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1.
Hematology ; 29(1): 2340149, 2024 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-38626148

RESUMEN

OBJECTIVES: Patients with myeloproliferative neoplasms (MPNs) are at higher risk of developing secondary malignancies. In this study, we focused on patients with MPNs that complicated lymphoid neoplasms. To analyze the real-world status of lymphoid neoplasm treatment in patients with pre-existing MPNs in Japan, we conducted a multicenter retrospective study. METHODS: Questionnaires were sent to collect the data on patients who were first diagnosed with either polycythemia vera, essential thrombocythemia or myelofibrosis and who later were complicated with lymphoid neoplasms defined as malignant lymphoma, multiple myeloma, or chronic lymphocytic leukemia/small cell lymphoma. RESULTS: Twenty-four patients with MPNs complicated by lymphoid neoplasms were enrolled (polycythemia vera, n = 8; essential thrombocythemia, n = 14; and primary myelofibrosis, n = 2). Among these, diffuse large B-cell lymphoma (DLBCL) was the most frequently observed (n = 13, 54.1%). Twelve (92.3%) of the patients with DLBCL received conventional chemotherapy. Among these 12 patients, regarding cytoreductive therapy for MPNs, 8 patients stopped treatment, one continued treatment, and two received a reduced dose. Consequently, most patients were able to receive conventional chemotherapy for DLBCL with a slightly higher dose of granulocyte colony-stimulating factor support than usual without worse outcomes. All 3 patients with multiple myeloma received a standard dose of chemotherapy. CONCLUSION: Our data indicate that if aggressive lymphoid neoplasms develop during the course of treatment in patients with MPNs, it is acceptable to prioritize chemotherapy for lymphoma.


Asunto(s)
Leucemia Linfocítica Crónica de Células B , Linfoma , Mieloma Múltiple , Trastornos Mieloproliferativos , Policitemia Vera , Trombocitemia Esencial , Humanos , Trombocitemia Esencial/tratamiento farmacológico , Trombocitemia Esencial/epidemiología , Estudios Retrospectivos , Japón/epidemiología , Trastornos Mieloproliferativos/tratamiento farmacológico , Trastornos Mieloproliferativos/epidemiología , Trastornos Mieloproliferativos/diagnóstico , Linfoma/epidemiología , Linfoma/etiología , Linfoma/terapia
2.
Int J Hematol ; 118(1): 47-53, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37058247

RESUMEN

Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T) is a rare disease, which presents with features of myelodysplastic syndromes with ring sideroblasts and essential thrombocythemia, as well as anemia and marked thrombocytosis. SF3B1 and JAK2 mutations are often found in patients, and are associated with their specific clinical features. This study was a retrospective analysis of 34 Japanese patients with MDS/MPN-RS-T. Median age at diagnosis was 77 (range, 51-88) years, and patients had anemia (median hemoglobin: 9.0 g/dL) and thrombocytosis (median platelet count: 642 × 109/L). Median overall survival was 70 (95% confidence interval: 68-not applicable) months during the median follow-up period of 26 (range: 0-91) months. A JAK2V617F mutation was detected in 46.2% (n = 12) of analyzed patients (n = 26), while an SF3B1 mutation was detected in 87.5% (n = 7) of analyzed patients (n = 8). Like those with myelodysplastic syndromes or myeloproliferative neoplasms, patients often received erythropoiesis-stimulating agents and aspirin to improve anemia and prevent thrombosis. This study, which was the largest to describe the real-world characteristics of Japanese patients with MDS/MPN-RS-T, showed that the patients had similar characteristics to those in western countries.


