RESUMEN
A 77-year-old Japanese man with disseminated Mycobacterium avium complex (MAC) disease due to anti-interferon-gamma autoantibodies received rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) chemotherapy because of non-Hodgkin lymphoma complication. The hepatobiliary nodules due to MAC resolved with R-CHOP and multidrug antimycobacterial treatment. R-CHOP could serve as an alternative adjunctive therapy for patients with anti-interferon-gamma autoantibodies.
RESUMEN
OBJECTIVES: Increasing numbers of reports have described hematopoietic improvement after iron chelation therapy in iron-overloaded patients. These observations indicate that excess iron could affect hematopoiesis unfavorably. To investigate how excess iron affects hematopoiesis in vivo, we generated iron-overloaded mice and examined hematopoietic parameters in these mice. METHODS: We generated iron-overloaded mice by injecting 200 mg of iron dextran into C57BL/6J mice, and immature hematopoietic cells in the bone marrow were evaluated by flow cytometric analyses, colony-forming assays, and bone marrow transplantation analyses. We also examined changes in molecular profiles of the hematopoietic microenvironment. RESULTS AND CONCLUSIONS: Iron-overloaded (IO) mice did not show significant defects in the hematopoietic data of the peripheral blood. Myeloid progenitor cells in the bone marrow were increased in IO mice, but the number and function of the erythroid progenitors and hematopoietic stem cells were not significantly affected. However, bone marrow transplantation from normal donors to IO recipients showed delayed hematopoietic reconstitution, which indicates that excess iron impacts the hematopoietic microenvironment negatively. Microarray and quantitative RT-PCR analyses on the bone marrow stromal cells demonstrated remarkably reduced expression of CXCL12, VCAM-1, Kit-ligand, and IGF-1 in the iron-overloaded mice. In addition, erythropoietin and thrombopoietin levels were significantly suppressed, and increased oxidative stress was observed in the IO bone marrow and liver. Consequently, our findings indicate that excess iron can damage bone marrow stromal cells and other vital organs, disrupting hematopoiesis presumably by increased oxidative stress.
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Células de la Médula Ósea/metabolismo , Médula Ósea/metabolismo , Microambiente Celular/genética , Sobrecarga de Hierro/genética , Hígado/metabolismo , Células Madre Mesenquimatosas/metabolismo , Animales , Médula Ósea/patología , Células de la Médula Ósea/patología , Trasplante de Médula Ósea , Quimiocina CXCL12/genética , Quimiocina CXCL12/metabolismo , Eritropoyetina/genética , Eritropoyetina/metabolismo , Expresión Génica , Factor I del Crecimiento Similar a la Insulina/genética , Factor I del Crecimiento Similar a la Insulina/metabolismo , Sobrecarga de Hierro/inducido químicamente , Sobrecarga de Hierro/metabolismo , Sobrecarga de Hierro/patología , Complejo Hierro-Dextran , Hígado/patología , Células Madre Mesenquimatosas/patología , Ratones , Ratones Endogámicos C57BL , Análisis por Micromatrices , Estrés Oxidativo , Factor de Células Madre/genética , Factor de Células Madre/metabolismo , Trombopoyetina/genética , Trombopoyetina/metabolismo , Molécula 1 de Adhesión Celular Vascular/genética , Molécula 1 de Adhesión Celular Vascular/metabolismoRESUMEN
Bone marrow mononuclear cells from 93 patients with hematological malignancies after allogeneic hematopoietic stem cell transplantation (AHSCT) were analyzed using flow cytometry (FCM). The disease was acute myeloblastic leukemia (50 patients), acute lymphoblastic leukemia, and others. Conditioning was myeloablative (80 patients) or reduced intensity. The stem cell source was bone marrow (75 patients), peripheral blood stem cells, or cord blood. After AHSCT, granulocyte colony-stimulating factor was given to all patients. All patients showed engraftment of the donor cells. FCM was conducted on a median of 22 days after AHSCT. The gate was set around a granulocytic region consisting of immature granulocytes. The positivity rates of CD13, CD14, CD15, CD33, CD34, CD56, and HLA-DR in the cells were 59.9 ± 27.4%, 5.8 ± 8.8%, 98.3 ± 1.9%, 92.3 ± 12.4%, 2.6 ± 5.8%, 24.3 ± 16.7%, and 9.1 ± 6.6%, respectively. The greatest value of CD56 positivity was 73.1%. On the basis of CD56 expression, cases of more than 24% CD56 positivity were assigned to the CD56-high group (39 patients), while the rest were assigned to the CD56-low/negative group. There were no significant differences between the two groups in terms of disease status, sex, donor, hematopoietic stem cells, days of FCM analysis, or peripheral blood cell counts around the days of performing FCM. These results indicate that CD56 can be expressed in normal immature granulocytes at a variety of expression levels in regenerative bone marrow. Attention should be paid when evaluating aberrant antigen expression of CD56 in granulocytes.
