ABSTRACT
Objective@#To investigate the influence of additional clonal chromosome abnormalities in Ph negative cells (CCA/Ph-) on the efficacy of chronic myeloid leukemia (CML) after tyrosine kinase inhibitors (TKI) treatment.@*Methods@#The clinical data of 28 CML patients with CCA/Ph- treated in Henan Cancer Hospital from July 2014 to December 2017 were analyzed retrospectively. The univariate analysis was carried out by Kaplan-Meier method. Multivariate analysis was done by Cox proportional risk model.@*Results@#A total of 28 CCA/Ph-patients were recruited including 17 males and 11 females with median age of 42.5 years old. The most common CCA/Ph-were trisomy 8 (60.7%), monosomy 7 (14.3%). 64.3% CCA/Ph-were transient and 35.7% recurrent (more than 2 times). Cytopenia in two or three lineages of peripheral blood was seen in 42.9% patients. As to the efficacy, 89.3% patients achieved major cytogenetic response (MCyR), 25% with major molecular response (MMR). The median follow-up time was 26.5 months. Treatment failure (TF) of TKI occurred in 32.1% patients with median duration of response 8 (1-41) months. Univariate analysis showed that TF rate was significantly correlated with the frequency of CCA/Ph-and cytopenia (all P<0.05). The MMR rate was also significantly correlated with cytopenia (P<0.05). Cytopenia of two lineages or pancytopenia was an independent risk factor related to MMR rate (RR=3.868, 95%CI 1.216-12.298, P=0.022) .@*Conclusions@#Cytopenia in CCA/Ph-appears to be an independent risk factor of MMR in CML patients with TKI treatment. The recurrent CCA/Ph-may link to higher treatment failure rate. Drug withdrawal or alternative strategy should be considered according to response and the ABL kinase mutations.
ABSTRACT
Whether allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the optimal treatment for adult patients with Philadelphia chromosome-negative acute lymphoblastic leukemia (Ph - ALL) after the first complete remission (CR1) or not is still a controversial issue. Many studies have recently reported that adolescents and young adults with Ph- ALL may benefit from pediatric chemotherapy. Conventional baseline risk factors cannot satisfactorily predict prognosis, conversely, the early minimal residual disease (MRD) is the best stratification tool to offer different treatments for patients with different MRD status. Novel therapies, such as CAR-T cells and blinatumomab, have shown promising results in relapsed/refractory patients. Through comprehensive application of pediatric chemotherapy protocols, MRD driven strategy and novel therapies, it is hopeful to change the paradigm of how to treat adults with Ph - ALL in the future, and to bring improved outcomes and decreased adverse events.
ABSTRACT
The knowledge and understanding of myeloproliferative neoplasms (MPN) over the last hundred years has been reviewed in this article,focusing on clinical practice.The identification of JAK2 V617F gene mutation leads Philadelphia chromosome-negative (Ph) MPN into a new era of molecular biology.These advances not only provide a reliable diagnostic tool and important evidence for diagnosis of MPN,also induce a lot of investigation and manufacture of targeting drugs to JAK2 mutation.However,JAK2 V617F mutation is not the gold standard for the diagnosis of MPN,as unique as bcr-abl in CML.Certain routine lab results and differentiation with some other diseases are still necessary.A JAK1/JAK2 inhibitor,ruxolitinib,has been approved for clinical use,but indication should be followed.Further follow-up is needed to assess the longterm outcomes with respect to efficacy and safety.It is not time to give up conventional medicine,such as hydroxyurea or aspirin.
ABSTRACT
Objective To retrospectively analyze the efficacy of a pediatric treatment protocol,BFM90,in adult patients with acute lymphobiastic leukemia (ALL) up to the age of 60 years. Methods From August 2004 to October 2007,60 adult patients (median age,40 years; range,18 to 60 years) with Philadelphia chromosome-negative ALL were treated with the BFM90 protocol. Clinical effect were historically compared with that of the 35 patients (median age,42 years; range,18 to 56 years) who were treated with Hyper-CVAD protocol. Results At 42 months,complete remission (CR) rate,event-free survival (EFS) and overall survival (OS) rates were 93 % (56 patients),60 % (36 patients) and 65 % (39 patients),respectively.Age is an important prognostic factor,with 45 years of age as best cutoff. CR (P=0.02),OS (P ＜0.001),and EFS (P ＜0.001) of BFM90 were compared superiorly with that of the previous Hyper-CVAD experience.Conclusion These results suggest that pediatric protocol superior to the outcome of adult patients with Philadelphia chromosome-negative ALL.
ABSTRACT
BACKGROUND: Philadephia chromosome negative chronic myeloproliferative disease (CMPD) is a clonal disorder which includes polycythemia vera (PV), essential thrombocythemia (ET) and idiopathic myelofibrosis (IMF). CMPD has chronic course and different clinical features with low rate of conversion to leukemia. We evaluated the clinical features of CMPD. METHODS: Since 1990, 57 cases of CMPD (18 PV, 35 ET and 4 IMF) were analysed and their clinical characteristics, survival and manner of evolution were evaluated retrospectively. RESULTS: Median age of 57 CMPD patients was 61 (range, 14~90) years and male to female ratio was 1:0.8. Most common clinical manifestations were dizziness/weakness (38.6%), headache (21.2%), cardiovascular events (19.3%) and other symptoms. Treatment with hydroxyurea was most frequent during clinical course of CMPD. Anagrelide was introduced in 12 patients recently. Complication of disease itself and treatment was not frequent except bleeding (3 cases) and thrombotic event (10 cases). Conversion to acute lekemia was none. Ten year overall survival was 83.3% in PV, 60.1% in ET and 4 cases of IMF were all alive at the 6 year follow up. CONCLUSION: CMPD is a chronic disease and long term control is much improved but definitive treatment without complication should be further investigated.