Asunto(s)
Anemia Sideroblástica , Síndromes Mielodisplásicos , Enfermedades Mielodisplásicas-Mieloproliferativas , Neoplasias , Trombocitosis , Humanos , Anemia Sideroblástica/genética , Estudios Retrospectivos , Pueblos del Este de Asia , Síndromes Mielodisplásicos/genética , Enfermedades Mielodisplásicas-Mieloproliferativas/genética , Trombocitosis/genética , Neoplasias/complicaciones , Mutación , Factores de Empalme de ARN/genética
3.
J Clin Exp Hematop ; 62(4): 249-252, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36575001

RESUMEN

In this study, we examined a cohort of Japanese patients with acute myeloid leukemia (AML) with cup-like nuclei. In particular, we attempted to provide a detailed definition of the clinical features of AML with cup-like nuclei. The clinical records of patients diagnosed with de novo AML were collected retrospectively. We showed that approximately 23% of all patients with AML diagnosed during the study period had AML with cup-like nuclei. All three cup-like AML cases had FLT3-ITD mutations. In addition, we reported a high incidence of disseminated intravascular coagulation and acute cerebral infarction in patients with AML with cup-like nuclei. Our results show that AML with cup-like nuclei may be more common than expected. Due to these unique characteristics, recognition of this morphology is recommended.


Asunto(s)
Coagulación Intravascular Diseminada , Leucemia Mieloide Aguda , Humanos , Coagulación Intravascular Diseminada/etiología , Estudios Retrospectivos , Incidencia , Nucleofosmina , Mutación , Leucemia Mieloide Aguda/epidemiología , Leucemia Mieloide Aguda/genética , Enfermedad Aguda , Infarto Cerebral , Tirosina Quinasa 3 Similar a fms/genética , Pronóstico
4.
Intern Med ; 61(22): 3421-3424, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36385048

RESUMEN

Immune checkpoint inhibitors (ICIs) are widely used for the treatment of various cancers. However, paradoxical exacerbation of neoplasms, referred to as "hyperprogressive disease," has been reported in a proportion of patients treated with anti-programmed cell death-1 (PD-1)/PD-1 ligand (PD-L1) blockade. We herein report a case of acute adult T-cell leukemia (ATL) that developed shortly after the administration of nivolumab, a PD-1 inhibitor, to treat non-small-cell lung cancer. There were no signs of ATL before the administration of nivolumab, and seropositivity for human T-cell leukemia virus type-1 (HTLV-1) was confirmed after the development of acute ATL. We speculate that nivolumab likely contributed to the development of acute ATL.


Asunto(s)
Carcinoma de Pulmón de Células no Pequeñas , Leucemia-Linfoma de Células T del Adulto , Neoplasias Pulmonares , Humanos , Neoplasias Pulmonares/terapia , Carcinoma de Pulmón de Células no Pequeñas/terapia , Receptor de Muerte Celular Programada 1 , Nivolumab/efectos adversos , Leucemia-Linfoma de Células T del Adulto/tratamiento farmacológico
5.
EJHaem ; 3(3): 992-995, 2022 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-35941885

RESUMEN

Paroxysmal cold hemoglobinuria (PCH) is an extremely rare subtype of autoimmune hemolytic anemia (AIHA) in adults. PCH is caused by the biphasic Donath-Landsteiner (DL) antibody which fixes complement to red blood cells at low temperatures and dissociates at warmer temperatures, leading to complement-mediated intravascular hemolysis. Autoimmune hematological disorders including AIHA and immune thrombocytopenia have been reported to develop following the mRNA COVID-19 vaccination. However, PCH developing subsequent to mRNA vaccination has never been reported. We report a 59-year-old male who developed PCH approximately a month after his second mRNA COVID-19 vaccination.