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Antígeno CD56/metabolismo , Granulocitos/citología , Granulocitos/metabolismo , Trasplante de Células Madre Hematopoyéticas , Adolescente , Adulto , Anciano , Antígenos de Superficie/metabolismo , Antígeno CD56/genética , Femenino , Expresión Génica , Humanos , Inmunofenotipificación , Masculino , Persona de Mediana Edad , Trasplante Homólogo , Adulto JovenAsunto(s)
Enfermedad Granulomatosa Crónica/diagnóstico , Neprilisina/deficiencia , Neutrófilos/inmunología , Infecciones Estafilocócicas/diagnóstico , Expresión Génica , Enfermedad Granulomatosa Crónica/complicaciones , Enfermedad Granulomatosa Crónica/inmunología , Enfermedad Granulomatosa Crónica/microbiología , Humanos , Masculino , Neprilisina/genética , Neprilisina/inmunología , Neutrófilos/patología , Infecciones Estafilocócicas/complicaciones , Infecciones Estafilocócicas/inmunología , Infecciones Estafilocócicas/microbiología , Staphylococcus aureus/inmunología , Factores de Tiempo , Adulto JovenRESUMEN
Chronic myelogenous leukemia (CML) has a typical progressive course with transition from a chronic phase to a terminal blast crisis phase. The mechanisms that lead to disease progression remain to be elucidated. To understand the role of aberrant methylation in the progression of CML, DNA methylation patterns in 16 patients with CML blast crisis were analyzed. Methylation status was analyzed by methylation-specific PCR (MSP) for 13 genes, including cell cycle regulating genes, DNA repair genes, apoptosis-related genes, a differentiation-associated gene and a cytokine signaling gene. The frequency of samples with methylation in each of the following genes were: p15, 18%; MGMT, 12%; RARß, 12%; p16, 6%; DAPK, 6% and FHIT, 6%. In total, four (25%) cases showed methylation of at least one gene. None of the 16 cases showed hypermethylation of the hMLH1 or hMSH2 genes. These results suggest that hypermethylation of cell cycle control genes, but not DNA mismatch repair genes, play a significant role in the progression of CML.
