RESUMO
Background: Myelodysplastic neoplasms (MDS) are characterized by cytopenia, morphologic dysplasia, and genetic abnormalities. Multiparameter flow cytometry (FCM) is recommended in the diagnostic work-up of suspected MDS, but alone is not sufficient to establish the diagnosis. Our aim was to investigate the diagnostic power of FCM in a heterogeneous population of patients with cytopenia, excluding cases with increased blast count. Methods: We analyzed bone marrow samples from 179 patients with cytopenia (58 MDS, 121 non-MDS) using a standardized 8-color FCM method. We evaluated the sensitivity, specificity, and accuracy of several simple diagnostic approaches, including Ogata score, extended Ogata score, the WHO and ELN iMDSFlow recommended "3 aberrations in two cell compartments method," and the combination of the Ogata score and "3 aberrations in two cell compartments method." The patients were followed until the diagnosis was confirmed, with a median follow-up of 2 months (range 0.2-27). Results: The combination of Ogata score and "3 aberrations in two cell compartments method" achieved the highest diagnostic accuracy (78%) with sensitivity and specificity 61% and 86%, respectively. When using only the "3 aberrations in two cell compartments method," the accuracy was 77% with a sensitivity of 72% and a specificity of 79%. The most frequently observed etiologies among the false positive cases were substrate deficiencies, inflammation/infection, or toxic effects. MDS can be excluded in all these cases after a thorough clinical evaluation and a relatively short follow-up. Conclusion: FCM remains an important but supplementary part in an integrated diagnostic process of MDS with low blasts. The combination of the Ogata score and the "3 aberrations in two cell compartments method" slightly improves accuracy compared to the detection of "3 aberrations in two cell compartments method" alone.
Assuntos
Citometria de Fluxo , Síndromes Mielodisplásicas , Humanos , Citometria de Fluxo/métodos , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/patologia , Masculino , Feminino , Idoso , Pessoa de Meia-Idade , Diagnóstico Diferencial , Idoso de 80 Anos ou mais , Adulto , Adulto Jovem , Medula Óssea/patologia , Sensibilidade e Especificidade , Seguimentos , CitopeniaRESUMO
INTRODUCTION: Minimal residual disease is associated with longer overall survival in patients with chronic lymphocytic leukemia. AIM: The aim of the authors was to determine the clinical significance of remission and minimal residual disease on the survival of patients with chronic lymphocytic leukemia. METHODS: Data from 42 first-line treated patients with chronic lymphocytic leukemia were analyzed. Minimal residual disease was determined by flow cytometry. RESULTS: Overall response and complete remission was achieved in 91%, 86%, 100% and 87%, 0%, 60% of patients with fludarabine-based combinations, single-agent fludarabine and cyclophosphamide + vincristin + prednisolone regimen, respectively. Minimal residual disease eradication was feasible only with fludarabine-based combinations in 60% of these cases. The ratio of minimal residual disease was 0.5% on average. During a median follow-up period lasting 30 months, the overall survival of patients with fludarabine-resistant disease proved to be significantly shorter (p = 0.04), while complete remission without minimal residual disease was associated with significantly longer progression free survival (p = 0.02). CONCLUSION: Only fludarabine-based combinations were able to eradicate minimal residual disease in patients with chronic lymphocytic leukemia. Complete remission without minimal residual disease may predict longer progression free survival in these patients.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Neoplasia Residual/diagnóstico , Neoplasia Residual/tratamento farmacológico , Vidarabina/análogos & derivados , Adulto , Idoso , Ciclofosfamida/administração & dosagem , Intervalo Livre de Doença , Feminino , Citometria de Fluxo , Hospitais de Condado/estatística & dados numéricos , Humanos , Hungria/epidemiologia , Leucemia Linfocítica Crônica de Células B/epidemiologia , Masculino , Pessoa de Meia-Idade , Neoplasia Residual/epidemiologia , Prednisolona/administração & dosagem , Indução de Remissão , Resultado do Tratamento , Vidarabina/administração & dosagem , Vincristina/administração & dosagemRESUMO
The 27-year-old pregnant woman has been overweight since her childhood. Endocrinological assessments did not confirm hormonal disease. Her pregnancy was without complication. A signs of intrauterine distress were observed and elective caesarean section was performed under heparin protection because of anatomy unsuitable for delivery per vias naturals. The mother's bodyweight was 184 kg. By monitoring the change in fX activity LMWH treatment (Enoxaparin) initiated with a dose of 120 mg twice daily and then the dose was gradually elevated to 200 mg twice daily thereby achieving the lower range of the desired therapeutic effect. Apart from mild disorder of wound healing, the recovery was free of complication. The patient suffered from thrombophilia (extremely overweight, pregnant, thrombophlebitis under the knee, surgery, and postoperative immobilization). In case of quite extreme bodyweight there is no dosage recommendation or clinical practice for LMWH. Because of the extreme overweight and the therapeutic dose titration test of heparin, monitoring of fX activity by measurement of inhibition, dosage of heparin other than the recommended (abdominal wall instead of upper arm SC), and the very fluctuating heparin dosage which is well correlating with clinical practice, it is reasonably expected that this case will take interest.