6.
Int J Hematol ; 116(5): 696-711, 2022 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-35809214

RESUMEN

The presence of a JAK2 V617F or JAK2 exon 12 mutation is one of the three major criteria listed for the diagnosis of polycythemia vera (PV) in the 2017 World Health Organization Classification. However, a nationwide study has not yet been conducted in Japan since the discovery of JAK2 mutations. Therefore, the Japanese Society of Hematology (JSH) retrospectively analyzed the clinical characteristics of 596 Japanese patients with PV diagnosed between April 2005 and March 2018. Among the 473 patients with complete data on JAK2 mutations available, 446 (94.3%) and 10 (2.1%) were positive for the JAK2 V617F and JAK2 exon 12 mutations, respectively. During a median follow-up of 46 months (range: 0-179 months), 47 (7.9%) deaths occurred. The major causes of death were secondary malignancies (23.4%), acute leukemia (12.8%), non-leukemic progressive disease (10.6%) and thrombotic (6.4%) and hemorrhagic complications (6.4%). Thrombotic and hemorrhagic events occurred during the clinical course in 4.0% (n = 24) and 3.5% (n = 21) of patients, respectively. These results show that the international PV prognostic score (age, venous thrombosis and leukocytosis) is applicable to Japanese patients with PV.


Asunto(s)
Hematología , Policitemia Vera , Trombosis , Humanos , Policitemia Vera/complicaciones , Japón/epidemiología , Janus Quinasa 2/genética , Estudios Retrospectivos , Trombosis/etiología , Mutación
7.
Jpn J Clin Oncol ; 52(8): 880-886, 2022 08 05.
Artículo en Inglés | MEDLINE | ID: mdl-35575284

RESUMEN

BACKGROUND: The prognosis of Philadelphia chromosome-negative myeloproliferative neoplasms is relatively favorable, but the quality of life can be severely affected by myeloproliferative neoplasm-related symptoms such as fatigue, pruritus, night sweats, bone pain, fever and weight loss. In this study, we administered hochuekkito, a traditional herbal medicine, to patients with myeloproliferative neoplasms and investigated whether there was a reduction in myeloproliferative neoplasm-related symptoms. METHODS: We conducted a randomized parallel-group pilot study. Patients were assigned to a hochuekkito administration or non-hochuekkito administration group. Myeloproliferative neoplasm-related symptoms based on Myeloproliferative Neoplasm Symptom Assessment Form total symptom score and European Organization for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30 were examined before hochuekkito administration and 4 and 8 weeks after administration. RESULTS: Among the 42 patients included in the analysis, 21 were assigned to the hochuekkito group and 21 were assigned to the control group. After administering hochuekkito, the median values of Myeloproliferative Neoplasms Symptom Assessment Form total symptom score at 4 and 8 weeks in the hochuekkito group demonstrated a decreasing trend; however, the difference between the two groups was not significant. CONCLUSIONS: In this study, we were unable to demonstrate significant differences between the hochuekkito and control groups in terms of the efficacy of hochuekkito in treating myeloproliferative neoplasm-related symptoms. However, there were cases that presented prominent improvement in symptoms in the hochuekkito group. The only reported adverse event was grade 1 impaired hepatic function. Therefore, hochuekkito might be a therapeutic option for patients with severely affected quality of life due to myeloproliferative neoplasm-related symptoms.


Asunto(s)
Medicamentos Herbarios Chinos , Trastornos Mieloproliferativos , Calidad de Vida , Medicamentos Herbarios Chinos/farmacología , Medicamentos Herbarios Chinos/uso terapéutico , Fatiga , Humanos , Trastornos Mieloproliferativos/diagnóstico , Trastornos Mieloproliferativos/tratamiento farmacológico , Neoplasias/tratamiento farmacológico , Proyectos Piloto , Extractos Vegetales/farmacología , Extractos Vegetales/uso terapéutico , Perfil de Impacto de Enfermedad
8.
Rinsho Ketsueki ; 63(1): 20-25, 2022.
Artículo en Japonés | MEDLINE | ID: mdl-35135947

RESUMEN

Immunosuppressive therapies, including antithymocyte globulin and cyclosporine (CsA), are used for the treatment of aplastic anemia, but they reportedly cause lymphoproliferative diseases. Here, we report two cases of aplastic anemia in which diffuse large B-cell lymphoma developed during treatment with CsA. In both the cases, CsA was discontinued and combination therapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisolone) plus the thrombopoietin receptor agonist eltrombopag was initiated. Furthermore, supportive care, including blood transfusion and granulocyte colony-stimulating factor, was provided. After six or eight courses of R-CHOP therapy, a complete metabolic response was achieved without serious adverse events. These cases illustrate the safety of combining R-CHOP with eltrombopag therapy in patients at a high risk of severe pancytopenia.