RESUMEN
In the rituximab era, several large studies have suggested that full-dose rituximab plus cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) might be the best treatment for patients with diffuse large B-cell lymphoma (DLBCL) aged 60 years and older. However, it remains unclear whether this is also the case for those aged 70 years and older. Previously untreated patients with DLBCL aged 70 years and older (elderly) were treated with R-70%CHOP, and patients younger than 70 years (younger) were treated with full-dose R-CHOP every 3 weeks, for a total of 6-8 cycles. Complete remission (CR) rates in elderly versus younger patients were 75 vs. 78% (p = 0.7), respectively. The 3-year overall survival, event-free survival and progression-free survival of elderly versus younger patients were 58 vs. 78% (p < 0.05), 45 vs. 70% (p < 0.05) and 64 vs. 72% (p = 0.43), respectively. Severe adverse events were more frequent in the elderly, even with the dose reduction in that age group. Three-year PFS with R-70%CHOP for patients aged 70 years and older was not significantly worse than that with full-dose R-CHOP for younger patients, suggesting that R-70% CHOP might be a reasonable choice for patients with DLBCL aged 70 years and older, especially for those with comorbidities.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Anciano , Anciano de 80 o más Años , Anticuerpos Monoclonales de Origen Murino/administración & dosificación , Anticuerpos Monoclonales de Origen Murino/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Pueblo Asiatico , Ciclofosfamida/administración & dosificación , Ciclofosfamida/efectos adversos , Supervivencia sin Enfermedad , Doxorrubicina/administración & dosificación , Doxorrubicina/efectos adversos , Esquema de Medicación , Femenino , Humanos , Japón , Estimación de Kaplan-Meier , Leucopenia/inducido químicamente , Linfoma de Células B Grandes Difuso/etnología , Masculino , Estudios Retrospectivos , Rituximab , Trombocitopenia/inducido químicamente , Resultado del Tratamiento , Vincristina/administración & dosificación , Vincristina/efectos adversosRESUMEN
We report on a case of severe renal failure in a 61-year-old female with multiple myeloma (MM). Two months prior to admission, the patient was diagnosed to have anemia and progressive renal failure associated with urinary Bence Jones protein and was referred to our hospital. A bone marrow biopsy revealed 40% plasma cells with κ light chain restriction. Thus, she was considered to have MM. A renal biopsy revealed neoplastic plasma cell infiltration within the kidney, moderate interstitial fibrosis, tubular atrophy, and punctate, electron-dense material along the peripheral capillary walls, tubular basement membrane, and in the interstitium of the kidney. This suggested that a combination of compression of the tubules and the microvasculature by the infiltrative process, and local light chain deposition-mediated tissue damage might be implicated in the development of renal failure in this patient. Despite a remission of bone marrow plasmacytosis with a bortezomib-based regimen, her renal function gradually deteriorated and a periodic hemodialysis program was finally required. Although the clinical impact of the direct kidney infiltration of neoplastic plasma cells on the longitudinal changes in renal function remains to be delineated, it is reasonable to consider that the infiltration of neoplastic plasma cells associated with local light chain depositions may result in irreversible renal injuries. Obviously, further studies and accumulation of additional experience with renal biopsy are required to better determine the precise and prognostic relationship between renal outcome and morphological alterations among MM patients with varying degrees of renal impairment.
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Riñón/patología , Mieloma Múltiple/complicaciones , Células Plasmáticas/patología , Insuficiencia Renal/etiología , Proteína de Bence Jones/orina , Ácidos Borónicos/uso terapéutico , Bortezomib , Femenino , Humanos , Persona de Mediana Edad , Mieloma Múltiple/tratamiento farmacológico , Mieloma Múltiple/patología , Pirazinas/uso terapéutico , Insuficiencia Renal/patologíaRESUMEN
Deferasirox (DFX) is a newly developed oral iron chelator that enables effective chelation with once daily administration. We describe here a case of transfusional-iron overloaded patient who experienced hematopoietic recovery after DFX administration. A 75-year-old woman with iron overload, who had been diagnosed with MDS (RCMD) and had received a transfusion of red blood cells and platelets regularly for 3 years, enrolled in the phase I clinical trial of ICL670 (DFX) in Japan. DFX administration steadily decreased her serum ferritin levels and chelated overloaded iron effectively. Interestingly, a year after initiation of the trial, she needed fewer blood transfusions, and no more transfusions after the 17th month of the trial. Even after suspending transfusions, her hemoglobin level and platelet count increased continuously, and she now has stable disease without blood transfusions. She has not received any specific treatment for MDS during this period. Examination of the bone marrow aspirates in the 35th month revealed dysplastic cells, indicating no remarkable change in the state of MDS. This case suggests that excess iron hampers hematopoiesis and that adequate iron chelation may improve hematological data in some iron-overloaded patients.
