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Aug 2024 DOI 10.14302/issn.2578-2371.jslr-24-5157
Uluc GünayCorresponding author
Introduction It was seen that splenectomy creates a disability situation in an individual, and in order to eliminate it, people applied to health boards to get a report to eliminate their social and economic losses recognized to them. Objective To examine the reason for surgery, method of surgery and the type of report they wanted to receive in splenectomised patients who applied to the general surgery committee polyclinic in 2017-2018-2019-2020 and 2023 when the pandemic ended. Materials and Methods Patients who applied to general surgery outpatient clinics were asked whether they had any surgery related to general surgery, and epicrises and pathology results of splenectomised patients were seen and recorded. Results Of the 23 splenectomised patients, 15 were female and 8 were male. Of the 15 female patients, 3 were splenectomised for ovarian ca, 3 for gastric ca, 2 for distal pancreatic ca, 2 for lymphoma, 1 for colon ca, 1 for traumatic cause, 2 for ITP, 1 for sarcoidosis. In male patients, 4 were splenectomised for traumatic, 1 for colon ca, 1 for ITP, 1 for thalassemia major and 1 for CML. The mean age of female patients was 48.1 years and the mean age of male patients was 37.4 years. The most common reason for splenectomy in women was malignancy and the most common reason for splenectomy in men was trauma. Conclusion Splenectomized patients had applied to receive the most DSR.
Mar 2023 DOI 10.14302/issn.2641-5518.jcci-23-4506
Partanen AnuCorresponding author
Limited data exist on the mechanisms promoting clonal expression of BCR-ABL1 cells in various myeloproliferative disorders. We present a patient whose Janus Kinase (JAK) 2 V617F-negative idiopathic myelofibrosis (IMF) transformed to Philadelphia-positive chronic myeloid leukemia (CML). A 55-year-old man had anemia and splenomegaly. Trephine biopsy showed excess fibrosis without a JAK2 V617F mutation. Diagnosis of high-risk IMF with t(3;12) and del(16q) was made. Five years later a repeated trephine biopsy showed extensive fibrosis and t(9;22) with der(22)t(9;22). BCR-ABL1 fusion gene with typical p210 fusion transcript was found resulting in the diagnosis of CML. A modest treatment response was achieved with tyrosine kinase inhibitor (TKI) therapies, but the disease eventually progressed to a myeloid blast phase. With AML-based chemotherapy plus azacytidine and a second generation TKI the patient survived for years but succumbed 11 years after the initial diagnosis. Clonal evolution may cause atypical disease characteristics or a poor response to targeted therapy in myeloproliferative disorders.
Apr 2022 DOI 10.14302/issn.2372-6601.jhor-22-4133
Qing XinCorresponding author
Department of Pathology, Harbor-UCLA Medical Center, 1000 West Carson Street, Torrance, CA 90502, USA.
Background Monoclonal gammopathy of undetermined significance (MGUS) and chronic myeloid leukemia (CML) are diseases of different lineages. The diagnosis of both MGUS and CML in the same patient is a rare occurrence and has not been reported in much literature. Case Presentation We describe a 56-year-old man with a history of rheumatoid arthritis incidentally found to have an increase in IgA paraprotein. With less than 10% monoclonal plasma cells on the bone marrow biopsy and absence of hypercalcemia, renal failure, anemia and bone lesions, MGUS was diagnosed. The conventional cytogenetics at the time showed the presence of the Philadelphia chromosome in 30% of metaphases. However, there was no morphologic evidence of CML in the peripheral blood or bone marrow. Patient received no treatment and lost follow-up until 3 years later when a routine CBC showed leukocytosis and thrombocytosis. CML, chronic phase was diagnosed following a bone marrow aspiration and biopsy with Philadelphia chromosome observed in 100% of metaphases. Patient was treated with imatinib and later switched to dasatinib and complete molecular remission was continued to be achieved. Discussion and Conclusion Here we report a case of pre-leukemic CML as an incidental finding during the diagnosis of MGUS. The possible underlying mechanisms of the association are discussed although the exact cause of the coexistence is unclear.
Nov 2017 DOI 10.14302/issn.2372-6601.jhor-17-1761
Lee Mortensen GitteCorresponding author
AnthroConsult, Fynsgade 24, 8000 Aarhus C, Denmark
With the introduction of tyrosine kinase inhibitors (TKI), patients with chronic myeloid leukemia (CML) have obtained survival rates close to normal. It may appear paradoxical, then, that medication adherence is suboptimal in some health care settings. As the first of its kind, this study aimed to explore drivers and barriers to TKI treatment adherence in Danish CML patients. A literature study informed the design of qualitative interviews with 20 patients, individually and in focus groups, focusing on their disease perceptions of CML, their health-related quality of life (QoL) and medication adherence. The study showed that many participants had previously switched treatment due to lacking efficacy or intolerance but most felt their current disease burden was tolerable. Anxiety might, however, resurface if treatment stopped working or with the occurrence of infections or side effects, creating a state of ‘fragile peace’. To these patients, their role functioning – as professionals, spouses, parents and grandparents – was crucial to uphold a positive self-image and meaningful life. Whether treatment enabled or hindered this was thus decisive to their QoL and medication adherence. Our participants expressed high adherence rates with only one having intentionally non-adhered due to side effects and poor QoL. Most participants felt well-informed about CML and treatment and privileged to receive specialised personal care from the public health care system acting to motivate their medication adherence. As a novel finding, this study indicates that the prospect of treatment-free remission may positively affect ‘adherence’ suggest this should be explored in future studies.
Feb 2016 DOI 10.14302/issn.2372-6601.jhor-15-822
I.V. ChernikovCorresponding author
Institute of Chemical Biology and Fundamental Medicine SB RAS.
Small interfering RNA (siRNA) based drugs for overcoming multiple drug resistance of hematological malignancies could solve the problem of poor response to the chemotherapy and hight relapse rate. The main factor that significantly limits biomedical application of siRNA is inefficient delivery to target cells and tissues. The attachment of siRNA to molecules, which enter into the cell by natural transport mechanisms, can improve cellular uptake of siRNA. In current study the carrier-free cellular uptake of siRNA containig cholesterol residues conjugated to the 5’-end of the sense strand via oligomethylene linker of various length (here and after Ch-siRNA) was explored. The data demonstrate that cholesterol residue increase the accumulation of siRNA in all tested cell lines and the primary cells. The efficiency of Ch-siRNA accumulation in K562 cells depends greatly on the leangth of the linker connecting cholesterol and siRNA: Ch-siRNAs with linker of 10 - 12 methylene units accumulate the most efficiently in this cells. It was found that Ch-siRNA effectively accumulates in MOLT-3 (acute lymphoblastic leukemia, ALL), HL-60 (acute myelogenous leukemia, AML), K562 (chronic myelogenous leukemia CML) and primary peripheral blood mononuclear cells (PBMC) from patient with non-Hodgkin lymphoma (NHL) or healthy donor resulting in near 100% of transfected cell when used at 1 mM concentration.