Myeloproliferative neoplasms (MPNs) are classically divided into BCR RhoGEF and GTPase activating protein (BCR)-ABL proto‑oncogene 1 non‑receptor tyrosine kinase (ABL) positive chronic myeloid leukemia (CML) and BCR‑ABL negative MPNs, including essential thrombocythemia (ET). One of the major diagnostic criteria for ET is the absence of the philadelphia chromosome, thus when present it is almost indicative of CML. ET and CML are considered to be mutually exclusive; however, there are rare situations in which patients with ET present positive BCR‑ABL without the features of CML. Although from the literature review, the frequency of JAK2V617F mutation and BCR‑ABL translocation coexistence in MPNs is low, it may be higher than expected. The current study reported cases of two patients with an initial diagnosis of ET in the presence of JAK2V617F mutation and BCR‑ABL translocation by fluorescent in situ hybridization. Both patients presented with a heterozygous BCR‑ABL translocation, and absence of p190 and p210 transcripts, seemingly a der(9) in the background of an ET JAK2V617F mutation.
Early-stage chronic lymphocytic leukemia (CLL) with neurologic involvement is a rare condition and should require a careful follow-up. Although no standard protocol exists for this condition, intrathecal chemotherapy, combined with systemic chemoimmunotherapy, has been used previously. This case describes the treatment of a patient with CLL and symptomatic compromise of the central nervous system. Our results suggest that a combination of chemotherapy, radiotherapy, and ibrutinib, administered sequentially over a 2-year period, led to a near-complete resolution of the cerebral spinal fluid neoplastic infiltration. Importantly, this response has been maintained with ibrutinib monotherapy for more than 12 months.
the onset and progression of CLL. MYD88 mutations independently associate with shorter TTT in M-CLL subgroup, identifying cases with rapid progression. Introduction: Chronic lymphocytic leukemia (CLL) is a chronic lymphoproliferative disease characterized by highly biological heterogeneity and variable clinical course. Previous researches showed that 30%-40% of patients with CLL could survive for decades of years without initial need for treatment, while some patients rapidly succumb to the progression of disease. In recent years, data on prognostic value of molecular mutations and viral infections on CLL continue accumulating. Our study was determined to define variables correlated with time-to-treatment (TTT) in Chinese with chronic lymphocytic leukemia (CLL) and use these variables to develop a prognostic score. We determined correlation of the prognostic score with survival and compared this score with those developed for persons of predominately European descent with CLL in this study.
Methods:We collected clinical, molecular, serologic and virological parameters of 334 newly diagnosed and untreated CLL patients. Utilizing Chi-square test, survival analysis, log-rank test and Cox hazard regression analysis, we checked the correlations between variables and prognosis of our patients.Results: By analyzing 334 newly diagnosed and untreated CLL patients without treatment indication, we demonstrated that Binet stage B/C, lymphocyte level, TP53 abnormality, IGHV non-mutation and evidence of HBV and EBV infection were independently associated with TTT in multivariate analyses. We used these data to construct a prognostic scoring system that divided subjects into three cohorts of low, intermediate and high risks with median TTTs of 102 months (95% confidence interval 50-154 months), 15 months (5-25 months) and 6 months (3-9 months; p-value for trend <0.001). Corresponding median OS from diagnosis were not reached, not reached and 73 months (55-91 months; p-value for trend <0.001).Conclusions: We improved current risk stratification for patients with untreated CLL using combined clinical, molecular and virological variables and defined three different risk groups with this novel stratifica-
PORTUGUESE REAL-LIFE EXPERIENCE WITH IBRUTINIB OUTSIDE CLINICAL TRIALS -A MULTICENTER ANALYSIS
We report a case of bisoprolol-induced thrombocytopenia which resolved with drug discontinuation and steroid therapy. We review the mechanisms involved in drug-induced immune thrombocytopenia.
Background: Background:Primary central nervous system lymphoma (PCNSL) is a rare form of lymphoma confined to the central nervous system (CNS) that is characterized by an aggressive course, high rates of treatment resistance and short overall survival.
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