Asunto(s)
Anemia Aplásica , Linfoma de Células B Grandes Difuso , Receptores de Trombopoyetina/agonistas , Anemia Aplásica/complicaciones , Anemia Aplásica/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ciclofosfamida/uso terapéutico , Doxorrubicina/uso terapéutico , Humanos , Linfoma de Células B Grandes Difuso/complicaciones , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Prednisona/uso terapéutico , Rituximab/uso terapéutico , Vincristina/uso terapéutico
9.
Int J Hematol ; 115(2): 208-221, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-34727329

RESUMEN

We conducted a large-scale, nationwide retrospective study of Japanese patients who were diagnosed with essential thrombocythemia based on the diagnostic criteria in the World Health Organization classification. We investigated clinical characteristics, survival rates, and the incidence of thrombohemorrhagic events as well as risk factors for these events. A total of 1152 patients were analyzed in the present study. Median age at diagnosis was 65 years, the median platelet count was 832 × 109/L, and the positive mutation rates of JAK2V617F, CALR, and MPL were 62.8, 25.1, and 4.1%, respectively. Compared with European and American patients, Japanese patients were more likely to have cardiovascular risk factors and less likely to have systemic symptoms including palpable splenomegaly. Thrombocytosis was identified as a risk factor for hemorrhagic events and prognosis, but not for thrombotic events. The prognostic factors and risk classifications reported in Europe and the United States were generally applicable to Japanese patients. Regarding transformations, secondary myelofibrosis progressed in a time-dependent manner, but progression to acute leukemia was low in "true" ET patients. Skin cancers were less common and gastrointestinal cancers more common as secondary malignancies in Japanese patients, suggesting ethnic differences.


Asunto(s)
Trombocitemia Esencial/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Japón/epidemiología , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Análisis de Supervivencia , Trombocitemia Esencial/complicaciones , Trombocitemia Esencial/epidemiología , Adulto Joven
10.
Leuk Res Rep ; 16: 100269, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34631406

RESUMEN

Although a previous autopsy series demonstrated that pulmonary leukemic infiltration (PLI) is a major pulmonary complication in patients with acute myeloid leukemia (AML), an antemortem diagnosis of PLI is rare. Diverse pulmonary complications cause acute respiratory failure (ARF) in patients with AML undergoing chemotherapy. This article reports two elderly patients with AML who presented with ARF due to PLI mimicking severe pneumonia during induction chemotherapy. Accurate antemortem diagnosis of PLI was almost impossible without pathological examination since the clinical course was not typical of PLI. We recommend considering PLI in patients with AML who have an unknown etiology of ARF.

11.
Intern Med ; 60(20): 3309-3315, 2021 Oct 15.
Artículo en Inglés | MEDLINE | ID: mdl-33967141

RESUMEN

Primary skeletal muscle lymphoma is extremely uncommon, and there have only been eight previous case reports on primary skeletal muscle peripheral T-cell lymphoma, not otherwise specified (PSM-PTCL, NOS). We herein report an autopsy case of a 71-year-old woman with PSM-PTCL, NOS, who had a 24-year history of systemic sclerosis treated with immunosuppressive drugs. A post-mortem examination revealed infiltration of lymphoma cells positive for T-cell markers, cytotoxic markers, and p53. This case was considered to be one of other iatrogenic immunodeficiency-associated lymphoproliferative disorder (OIIA-LPD). This is the first case categorized under both PSM-PTCL, NOS, and OIIA-LPD.