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Benzoatos/administración & dosificación , Transfusión de Eritrocitos/efectos adversos , Hematopoyesis , Quelantes del Hierro/administración & dosificación , Sobrecarga de Hierro/tratamiento farmacológico , Sobrecarga de Hierro/etiología , Síndromes Mielodisplásicos/terapia , Transfusión de Plaquetas/efectos adversos , Triazoles/administración & dosificación , Anciano , Deferasirox , Femenino , Humanos , Síndromes Mielodisplásicos/sangre , Resultado del TratamientoRESUMEN
D-Allose, the C-3 epimer of D-glucose, is one of the rare sugars found in nature. In the present study, we have elucidated for the first time that various leukemia cell lines have different susceptibility to anti-proliferative activity of D-allose, and that this difference is related to the difference in induction of thioredoxin interacting protein (TXNIP) expression. We examined 5 leukemia cell lines (MOLT-4F, IM-9, HL-60, BALL-1 and Daudi), and found that MOLT-4F (T-cell lymphoblastic leukemia) had the highest susceptibility to D-allose, and that Daudi (Burkitt's lymphoma) had the lowest. D-Allose significantly slowed the cell cycle progression without causing apoptosis of MOLT-4F cells. Intracellular TXNIP expression was specifically and markedly enhanced in MOLT-4F cells by D-allose treatment, and subsequent increase of p27(kip1), a cell cycle inhibitor, was observed. On the other hand, D-allose did not increase TXNIP and p27(kip1) levels at all in Daudi cells. These results indicate that D-allose suppresses MOLT-4F cell proliferation possibly by the inhibition of cell cycle progression via induction of TXNIP expression.
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Proteínas de Ciclo Celular/metabolismo , Ciclo Celular/efectos de los fármacos , Glucosa/administración & dosificación , Leucemia/metabolismo , Leucemia/patología , Línea Celular Tumoral , Proliferación Celular/efectos de los fármacos , Relación Dosis-Respuesta a Droga , HumanosRESUMEN
Acute myeloblastic leukemia cases carrying the translocation t(8;16) (p11;p13) are characterized by the M4 and M5 subtypes, erythrophagocytosis by the blast cells and a poor prognosis, suggesting a new clinical entity. The t(8;16) fuses the MOZ gene which encodes a histone acetyltransferase, located on 8p11 with the CBP gene which also encodes a histone acetyltransferase, located on 16p13, and recent reports suggested that the chimeric transcription MOZ-CBP is essential for leukemogenesis. A 68-year-old woman who had been treated mainly with paclitaxel and carboplatin for preceding ovarian cancer was admitted to our hospital, complaining of right breast mass. She was diagnosed as having breast cancer and acute monocytic leukemia (M5b). Cytogenetic study with spectral karyotyping analysis revealed the development of 47 XX, + 8, t(8;16)(p11;p13). Eleven cases of therapy-related t(8;16) leukemia including the present case have been reported, but prior treatment with paclitaxel and carboplatin-based chemotherapy has never been reported. The relation of histone acetylase and therapy-related leukemia is discussed.
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Cromosomas Humanos Par 16 , Cromosomas Humanos Par 8 , Leucemia Monocítica Aguda/genética , Neoplasias Primarias Secundarias/genética , Translocación Genética , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Carboplatino/efectos adversos , Transformación Celular Neoplásica/genética , Femenino , Humanos , Leucemia Monocítica Aguda/inducido químicamente , Neoplasias Primarias Secundarias/inducido químicamente , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/tratamiento farmacológico , Paclitaxel/efectos adversosRESUMEN
Osteolysis and hypercalcemia are observed in 5-15%, and 10%, respectively, of malignant lymphoma patients during their clinical course. However, both osteolysis and hypercalcemia are uncommon at onset of the disease. We encountered a 24-year-old male non-Hodgkin's lymphoma patient who had multiple osteolytic lesion from the onset of the disease and repeated episodes of hypercalcemia during the clinical course. The patient died with refractory disease. We studied the expression of chemokines which might affect bone resorption using the reverse transcriptase-polymerase chain reaction (RT-PCR) method. Increased expressions of MIP-1alpha, MIP-1beta and RANKL, which are osteoclast-activating factors, were observed in the RNA derived from the patient's lymphoma cells. The secretion of osteoclast-activating factors such as MIP-1alpha by the tumor cells (and/or bone marrow stromal cells) might be involved in the etiology of osteolysis and hypercalcemia in some malignant lymphoma cases.
