent somatic SET-binding protein 1 (SETBP1) and splicing pathway gene mutations have recently been found in atypical chronic myeloid leukemia and other hematologic malignancies. "
2.	imatinib (Gleevec)FDA Link 10/15/2004 - "We conclude that TP53BP1-PDGFRB is a novel imatinib target in atypical chronic myeloid leukemia." 06/01/2003 - "Forty-eight patients with AML (n = 10), MDS (n = 8), myelofibrosis (n = 18), atypical chronic myeloid leukemia (CML; n = 7), chronic myelomonocytic leukemia (CMML; n = 3), or polycythemia vera (n = 2) were treated with imatinib 400 mg daily. " 10/01/2003 - "Imatinib mesylate elicits positive clinical response in atypical chronic myeloid leukemia involving the platelet-derived growth factor receptor beta." 01/01/2013 - "We report here on a rare case of BCR-ABL1-negative atypical chronic myeloid leukemia with a t(9;22)(p24;q11.2)translocation and a BCR-JAK2 fusion gene, with resistance to the tyrosine kinase inhibitors imatinib and dasatinib.At"
3.	Platelet-Derived Growth Factor Receptors (Platelet-Derived Growth Factor Receptor)IBA 05/01/2004 - "We report a case of BCR-ABL-negative atypical chronic myeloid leukemia (CML) with translocation t(4;22) (q12;q11.2) juxtaposing the breakpoint cluster region (BCR) and platelet-derived growth factor receptor-alpha (PDGFRA) genes. " 05/01/2004 - "Molecular and cytogenetic characterization of a novel translocation t(4;22) involving the breakpoint cluster region and platelet-derived growth factor receptor-alpha genes in a patient with atypical chronic myeloid leukemia." 10/01/2003 - "Imatinib mesylate elicits positive clinical response in atypical chronic myeloid leukemia involving the platelet-derived growth factor receptor beta." 06/15/2001 - "H4(D10S170), a gene frequently rearranged in papillary thyroid carcinoma, is fused to the platelet-derived growth factor receptor beta gene in atypical chronic myeloid leukemia with t(5;10)(q33;q22)."
4.	Protein-Tyrosine Kinases (Tyrosine Kinase)IBA 11/01/2007 - "A constitutively active SPTBN1-FLT3 fusion in atypical chronic myeloid leukemia is sensitive to tyrosine kinase inhibitors and immunotherapy." 05/18/2009 - "The analysis of a small number of patients with atypical chronic myeloid leukemia showing balanced chromosomal translocations has revealed diverse tyrosine kinase fusion genes, most commonly involving FGFR1, PDGFRA, PDGFRB, JAK2, and ABL. " 01/01/2013 - "We report here on a rare case of BCR-ABL1-negative atypical chronic myeloid leukemia with a t(9;22)(p24;q11.2)translocation and a BCR-JAK2 fusion gene, with resistance to the tyrosine kinase inhibitors imatinib and dasatinib.At"
5.	Platelet-Derived Growth Factor beta Receptor (Receptor, Platelet Derived Growth Factor beta)IBA 03/01/2014 - "Myelodysplastic/myeloproliferative neoplasms (MDS/MPNs), including chronic myelomonocytic leukemia, atypical chronic myeloid leukemia, MDS/MPN-Unclassifiable, ring sideroblasts associated with marked thrombocytosis, and juvenile myelomonocytic leukemia, are clonal hematologic diseases characterized by myeloid dysplasia, proliferation, and absence of the molecular lesions BCR/ABL, PDGFRA, PDGFRB, and FGFR1. " 05/18/2009 - "The analysis of a small number of patients with atypical chronic myeloid leukemia showing balanced chromosomal translocations has revealed diverse tyrosine kinase fusion genes, most commonly involving FGFR1, PDGFRA, PDGFRB, JAK2, and ABL. "
6.	Pyridinolcarbamate (P23)IBA 11/01/2002 - "Translocation t(9;22) (p23;q11) in atypical chronic myeloid leukemia (aCML) presenting osteolytic lesions." 04/01/2005 - "We have identified a t(8;9)(p21-23;p23-24) in seven male patients (mean age 50, range 32-74) with diverse hematologic malignancies and clinical outcomes: atypical chronic myeloid leukemia/chronic eosinophilic leukemia (n = 5), secondary acute myeloid leukemia (n = 1), and pre-B-cell acute lymphoblastic leukemia (n = 1). "
7.	Phosphotransferases (Kinase)IBA 09/01/2010 - "No abnormalities involving tyrosine kinases were detected; however, nine cytogenetically cryptic copy number imbalances were detected in seven patients, including hemizygous deletions of RUNX1 or CEBPA in two cases with atypical chronic myeloid leukemia. " 01/01/2012 - "Hematopoietic myeloproliferative neoplasms (MPNS) with rearrangements of the receptor tyrosine kinase FGFR1 gene, located on chromosome 8p11, are uncommon and associated with diverse presentations such as atypical chronic myeloid leukemia, acute myeloid leukemia, or an acute T- or B-lymphoblastic leukemia, reflecting the hematopoietic stem cell origin of the disease. "
8.	INCB018424IBA 06/01/2015 - "Erratum to: Atypical chronic myeloid leukemia with concomitant CSF3R T618I and SETBP1 mutations unresponsive to the JAK inhibitor ruxolitinib." 05/01/2015 - "Atypical chronic myeloid leukemia with concomitant CSF3R T618I and SETBP1 mutations unresponsive to the JAK inhibitor ruxolitinib."
9.	Granulocyte Colony-Stimulating Factor (G-CSF)IBA 09/05/2013 - "Recently, we identified high-frequency oncogenic mutations in the granulocyte-colony stimulating factor receptor (CSF3R) in CNL and in some patients with atypical chronic myeloid leukemia. "
10.	Tyrosine (L-Tyrosine)FDA Link 09/01/2010 - "No abnormalities involving tyrosine kinases were detected; however, nine cytogenetically cryptic copy number imbalances were detected in seven patients, including hemizygous deletions of RUNX1 or CEBPA in two cases with atypical chronic myeloid leukemia. "

Therapies and Procedures

1.	Transplantation (Transplant Recipients) 01/01/2013 - "In the present study, we describe unrelated umbilical cord blood transplantation (CBT) in a 7-year-old Taiwanese boy with atypical chronic myeloid leukemia (BCR-ABL 1 negative). " 01/01/2013 - "Successful transplantation of ethnically mismatched cord blood in a boy with atypical chronic myeloid leukemia."
2.	Hematopoietic Stem Cell Transplantation 12/01/2004 - "Outcome of hematopoietic stem cell transplantation in patients with atypical chronic myeloid leukemia."
3.	Immunotherapy 11/01/2007 - "A constitutively active SPTBN1-FLT3 fusion in atypical chronic myeloid leukemia is sensitive to tyrosine kinase inhibitors and immunotherapy."
4.	Drug Therapy (Chemotherapy) 12/01/2004 - "Atypical chronic myeloid leukemia (aCML) occurs rarely and is associated with a poor prognosis when treated with conventional chemotherapy. "