RESUMEN
Autoimmune manifestations are a common occurrence with chronic lymphocytic leukaemia (CLL). We describe a case of CLL-associated immune thrombocytopenia (ITP) that had a loss of response to standard treatment for ITP. The thrombopoeitin receptor agonist, eltrombopag, was successfully used preoperatively to increase the platelet count to a safer level, in this instance to facilitate laparoscopic splenectomy.
Asunto(s)
Benzoatos/uso terapéutico , Hidrazinas/uso terapéutico , Leucemia Linfocítica Crónica de Células B/complicaciones , Leucemia Linfocítica Crónica de Células B/tratamiento farmacológico , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Púrpura Trombocitopénica Idiopática/etiología , Pirazoles/uso terapéutico , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Recent developments have led to remarkable improvements in the assessment and treatment of patients with multiple myeloma (MM). New technologies have become available to precisely evaluate the biology and extent of the disease, including information about cytogenetics and genetic abnormalities, extramedullary manifestations and minimal residual disease. New, more effective drugs have been introduced into clinical practice, which enable clinicians to significantly improve the outcome of patients but also pose new challenges for the prevention and management of their specific side effects. Given these various new options and challenges, it is important to identify the minimal requirements for diagnosis and treatment of patients, as access to the most sophisticated advances may vary depending on local circumstances. Here, we propose the minimal requirements and possible options for diagnosis, monitoring and treatment of patients with multiple myeloma.
Asunto(s)
Mieloma Múltiple/terapia , Humanos , Monitoreo Fisiológico , Mieloma Múltiple/fisiopatología , Recurrencia , Resultado del TratamientoRESUMEN
Multiple myeloma rarely presents with a fever of unknown origin and diagnosis may be delayed. We describe a case of myeloma presenting in this way with raised serum-free light chains and TP53 deletion on cytogenetics. The patient developed thrombotic thrombocytopenia purpura (TTP) following bortezomib therapy but recovered spontaneously and was successfully re-challenged. We believe this is only the second case to describe this phenomenon post-bortezomib and the first to rechallenge the patient successfully without further recurrence of TTP. Possible mechanisms for this successful rechallenge are discussed.
Asunto(s)
Ácidos Borónicos/efectos adversos , Fiebre de Origen Desconocido/inducido químicamente , Fiebre de Origen Desconocido/diagnóstico , Mieloma Múltiple/diagnóstico , Púrpura Trombocitopénica Trombótica/inducido químicamente , Púrpura Trombocitopénica Trombótica/diagnóstico , Pirazinas/efectos adversos , Proteínas ADAM/sangre , Proteína ADAMTS13 , Bortezomib , Femenino , Fiebre de Origen Desconocido/sangre , Humanos , Persona de Mediana Edad , Mieloma Múltiple/tratamiento farmacológico , Púrpura Trombocitopénica Trombótica/sangreRESUMEN
Treating systemic malignancies requires specialist skill and experience, with active treatment often providing the best initial palliation of symptoms. A full management plan involving general practitioner, patient and others is essential at this stage of HIV disease.
