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    • Delays in diagnosis due to

    2019-04-30

    Delays in diagnosis due to the subtlety and focal nature of the intravascular infiltrates often lead to a terminal disease with death before chemotherapy is initiated. R-CHOP plus CNS prophylaxis is the treatment of choice in patients [30,31] without glycyrrhetinic acid involvement. CNS involvement and recurrence is still challenging despite the improvement in clinical outcomes induced by Rituximab plus chemotherapy. CNS prophylaxis is strongly recommended because CNS recurrence at 3 years is still as high as 25% [32,33]. The fact that several patients have CNS involvement at diagnosis or relapse supports the recommendation to use chemotherapy combinations containing drugs with high CNS bioavailability such as high dose Methrotrexate and high dose Cytarabine in these patients. Even though high-dose chemotherapy supported by autologous stem cell support (HDC/ASCT) seems to be a useful option in younger patients with unfavorable features [34], the main indication for HDC/ASCT remains consolidation in chemosensitive relapsing disease, as is the case for other diffuse large B cell lymphomas in a small proportion of patients.
    Introduction Epigenetic changes play an important role in the pathogenesis of myeloid neoplasms. Hence, the role of DNA methyltransferase inhibitors such as azacitidine in achieving promising outcomes for patients with MDS has been investigated. Azacitidine was shown to significantly prolong the survival of high-risk MDS patients [1]. However, the characteristics of MDS are very heterogeneous. Significant degrees of myelofibrosis have been reported in approximately 10% of MDS patients. Myelofibrosis was previously shown to be a poor prognostic factor in MDS. The overall survival (OS) achieved with allogeneic SCT was shown to be shorter in MDS patients with severe myelofibrosis than that in those without myelofibrosis [2]. Azacitidine has been shown to have limited therapeutic activity in patients with primary myelofibrosis and post-essential thrombocythemia (ET) / polycythemia vera (PV) myelofibrosis [3]. Thalidomide, lenalidomide, and pomalidomide have also been shown to exhibit the therapeutic activity in patients with myelofibrosis with myeloid metaplasia, primary myelofibrosis, or post-ET / PV myelofibrosis [4–6]. Previous studies suggested that the etiology of primary myelofibrosis may be associated with the activation of Janus kinase (JAK)-signal transducer and activator of transcription (STAT) signaling [7] and an increase in inflammatory cytokine levels [8]. Ruxolitinib, a potent JAK1 and 2 inhibitor, has demonstrated reductions in splenomegaly and disease-related symptoms, and an improvement in overall survival in patients with myelofibrosis [9]. Myelofibrosis with MDS may also be associated with the activation of JAK–STAT signaling and an increase in cytokine levels.
    Case report A 74-year-old man was transferred to our hospital with cytopenia. An initial peripheral blood examination revealed anemia: hemoglobin (Hb) 9.5g/dL, platelets (Plt) 71×109/L, and white blood cells (WBC) 1.84×109/L with 0% blasts, 10% band forms, 23% segmented neutrophils, 2% monocytes, and 65% lymphocytes. His ferritin serum level was 197ng/mL (30–310). Bone marrow biopsy showed hyperplastic bone marrow with myelofibrosis and an increased number of megakaryocytes (Fig. 1A). Differential counts were 29.0% erythroblasts, 10.3% myeloblasts, 2.3% promyelocytes, 7.0% myelocytes, 1.7% metamyelocytes, 8.6% band forms, 10.5% segmented neutrophils, 1.3% monocytes, 1.6% eosinophils, 0.6% basophils, and 25.9% lymphocytes. Blast cells in his bone marrow stained positive for myeloperoxidase and, based on an immunohistochemical analysis, were also found to express CD34. Dysplasia was observed in tri-lineage cells, and was severe in megakaryocytes and mild in neutrophils and erythroblasts (Fig. 1B and C). A cytogenetic examination of bone marrow cells by G-banding analysis showed the normal karyotype, 46,XY[20/20]. The JAK2-V617F mutation was not detected. He was diagnosed as refractory anemia with an excess of blasts (RAEB)-2 with myelofibrosis according to the 2008 World Health Organization (WHO) classification. The risk group was classified as intermediate-2 in the International Prognostic Scoring System (IPSS), high in the revised IPSS, and high in the WHO classification-based prognostic scoring system (WPSS). This myelofibrosis was assessed as grade 3 following the European consensus guidelines. Allogeneic SCT was judged to be an unsuitable treatment because of his age and complications (type 2 diabetes mellitus, and angina pectoris). Therefore, he was treated with azacitidine. He required a red blood cell transfusion before being treated with azacitidine. The risk group was classified as low in the prognostic score for the azacitidine treatment [10].