Chronic myelomonocytic leukemia has been reported as the mos
Chronic myelomonocytic leukemia has been reported as the most prevalent concomitant hematological neoplasm, followed by myelodysplastic, myeloproliferative syndrome, and acute myeloid leukemia; on the other hand, Lymphoproliferative neoplasms were less frequency associated with SM-AHMND. In another retrospective study of 342 consecutive patients with SM at the Mayo Clinic extending from 1976 to 2007, their investigation demonstrated that the proportion of SM in ISM, SM-AHMND, ASM, and mast cell leukemia is 46%, 40%, 12% and 1%, respectively. Regarding SM-AHMND, 123 (89%) were associated with myeloid neoplasm, while the remainder were lymphoma (n = 7), myeloma (n = 5), chronic lymphocytic leukemia (n = 2), or primary amyloidosis (n = 1). The sub-classification of SM-AHMND associated with myeloid neoplasm was as follows: 55 (45%) myeloproliferative neoplasm, 36 (29%) chronic myelomonocytic leukemia, 28 (23%) myelodysplastic syndrome and 4 (3%) acute leukemia. Although lymphoid neoplasms were less frequently associated with SM-AHMND, in the case of lymphoid malignancies, almost all B cell neoplasms may develop currently with SM. This phenomenon may be attributed to interactions between the B cell neoplasms and mast cells. One study based on patient pathological findings suggested that angiogenesis and mast cell density with tryptase activity would increase simultaneously pathological progression in B cell non-Hodgkin\'s lymphomas. For ISM treatment, managing dub ubiquitin symptoms such as anaphylaxis, pruritus, malabsorption, and flashing could be achieved by interferon-alpha, corticosteroids, and cytoreductive therapies, such as cladribine. The small-molecule kinase inhibitors, such as imatinib, have yielded only modest clinical benefit, because of unrecognized complexities in their molecular pathogenesis. The survival duration of ISM was not significantly different from the normal population. On the other hand, treatment and outcome for SM-AHMND is dependent on hematological non-mast cell lineage disease. The clinical course and prognosis are strongly correlated with hematological non-mast cell lineage, and the standard regimen for this non-mast cell disease should be administration together with SM management. An earlier study reported the overall median survival rate for patients with SM-AHMND was 24 months, but this survival time varied by subgroup. For example, SM-myeloproliferative neoplasm patients had a significant longer survival time (31 months) than those with SM-chronic myelomonocytic leukemia (15 months), those with SM-myelodysplastic syndrome (13 months), and those with SM-acute leukemia (11 months). Lim et al identified weight loss, advanced age, thrombocytopenia, anemia, hypoalbuminemia, and excess BM blast as adverse prognostic factors for SM. Schipper et al reported a 74-year-old male patient with SM coexisting with DLBCL, and the survival time of this patient was merely seven months owing to lymphoma progression despite immune-chemotherapy administration. In our case, the patient responded well to standard regimen for lymphoma and thereafter experienced long-term survival time.
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Introduction The term “Multiple primary neoplasm (MPN)” was first documented by Billroth et al in 1889. Recently, a trend towards an increase prevalence rate of MPN was noted and probably due to improved treatment results and prognosis of various cancers. Additionally, early tumor detection through an elevated frequency of cancer screening also may impact this increased diagnosis of MPN. Based on the Surveillance Epidemiology and End Results (SEER) data between 1973 and 2000, cancer survivors had a 14% higher risk of developing a new malignancy, and an heightened absolute risk of 21 cancer cases per 10,000 person-years than the general population. MPN has been identified in those cases harboring multiple tumors that meet the criteria by Warren and Gates as follows: 1) Each neoplasm must be malignant arising from histological evaluation; 2) Each neoplasm must be anatomically separate and distinct. If the intervening mucosa demonstrates dysplasia, it must be considered as a multicentric primary lesion and not as two separate neoplasms; and 3) The possibility that the second neoplasm represents a metastasis should be excluded. However, the differentiation of second primary cancer from metastasis is still arguable mainly under the assistance of distance, diagnosis time difference or even molecular analysis of different tumors.