Asunto(s)
Linfoma de Células T Periférico , Trastornos Linfoproliferativos , Anciano , Autopsia , Femenino , Humanos , Inmunosupresores , Linfoma de Células T Periférico/diagnóstico , Músculo Esquelético
12.
J Clin Exp Hematop ; 61(2): 102-108, 2021 Jun 05.
Artículo en Inglés | MEDLINE | ID: mdl-33994430

RESUMEN

Lymphoma-associated hemophagocytic lymphohistiocytosis (HLH) has a significantly poor prognosis among secondary HLH. We describe the rare case of a 74-year-old female with secondary HLH presenting with a rapidly fatal course. Post-mortem examination revealed Epstein-Barr virus (EBV) -positive diffuse large B-cell lymphoma (DLBCL). We were unable to make a definite antemortem diagnosis because the patient did not exhibit lymphadenopathy and bone marrow biopsy demonstrated hemophagocytosis without evidence of lymphoma. She died of multiple organ failure on the twelfth day of hospitalization despite a temporary response to steroids. Autopsy revealed diffuse lymphoma cell infiltration of the bone marrow, liver and spleen, suggesting "bone marrow-liver-spleen" (BLS)-type large B-cell lymphoma (LBCL). BLS-type LBCL is a rare and clinically aggressive lymphoma, usually associated with fever, cytopenia and HLH. The disease has a high mortality rate due to the delay in diagnosis and a highly aggressive clinical course. Further studies are required to improve our understanding of this rare extranodal DLBCL.


Asunto(s)
Médula Ósea/patología , Infecciones por Virus de Epstein-Barr/complicaciones , Hígado/patología , Linfohistiocitosis Hemofagocítica/complicaciones , Linfoma de Células B Grandes Difuso/complicaciones , Bazo/patología , Anciano , Autopsia , Biopsia , Infecciones por Virus de Epstein-Barr/patología , Femenino , Humanos , Linfohistiocitosis Hemofagocítica/patología , Linfoma de Células B Grandes Difuso/patología
13.
Rinsho Ketsueki ; 61(10): 1511-1513, 2020.
Artículo en Japonés | MEDLINE | ID: mdl-33162450

RESUMEN

The 2017 World Health Organization (WHO) classification states that acute promyelocytic leukemia (APL) always presents with strong myeloperoxidase staining. However, we herein report of a 40-year-old woman with the microgranular variant of acute promyelocytic leukemia presenting with weak myeloperoxidase (MPO) staining. The leukemic cells were morphologically similar to monocytic cells, showing distorted-shaped nuclei and weak MPO staining. However, flow cytometry revealed positivity of CD2, CD34, and human leucocyte antigen-DR (HLA-DR) and pointed toward a diagnosis of APL. PML-RARA mRNA detection finally led the patient to a definitive diagnosis. The patient achieved complete remission by induction chemotherapy including tretinoin, cytarabine and idarubicin, and no differentiation syndrome was observed.


Asunto(s)
Leucemia Promielocítica Aguda , Adulto , Femenino , Citometría de Flujo , Humanos , Leucemia Promielocítica Aguda/diagnóstico , Leucemia Promielocítica Aguda/tratamiento farmacológico , Peroxidasa , Coloración y Etiquetado , Tretinoina
14.
Rinsho Ketsueki ; 60(8): 910-914, 2019.
Artículo en Japonés | MEDLINE | ID: mdl-31484888

RESUMEN

A 72-year-old woman with chronic myeloid leukemia (CML) and cirrhosis complicated with blood blisters on her right upper arm and ascites was admitted. She presented with shock vital on admission. Initial gram staining of blood cultures showed gram-positive cocci in chains, suggesting streptococcal toxic shock syndrome (STSS). Amputation of the right upper arm was performed owing to necrotizing fasciitis. Despite continued antibiotic therapy and systemic management, the blood blisters rapidly spread to the skin of the whole body, and she died 41 h after admission. Blood and fluid cultures from the blisters showed group B streptococci. Reports of patients with leukemia complicated with STSS are rare, and all cases have followed fatal courses. Particularly in this case, various risk factors, such as neutropenia due to tyrosine kinase inhibitor, neutrophil dysfunction due to cirrhosis, and elderly CML, overlapped. In the future, we believe that the lives of patients with leukemia complicated with STSS may be saved by establishing treatment methods and determining the detailed pathogenesis of STSS.