Asunto(s)
Neoplasias Encefálicas/etiología , Infecciones por VIH/complicaciones , Linfoma Relacionado con SIDA , Linfoma no Hodgkin/etiología , Displasia del Cuello del Útero/etiología , Neoplasias del Cuello Uterino/etiología , Neoplasias Encefálicas/terapia , Femenino , Infecciones por VIH/terapia , Enfermedad de Hodgkin/etiología , Enfermedad de Hodgkin/terapia , Humanos , Linfoma Relacionado con SIDA/terapia , Linfoma no Hodgkin/terapia , Masculino , Cuidados Paliativos , Pronóstico , Factores de Riesgo , Neoplasias del Cuello Uterino/terapia , Displasia del Cuello del Útero/terapiaAsunto(s)
Neoplasias del Yeyuno/inmunología , Leucemia Linfocítica Crónica de Células B/inmunología , Paraproteinemias/inmunología , Anciano , Electroforesis de las Proteínas Sanguíneas , Femenino , Humanos , Inmunoglobulinas/sangre , Neoplasias del Yeyuno/complicaciones , Leucemia Linfocítica Crónica de Células B/complicaciones , Paraproteinemias/complicacionesRESUMEN
AIM: Genetic haemochromatosis is a common disorder resulting in increased iron deposition in the liver and other organs but can be difficult to diagnose. The aim of this study was to assess the diagnostic value of the conventional tests for iron overload (percentage saturation of transferrin, serum ferritin and grading of iron staining on liver biopsy) and compare these with the newer quantitative biochemical measurements of liver iron. METHOD: A retrospective analysis was made of 108 consecutive patients referred for quantitative liver iron measurements. Iron studies were obtained in 66 of the 108 subjects of whom 60 had abnormal screening tests defined as percent saturation of transferrin (> 60%) and/or ferritin > 350 micrograms/L for females and > 450 micrograms/L for males. Based on clinical features, biochemical data and treatment outcome these 60 subjects were classified as either genetic haemochromatosis, nongenetic haemochromatosis or indeterminate. One patient with treated genetic haemochromatosis was excluded from subsequent analysis. RESULTS: Although the serum ferritin (p < 0.002), percentage saturation of transferrin (p < 0.001), histological iron grade (p < 0.0001) were significantly higher in the genetic haemochromatosis than nongenetic haemochromatosis group there was considerable overlap. Similarly for the hepatic iron concentration (HIC) (p < 0.0001) overlap occurred. The hepatic iron index (HIC/age) gave the best separation with only three cases being misclassified. A correlation between the HII and histological iron index (visualised iron score corrected for age) in 15 subjects gave an r value of 0.72. CONCLUSION: Based on this study we feel that in addition to visual grading of iron in liver biopsies, the hepatic iron index is helpful in establishing a diagnosis of genetic haemochromatosis.
Asunto(s)
Ferritinas/sangre , Hemocromatosis/diagnóstico , Hierro/análisis , Hígado/química , Transferrina/análisis , Adulto , Anciano , Biopsia , Femenino , Hemocromatosis/sangre , Hemocromatosis/genética , Humanos , Hígado/patología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Sensibilidad y EspecificidadRESUMEN
AIM: To determine the prevalence of antibodies to hepatitis C (anti-HCV) in patients who have undergone bone marrow transplantation in Wellington, prior to the introduction of hepatitis C screening, and to contrast these results with the prevalence of anti-HCV in the Wellington haemophiliac population. METHOD: Serum specimens were obtained from 30 patients who had undergone bone marrow transplantation for the treatment of haematological disorders, and from 29 haemophiliacs. Specimens were analysed using a second generation HCV immunoassay. RESULTS: Exposure to blood products was high in bone marrow transplant recipients with subjects receiving red cells or platelets from an average of 53 donors (range 15-100, SD 23.2) during their transplant procedure. Despite the high usage of blood products, only one of the 30 patients tested was positive for hepatitis C on the basis of second-generation antibody testing. Confirmatory testing in this patient, (anti-HCV immunoblot assay) was negative. In contrast, 26 of 29 (89%) haemophiliac patients tested were positive for anti-HCV. CONCLUSION: Although the infective risk of blood products cannot be underestimated, the risk of patients contracting hepatitis C from multiple single-unit transfusions, prior to the introduction of screening for hepatitis C was low. This contrasts with the high risk of hepatitis C seroconversion in patients exposed to pooled plasma products.