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Hipercalcemia/diagnóstico , Linfoma de Células B/diagnóstico , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma no Hodgkin/diagnóstico , Proteínas Inflamatorias de Macrófagos/biosíntesis , Osteólisis/diagnóstico , Fosfatasa Ácida/metabolismo , Adulto , Biopsia , Células de la Médula Ósea/citología , Resorción Ósea , Proteínas Portadoras/biosíntesis , Quimiocina CCL3 , Quimiocina CCL4 , Quimiocinas/biosíntesis , Resultado Fatal , Humanos , Inmunofenotipificación , Isoenzimas/metabolismo , Dolor de la Región Lumbar , Linfoma de Células B/patología , Linfoma de Células B Grandes Difuso/patología , Linfoma no Hodgkin/patología , Imagen por Resonancia Magnética , Masculino , Glicoproteínas de Membrana/biosíntesis , Osteoclastos/metabolismo , Ligando RANK , Receptor Activador del Factor Nuclear kappa-B , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Fosfatasa Ácida TartratorresistenteRESUMEN
Methylation profile was analyzed in eleven cases of therapy-related leukemia (t-leukemia) for p14, p15, p16, Rb, hMLH1, hMSH2, MGMT, APC, RAR beta, DAPK, RIZ1, FHIT, and SOCS-1 genes by using methylation specific polymerase chain reaction (MSP) analysis. Six (55%) of eleven cases showed methylation of at least one gene. The average time to the development of t-leukemia after the treatment of the primary tumor was significantly shorter in patients with methylation than those without methylation (49.3 months vs. 133.2 months, P=0.044). These results suggest that hypermethylation might be involved in the development of t-leukemia.
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Islas de CpG , Metilación de ADN , Leucemia/tratamiento farmacológico , Leucemia/genética , Leucemia/radioterapia , Regiones Promotoras Genéticas , Anciano , Anciano de 80 o más Años , Antineoplásicos/efectos adversos , Células de la Médula Ósea/metabolismo , Reparación del ADN , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Reacción en Cadena de la Polimerasa , Radioterapia/efectos adversosRESUMEN
Th2 dominancy in the peripheral T helper (Th) cell subsets were reported to be involved in the pathogenesis of pure red cell aplasia (PRCA). We encountered a PRCA case secondary to plasma cell dyscrasia that showed Th2 dominancy at the relapse of PRCA. Increased expression of c-maf, a transcriptional factor which induces Th2 differentiation of naive T-cells, and elevated expression of interleukin (IL)-4 were observed in the RNA derived from patient's bone marrow at relapse of PRCA. Following the administration of methylprednisolone which improved PRCA, normalization of Th1/Th2 ratio and decreased expression of c-maf and IL-4 were observed, which suggests that the upregulation of c-maf might have played a role in the pathogenesis of PRCA secondary to plasma cell dyscrasia.