Asunto(s)
Leucemia Mielógena Crónica BCR-ABL Positiva , Cirrosis Hepática , Choque Séptico , Infecciones Estreptocócicas , Anciano , Femenino , Humanos , Choque Séptico/etiología , Streptococcus agalactiae , Streptococcus pyogenes
16.
Int J Hematol ; 108(6): 588-597, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30151740

RESUMEN

Dasatinib induces lymphocytosis of large granular lymphocytes (LGLs) in a proportion of patients with chronic myelogenous leukemia (CML), and is associated with better clinical outcomes. LGLs consist of cytotoxic T lymphocytes and natural killer cells; however, the context and phenotypic/functional features of each type of LGL are unknown. To better define features of these LGLs, we investigated lymphocytosis in CML patients treated with dasatinib. D57-positive and CD4-positive type I T-helper (Th) cells (CD57+ Th cells) rarely occur in CML patients without lymphocytosis and in healthy individuals; however, a substantial increase in the proportion of CD57+ Th cells was observed in CML patients treated with dasatinib. In addition, these cells showed appreciable levels of cytocidal activity via cytotoxic degranulation. Analysis of T-cell receptor α and ß sequences showed a skewed T-cell repertoire in the CD57+ Th cells. Furthermore, patients with LGLs and CD57+ Th lymphocytosis achieved stronger molecular responses than did those without lymphocytosis. While further studies are warranted, our observations suggest that dasatinib induces the expansion of CD57+ Th-LGLs, which may play a crucial role in the dasatinib-induced response against Philadelphia chromosome-positive leukemia.


Asunto(s)
Linfocitos T CD4-Positivos/inmunología , Linfocitos T CD4-Positivos/metabolismo , Antígenos CD57/metabolismo , Dasatinib/efectos adversos , Inmunidad Celular/efectos de los fármacos , Leucemia Mielógena Crónica BCR-ABL Positiva/inmunología , Leucemia Mielógena Crónica BCR-ABL Positiva/metabolismo , Adulto , Anciano , Linfocitos T CD4-Positivos/efectos de los fármacos , Citotoxicidad Inmunológica , Dasatinib/uso terapéutico , Femenino , Perfilación de la Expresión Génica , Humanos , Inmunofenotipificación , Células Asesinas Naturales/efectos de los fármacos , Células Asesinas Naturales/inmunología , Células Asesinas Naturales/metabolismo , Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Leucemia Mielógena Crónica BCR-ABL Positiva/patología , Linfocitosis/patología , Masculino , Persona de Mediana Edad , Receptores de Antígenos de Linfocitos T alfa-beta/genética , Subgrupos de Linfocitos T/efectos de los fármacos , Subgrupos de Linfocitos T/inmunología , Subgrupos de Linfocitos T/metabolismo , Linfocitos T Citotóxicos/efectos de los fármacos , Linfocitos T Citotóxicos/inmunología , Linfocitos T Citotóxicos/metabolismo , Resultado del Tratamiento
17.
Int J Hematol ; 108(5): 491-498, 2018 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-30121892

RESUMEN

Cytoreductive therapy is used in high-risk essential thrombocythemia (ET) to reduce risk of thrombohemorrhagic complications. Anagrelide is an orally active, quinazolone-derived platelet-lowering agent approved for first-line treatment of high-risk ET in Japan. Long-term safety and efficacy data were collected from 53 Japanese high-risk ET patients (Study 308); 41 patients who completed Study 308 entered this phase 3b, open-label extension (Study 309; NCT01467661). Reductions in mean platelet counts occurred throughout the study, from 1021.6 × 109/L (at Study 308 baseline) to 675.4 × 109/L at final assessment. At month 48 (since Study 308 enrollment), mean platelet count was 444.5 × 109/L in the 10 patients who completed 4 years of therapy. Overall, platelet counts decreased from 1088.3 × 109/L at Study 308 baseline (n = 33) to 473.5 × 109/L at final assessment (n = 31). Long-term platelet count reductions were maintained without marked changes in mean anagrelide dose. Anagrelide was generally well tolerated, with anemia (54.7%) and headache (49.1%) as the most frequent adverse events. These findings indicate that anagrelide effectively reduces platelet counts in high-risk Japanese ET patients, with titration resulting in a well-tolerated, effective and sustainable dose. In conclusion, these results support anagrelide administration to high-risk Japanese ET patients using individualized dosing strategies defined in instructions previously approved in Europe and the USA.