Asunto(s)
Bancos de Sangre/normas , Trasplante de Médula Ósea , Hemofilia A/terapia , Hepatitis C/epidemiología , Reacción a la Transfusión , Adolescente , Adulto , Transfusión Sanguínea/estadística & datos numéricos , Niño , Preescolar , Femenino , Hepacivirus/inmunología , Anticuerpos Antihepatitis/análisis , Hepatitis C/etiología , Hepatitis C/inmunología , Anticuerpos contra la Hepatitis C , Humanos , Masculino , Persona de Mediana Edad , Nueva Zelanda/epidemiología , Prevalencia , Estudios SeroepidemiológicosAsunto(s)
Hidroxiurea/efectos adversos , Linfoma no Hodgkin/inducido químicamente , Policitemia Vera/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biopsia , Médula Ósea/patología , Humanos , Hidroxiurea/uso terapéutico , Hígado/patología , Linfoma no Hodgkin/tratamiento farmacológico , Linfoma no Hodgkin/patología , Masculino , Persona de Mediana Edad , Policitemia Vera/diagnósticoAsunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ascitis/patología , Células Plasmáticas/patología , Anciano , Ascitis/tratamiento farmacológico , Ascitis/etiología , Femenino , Humanos , Neoplasias Intestinales/patología , Neoplasias Intestinales/cirugía , Complicaciones PosoperatoriasRESUMEN
Chromosome in situ hybridization studies showed that the normal karyotype of leukemic cells from a patient with Ph1-negative, BCR-positive chronic myeloid leukemia (CML) concealed a complex t(9;22;20)(q34;q11;p13). The close association of 5'-BCR and 3'-ABL was demonstrated by field inversion gel electrophoresis, and in situ hybridization showed that BCR-ABL was located on the short arm of chromosome 20. Our findings further indicate that chromosome rearrangement is the cause of BCR-ABL gene fusion in leukemic cells that show a normal karyotype. Results from in situ hybridization studies were consistent with formation of the t(9;22;20) by a two step chromosomal rearrangement, but field inversion gel electrophoresis results indicated a more complex rearrangement.
Asunto(s)
Cromosomas Humanos Par 20/ultraestructura , Cromosomas Humanos Par 22/ultraestructura , Cromosomas Humanos Par 9/ultraestructura , Proteínas de Fusión bcr-abl/genética , Reordenamiento Génico , Genes abl , Leucemia Mieloide Crónica Atípica BCR-ABL Negativa/genética , Translocación Genética , Anciano , Biomarcadores de Tumor , ADN de Neoplasias/análisis , Femenino , Marcadores Genéticos , Humanos , Cariotipificación , Leucemia Mieloide Crónica Atípica BCR-ABL Negativa/patología , Hibridación de Ácido Nucleico , Mapeo RestrictivoRESUMEN
Eleven patients with progressive hairy cell leukaemia (three nonsplenectomised) were treated with recombinant alpha-2 interferon (Intron-A or Roferon-A) subcutaneously three times per week at a dosage of 3 x 10(6) units. Ten patients completed at least ten weeks of therapy and could be evaluated; one patient died of haemorrhage from severe thrombocytopenia after only three weeks treatment. Nine of the ten patients responded and all of these are regarded as good partial remissions (normalisation of all blood parameters but still discernible hairy cells in the marrow). Responding patients have all been followed for a median of two years and in one case 3 1/2 years since commencement of therapy. The patients are all transfusion independent and free of infection. We conclude that alpha-2 interferon therapy for progressive hairy cell leukaemia is effective therapy in both splenectomised and nonsplenectomised patients.
Asunto(s)
Interferón Tipo I/uso terapéutico , Leucemia de Células Pilosas/terapia , Adulto , Anciano , Recuento de Células Sanguíneas/efectos de los fármacos , Ensayos Clínicos como Asunto , Esquema de Medicación , Femenino , Estudios de Seguimiento , Humanos , Inyecciones Subcutáneas , Interferón Tipo I/administración & dosificación , Interferón Tipo I/efectos adversos , Leucemia de Células Pilosas/sangre , Masculino , Persona de Mediana Edad , Proteínas Recombinantes , Inducción de Remisión , Autoadministración , Factores de TiempoAsunto(s)
Anemia Aplásica/etiología , Hepatitis B/complicaciones , Adulto , Anemia Aplásica/diagnóstico , Humanos , MasculinoRESUMEN
A case of transfusion-related AIDS is described which is believed to be the first published case to occur in New Zealand in a nonhaemophiliac patient. The human immunodeficiency virus (HIV) positive donor was shown to be the source of infection in five further HIV positive recipients.