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Spontaneous Remission in a Patient with Chronic Myeloid Leukemia: A Case Report.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm in which granulocytic cells are the main proliferative component. At diagnosis, more than 90% of CML cases have the characteristic Philadelphia chromosome, containing the BCR::ABL1 fusion gene. The natural history of untreated CML is an initial indolent chronic phase which will be followed by an accelerated phase, blast phase, or both. Tyrosine kinase inhibitors (TKIs) have dramatically altered the natural history of CML. TKI discontinuation with the goal of treatment-free remission is currently part of current management recommen…

Imatinib-Induced Lichen Planus in Chronic Myeloid Leukemia: A Case Series.

Chronic myeloid leukemia (CML) is a myeloproliferative disorder in which the Philadelphia chromosome is the cytogenetic hallmark. It is characterized by the t (9;22) translocation, which in turn creates the chimeric BCR-ABL oncogene coding for a constitutively activated tyrosine kinase. Imatinib mesylate is a tyrosine kinase inhibitor that targets the BCR-ABL protein, c-KIT, and platelet-derived growth factor (PDGF) receptors and is used to treat CML, gastrointestinal stromal tumors, and dermato-fibrosarcoma protuberant. The development of the specific inhibitor of BCR-ABL tyrosine kinase has …

[Real world data on myeloproliferative neoplasms in Japan].

Since the discovery of the JAK2V617F, MPL gene, and Calreticulin gene mutations, remarkable changes have occurred in the identification of the pathology of Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs). The diagnostic criteria of polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the world health organization classification systems have also been amended to include these driver gene mutations. Additionally, treatment algorithms for each disease have been reviewed. Following these changes, real world data form several countries based on national su…

[Chronic myeloid leukemia presenting with marked eosinophilia].

An 80-year-old female with fever, edema in the lower extremities, and marked eosinophilia was referred to our hospital. Based on the presence of the Philadelphia chromosome, she was diagnosed with chronic myeloid leukemia (CML). Although imatinib induced a complete cytogenetic response (CCyR), CML relapsed after 28 months of starting it. A CCyR was achieved again by nilotinib but was lost after about 14 months. Only transient response occurred to dasatinib, and the patient died. At relapse, neutrophilia was more predominant than eosinophilia. We reviewed 6 patients with CML whose eosinophil ra…

Neuroendocrine tumor of cecum in patient treated with imatinib mesylate for blastic phase of chronic myeloid leukemia.

Imatinib mesylate (IM), a tyrosine kinase inhibitor, is the treatment of choice in patients with chronic myeloid leukemia (CML). It is considered a very safe drug, with mostly mild and reversible side effects. Lately, it has been suggested that adverse events may occur after a long term. We report a case of a 72-year-old woman diagnosed with blastic phase of Philadelphia chromosome positive CML treated with IM for 28 months. The patient presented first with ascites as a side effect of the drug. When the ascites re-occurred, it was caused by neuroendocrine tumor (NET) with peritoneal carcinomat…

Sudden Visual Loss as an Initial Manifestation of Chronic Myeloid Leukemia.

Chronic myelogenous leukemia (CML) is a pluripotent stem cell disease characterized by anemia, granulocytosis and granulocytic immaturity, basophilia, thrombocytosis and splenomegaly. It is associated with a reciprocal chromosomal translocation t (q34; q11), resulting in a breakpoint cluster region-Abelson fusion gene (Philadelphia chromosome). Ophthalmic manifestations as the first and the only presentation of CML in patients are very rare. Ocular lesions in CML patients are frequently asymptomatic, and thus all patients should undergo an eye evaluation at the initial diagnosis. Here, we repo…

An unusual cause of recurrent laryngospasm: A case report.

INTRODUCTION: Laryngospasm is a partial or complete closure of the vocal cords, causing stridor and then complete airway obstruction. We present an unusual case of recurrent laryngospasm following cervical spine trauma. CASE REPORT: A 41-year-old pedestrian was hit by a car sustaining several spine fractures including a comminuted fracture of C1. These were initially unrecognised, and his cervical spine was not immobilised. During this time the patient experienced three episodes of laryngospasm requiring intubation. On day 11 his fractures were identified, and a Philadelphia collar was placed…

Characterization of a novel variant BCR-ABL1 fusion transcript in a patient with chronic myeloid leukemia: Implications for molecular monitoring.