Asunto(s)
Quinazolinas/administración & dosificación , Trombocitemia Esencial/tratamiento farmacológico , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Japón/epidemiología , Masculino , Persona de Mediana Edad , Recuento de Plaquetas , Quinazolinas/efectos adversos , Factores de Riesgo , Trombocitemia Esencial/sangre , Trombocitemia Esencial/epidemiología , Factores de Tiempo
18.
Rinsho Ketsueki ; 59(6): 669-674, 2018.
Artículo en Japonés | MEDLINE | ID: mdl-29973441

RESUMEN

The JAK2V617F mutation is the commonest major genetic mutation of myeloproliferative neoplasms (MPNs) and has been defined in the WHO diagnostic criteria for MPNs. However, there is still no approved in vitro diagnostic test kit available in Japan. We evaluated a JAK2V617F allele quantification kit (test method) in a prospective, multicenter clinical performance study involving patients with MPNs who were diagnosed with polycythemia vera, essential thrombocythemia, and primary myelofibrosis; healthy volunteers were also included in the analysis. Good correlation was observed between the allele burden determined using the test method vs. that determined using next-generation sequencing (NGS) in the patient group (r=0.998, y=1.071x-0.069; n=156). Furthermore, all allele burdens in the healthy group (n=54) were below the lower limit of the measurement range of the test method (0.042%). Our results confirmed that the test method could quantitatively measure the JAK2V617F allele burden in patients with MPN. Thus, the novel JAK2V617F allele quantification kit can be considered useful for the diagnosis of MPNs.


Asunto(s)
Pruebas Genéticas/instrumentación , Janus Quinasa 2/genética , Trastornos Mieloproliferativos/genética , Policitemia Vera/genética , Estudios de Casos y Controles , Humanos , Japón , Mutación , Estudios Prospectivos
20.
Oncology ; 94(2): 85-91, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29151104

RESUMEN

OBJECTIVE: The use of tyrosine kinase inhibitors led to an improvement in the prognoses of patients with chronic myeloid leukemia (CML). The aims of this study were to investigate the efficacy and safety of dasatinib in Japanese patients and to explore the factors that affect the achievement of molecular responses. METHODS: The primary endpoint was a major molecular response (MMR) by 12 months. The halving time for BCR-ABL1 transcripts was calculated using transcript levels. RESULTS: Thirty-two patients with chronic-phase CML (CML-CP) were enrolled and 30 received 100 mg dasatinib once daily. At 24 months of follow-up, 21 (72%) and 24 (83%) patients achieved an MMR by 12 and 24 months, respectively; the rates of a deep molecular response (DMR) by 12 and 24 months were 48 and 59%, respectively. A shorter halving time of BCR-ABL1 transcripts (≤10.6 days) accurately predicted both an MMR and a DMR. The incidence of pleural effusion was 50%. Our study reconfirmed the efficacy and safety of dasatinib treatment in Japanese patients with newly diagnosed CML-CP. In addition, the usefulness of the halving time of BCR-ABL1 transcripts was validated. CONCLUSION: These data emphasize the significance of an early treatment response in achieving a DMR during dasatinib therapy.


Asunto(s)
Antineoplásicos/uso terapéutico , Dasatinib/uso terapéutico , Proteínas de Fusión bcr-abl/genética , Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Leucemia Mieloide de Fase Crónica/tratamiento farmacológico , Inhibidores de Proteínas Quinasas/uso terapéutico , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Japón , Leucemia Mielógena Crónica BCR-ABL Positiva/genética , Leucemia Mieloide de Fase Crónica/genética , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos
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