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Proteínas de Unión al ADN/biosíntesis , Regulación de la Expresión Génica , Paraproteinemias/complicaciones , Proteínas Proto-Oncogénicas/biosíntesis , Aplasia Pura de Células Rojas/metabolismo , Anciano , Médula Ósea/metabolismo , Diferenciación Celular , Proteínas de Unión al ADN/genética , Proteínas de Unión al ADN/fisiología , Humanos , Cadenas kappa de Inmunoglobulina/análisis , Cadenas lambda de Inmunoglobulina/análisis , Inmunosupresores/uso terapéutico , Interferón gamma/biosíntesis , Interferón gamma/genética , Interleucina-4/biosíntesis , Interleucina-4/genética , Interleucinas/biosíntesis , Interleucinas/genética , Masculino , Metilprednisolona/uso terapéutico , Paraproteinemias/metabolismo , Paraproteínas/análisis , Proteínas Proto-Oncogénicas/genética , Proteínas Proto-Oncogénicas/fisiología , Proteínas Proto-Oncogénicas c-maf , ARN Mensajero/biosíntesis , ARN Mensajero/genética , Recurrencia , Aplasia Pura de Células Rojas/tratamiento farmacológico , Aplasia Pura de Células Rojas/etiología , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Células Th2/inmunología , Trombocitosis/etiologíaRESUMEN
Myelofibrosis following peripheral T-cell lymphoma has rarely been reported. Described here is a case of peripheral T-cell lymphoma with myelofibrosis and elevated transforming growth factor beta (TGF-beta). A 69 years old male was admitted due to anemia and thrombocytopenia. His bone marrow showed fibrosis and was infiltrated with small lymphoid cells and a few residual normal hematopoietic cells. He had presented with hepatosplenomegaly and left inguinal lymph node swelling. Biopsy of the left inguinal lymph node revealed diffuse mature small lymphoid cells with atypical nuclei. Immunophenotyping of the small lymphoid cells were positive for CD3, CD8, TCR alphabeta and HLA-DR and were negative for CD4, CD19, CD20 and CD56. T-cell receptor beta-chain gene was rearranged in bone marrow cells. He was diagnosed as having peripheral T-cell lymphoma complicated with myelofibrosis. Chemotherapy was administrated which improved his pancytopenia and symptoms. Two years later, anemia and thrombocytopenia developed rather quickly, he died because of progression of myelofibrosis with severe pancytopenia.
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Linfoma de Células T Periférico/complicaciones , Linfoma de Células T Periférico/diagnóstico , Mielofibrosis Primaria/etiología , Anciano , Anemia/etiología , Resultado Fatal , Reordenamiento Génico de la Cadena beta de los Receptores de Antígenos de los Linfocitos T , Humanos , Inmunofenotipificación , Linfoma de Células T Periférico/tratamiento farmacológico , Masculino , Trombocitopenia/etiología , Factor de Crecimiento Transformador beta/sangreRESUMEN
The development of central pontine myelinolysis (CPM) has rarely been reported in association with hemophagocytic syndromes (HPS). Here we report a unique case of Epstein-Barr Virus (EBV)-related HPS which was accompanied with CPM. A 72-year-old man who had no significant medical history was admitted to our hospital due to high fever and progressing dysphasia and dysarthria. Physical examination revealed anisocoria of the right pupil, fixed reaction to light, and paralysis of the left vagus nerves. Magnetic resonance imaging revealed low signal intensity on T1-weighted images and high signal intensity T2-weighted images in the patient's central midpontine lesion. Initial work-up showed anemia and thrombocytopenia with elevated levels of serum ferritin, lactate dehydrogenase, and soluble IL-2 receptor. Bone marrow aspiration revealed hemophagocytosis. The EBV genome was detected in the peripheral blood using the polymerase chain reaction method. He was diagnosed as having EBV-related HPS and CPM. Despite intensive treatment with methylpredonisolone, immunoglobulin, and etoposide, he died due to progressive disease and fungal septicemia. The etiology and relation between CPM and HPS are discussed.
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Infecciones por Virus de Epstein-Barr/complicaciones , Histiocitosis de Células no Langerhans/complicaciones , Mielinólisis Pontino Central/complicaciones , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Examen de la Médula Ósea , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/tratamiento farmacológico , Resultado Fatal , Histiocitosis de Células no Langerhans/diagnóstico , Histiocitosis de Células no Langerhans/tratamiento farmacológico , Humanos , Imagen por Resonancia Magnética , Masculino , Mielinólisis Pontino Central/diagnóstico , Mielinólisis Pontino Central/tratamiento farmacológico , Mielinólisis Pontino Central/etiologíaRESUMEN
Thrombocytosis is a rare finding in acute myeloblastic leukemia (AML). Here, we describe a patient with AML who relapsed with marked thrombocytosis. The patient was initially diagnosed as having AML (M4) with a low platelet count. The patient was started on combination chemotherapy including high-dose etoposide and achieved complete remission. However, the patient relapsed six months later with an extremely high platelet count (72.5 x 10(4)/microl). Cytogenetic analysis at relapse revealed the development of t(2;14)(p13;q32). Despite the repeated combination chemotherapy, the patient died with progressive disease. This case suggests that the additional chromosomal aberration t(2;14)(p13;q32) may be related to abnormal thrombocytosis in AML.