Molecular monitoring of BCR-ABL1 transcript levels using quantitative polymerase chain reaction is an essential part of the modern management of chronic myeloid leukemia patients treated with tyrosine kinase inhibitors. Establishing the diagnostic BCR-ABL1 fusion transcript is necessary in order to select appropriate primers and probes for such monitoring. A case is described in which quantitative polymerase chain reaction failed to detect the presence of BCR-ABL1 fusion transcript in a Philadelphia chromosome-positive chronic myeloid leukemia patient. Further investigation demonstrated a nove…

The Dercum-Muybridge Collaboration and the Study of Pathologic Gaits Using Sequential Photography.

In the late 1870s and 1880s, prior to the development of movie cameras or projectors, English-American photographer Eadweard Muybridge (1830-1904) photographed sequential images of people and animals in motion, using arrays of sequentially triggered single-image cameras. In 1885, Philadelphia neurologist Francis Dercum (1856-1931) collaborated with Muybridge at the University of Pennsylvania to photograph sequential images of patients with various neurological disorders involving abnormal movements, and particularly various gait disorders, including both the sensory ataxic gait of tabes dorsal…

Historical views, conventional approaches, and evolving management strategies for myeloproliferative neoplasms.

The classical Philadelphia chromosome-negative myeloproliferative neoplasms (MPN), which include essential thrombocythemia, polycythemia vera, and myelofibrosis (MF), are in a new era of molecular diagnosis, ushered in by the identification of the JAK2(V617F) and cMPL mutations in 2005 and 2006, respectively, and the CALR mutations in 2013. Coupled with increased knowledge of disease pathogenesis and refined diagnostic criteria and prognostic scoring systems, a more nuanced appreciation has emerged of the burden of MPN in the United States, including the prevalence, symptom burden, and impact …

Grisel's Syndrome Induced by Mycobacterium tuberculosis.

Grisel's syndrome is a non-traumatic subluxation of the atlantoaxial joints, which is caused by an inflammatory process in the upper neck. It is rare to find literary reports of Grisel's syndrome with an evident pathogen in a lesion. For the first time in Korea, we report a 36-year-old female with Grisel's syndrome having an atlantoaxial subluxation, which was caused by a retropharyngeal abscess secondary to pulmonary Mycobacterium tuberculosis. The patient was treated with an anti-tuberculosis regimen and was prescribed a Philadelphia collar for the control of torticollis. The result of magne…

Реактивация определённой инфекции вирусом гепатита В позволяет избежать мутанта G145R во время лечения дазатинибом хронической лейкемии миэлоида.

Реактивация вируса гепатита В (ГБВ) после иммуноподражающей терапии или трансплантации стволовых клеток является потенциально фатальным осложнением, которое может иметь место даже у пациентов с предварительным разрешением инфекции ГБВ. Dasatinib — небольшой ингибитор тиросина SRC и ABL, который был утвержден для лечения хронической миэлоидной лейкемии (CML) и хромосомной хромосомной острой лимфоболической лейкемии. Здесь мы сообщаем о первом случае возобновления заразной инфекции с иммунным мутантом G145R в пациенте CML, получающем dasatinib. Хотя дазатиниб не признан иммунопрепрессантом, наши

Совпадение в аннотации оригинала
Reactivation of hepatitis B virus (HBV) following immunosuppressive therapy or hematopoietic stem cell transplantation is a potentially fata … kinases SRC and ABL that has been approved for the treatment of chronic myeloid leukemia (CML) and Philadelphia chromosome-positive acute lymphoblastic leukemia. Here, we report the first case of reactivation of resolved infection with the HBV immune escape mutant G145R in a CML patient receiving dasatinib. Although dasatinib is not recognized as an immunosup …

Genetic events other than BCR-ABL1.

The BCR-ABL1 oncoprotein is the cause of chronic myeloid leukemia and occurs as a consequence of the translocation t(9;22), a well-defined genetic event that results in the formation of the Philadelphia chromosome. While this genomic aberration is recognized to be the main culprit of the chronic phase of chronic myeloid leukemia, the natural clonal evolution of this myeloproliferative neoplasm involves the accumulation of secondary alterations through genomic instability. Thus, efforts to dissect the frequency and nature of the genomic events at diagnosis and at later stages are producing valu…

The role of HMGA2 in the proliferation and expansion of a hematopoietic cell in myeloproliferative neoplasms.

Philadelphia chromosome-negative myeloproliferative neoplasms (MPN), which include polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are characterized by clonal proliferative hematopoiesis with increased blood cell count. Clonal expansion mechanisms in MPN and related disorders such as myelodysplastic syndromes (MDS) remain to be elucidated. Although mutations in the JAK2 gene lead to a proliferative hematopoiesis in majority of MPN and some MDS, the mutation alone does not cause a clonal expansion. In addition to JAK2 mutations, several genetic abnormalities, including …

Novel therapeutic agents against cancer stem cells of chronic myeloid leukemia.

Chronic myeloid leukemia (CML) is induced by the BCR-ABL oncogene, a product of Philadelphia (Ph) chromosome. The BCR-ABL kinase inhibitor imatinib is a standard treatment for Ph+ leukemia, and has been shown to induce a complete hematologic and cytogenetic response in most chronic phrase CML patients. However, imatinib does not cure CML, and one of the reasons is that imatinib does not kill leukemia stem cells (LSCs) in CML both in vitro and in vivo. Recently, several new targets or drugs have been reported to inhibit LSCs in cultured human CD34+ CML cells or in mouse model of BCR-ABL induced…

Chronic myeloid leukemia--from the National to the European Registry--limited experience of a single center.

Chronic myeloid leukemia (CML) is a hematopoietic stem cell disorder in which the diagnosis is confirmed by detection of a genetic marker: Philadelphia (Ph) chromosome in almost 90% of cases. Some of Ph1 negative patients, nevertheless, test positive for the abnormal gene, or the abnormal protein associated with this chromosome, when more sensitive studies, such as PCR or FISH are used and nowadays the diagnosis of CML is based, not only on cytogenetic, but also on molecular analysis. The better understanding of the CML biology provided by the latest researches requires a deeper knowledge abou…

Alexander Gordon, puerperal sepsis, and modern theories of infection control--Semmelweis in perspective.

Ignaz Semmelweis, a Hungarian doctor who practised in 19th century Vienna, is widely believed to be the father of modern infection control. … eral sepsis that began in Aberdeen in 1789. Gordon's thesis was reprinted three times in Edinburgh, Philadelphia, and London over the next 55 years, suggesting that Semmelweis (1847) could well have known of his work. Like Semmelweis, Gordon was persecuted for his findings.

Pathological interactions between hematopoietic stem cells and their niche revealed by mouse models of primary myelofibrosis.

Primary myelofibrosis (PMF) belongs to the Philadelphia-negative myeloproliferative neoplasms and is a hematological disorder caused by abnormal function of the hematopoietic stem cells. The disease manifests itself with a plethora of alterations, including anemia, splenomegaly and extramedullary hematopoiesis. Its hallmarks are progressive marrow fibrosis and atypical megakaryocytic hyperplasia, two distinctive features used to clinically monitor disease progression. In an attempt to investigate the role of abnormal megakaryocytopoiesis in the pathogenesis of PMF, several transgenic mouse mod…

Characterization of the heartworm Acanthocheilonema spirocauda (Leidy, 1858) Anderson, 1992 (Nematoda: Onchocercidae) in Scandinavia.

The heartworm Acanthocheilonema spirocauda (Leidy, Proc Acad Nat Sci Philadelphia 10:110-112, 1858) Anderson, 1992 is described from material collected from harbour seals in Scandinavia and compared with types and other specimens described by Anderson (Can J Zool 37:481-493, 1959) from harbour seals in eastern USA. Most morphometric characters of the material from USA fall within the ranges established for the Scandinavian one. Some intraspecific variability in the organisation of papillae on the male tail was detected among the Scandinavian specimens. Differences between the specimens from Sc…

Surfactant respiratory therapy using Surfaxin/sinapultide.

Respiratory distress syndrome (RDS) is a leading cause of mortality and morbidity in preterm infants. Surfactant replacement therapy has bee … o development of newer synthetic surfactants such as lucinactant (Surfaxin, Discovery Laboratories, Philadelphia) which contains the protein B mimic synthetic peptide, sinapultide. Recent phase 3 clinical trials with Surfaxin show promising results with similar efficacy as animal derived surfactants and yet avoiding the disadvantage associated with animal prod …