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Cromosomas Humanos Par 14 , Cromosomas Humanos Par 2 , Leucemia Mielomonocítica Aguda/complicaciones , Trombocitosis/genética , Translocación Genética , Adulto , Análisis Citogenético , Progresión de la Enfermedad , Resultado Fatal , Humanos , Leucemia Mielomonocítica Aguda/diagnóstico , Leucemia Mielomonocítica Aguda/genética , Masculino , Recuento de Plaquetas , Recurrencia , Trombocitosis/etiologíaAsunto(s)
Cromosomas Humanos Par 22 , Cromosomas Humanos Par 8 , Leucemia Monocítica Aguda/genética , Neoplasias Primarias Secundarias/genética , Translocación Genética , Acetiltransferasas/genética , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Histona Acetiltransferasas , Humanos , Leucemia Monocítica Aguda/inducido químicamente , Leucemia Monocítica Aguda/etiología , Masculino , Persona de Mediana Edad , Neoplasias Primarias Secundarias/inducido químicamente , Neoplasias Primarias Secundarias/etiología , Proteínas Nucleares/genética , Transactivadores/genética , Macroglobulinemia de Waldenström/tratamiento farmacológicoRESUMEN
A 50-year-old woman with a 4-year history of Evans syndrome was admitted to our hospital because of progressive nausea, appetite loss, body weight loss, diarrhea and abdominal pain. Abdominal ultrasonography revealed pleural effusion, ascites, bilateral hydronephrosis, dilatation of the bilateral ureter, and irregular wall thickness of the urinary bladder. Immunological studies revealed decreased complement components (C3; 72 mg/dl, C4; 7 mg/dl, CH50; 28.8 mg/dl), a x 80 antinuclear antibody titer (homogeneous pattern), antibody against single-stranded DNA 19 U/ml, anti-SS-A antibody over 500 U/ml and negativity for antibody against double-stranded DNA (anti-dsDNA Ab). Although the patient did not fulfill the criteria for systemic lupus erythematosus (SLE), we diagnosed her as having lupus cystitis. Bolus methylprednisolone (mPSL) therapy (1,000 mg mPSL over 3 days, div) was administered, followed by 60 mg PSL, and this led to immediate improvement of the patient's symptoms and laboratory data. Later, anti-dsDNA Ab became positive, and the patient thereby fulfilled the criteria for SLE. Lupus cystitis following Evans syndrome has rarely been reported. The present such case was treated successfully with bolus mPSL therapy.
Asunto(s)
Anemia Hemolítica Autoinmune/complicaciones , Cistitis Intersticial/etiología , Lupus Eritematoso Sistémico/etiología , Púrpura Trombocitopénica/complicaciones , Femenino , Humanos , Persona de Mediana Edad , SíndromeRESUMEN
Interferon-alpha (IFN-alpha) has been accepted as an effective agent in the treatment of chronic myelogenous leukemias (CML). Cardiac toxicity of IFN-alpha has rarely been reported in cases of CML. A 62-year-old woman with a two-year history of chronic myelogenous leukemia who had been treated with IFN-alpha (10 million U/3 days/week) given subcutaneously with oral hydroxycarbamide 500 mg, presented with chest pain, dyspnea and subconsciousness. Chest X-ray revealed cardiomegaly and congestion, and ultrasonography showed diffuse hypokinesis of the heart with decreased left ventricular ejection fraction (LVEF) 34%. She was diagnosed cardiomyopathy caused by IFN-alpha administration. She was treated with furosemide, dobutamine hydrochloride, milrinone and carperitide. The administration of IFN-alpha was terminated. LVEF was improved to 50% within one month from the onset of events, and the patient was discharged. We discuss herein the cardiomyopathy caused by IFN-alpha in CML.