“The Lolelaplap (Marshall Islands) in Us: Sailing West to East (Ralik→Ratak) to These Our Atolls (Aelon Kein Ad) Ad Jolet Jen Anij (Our Blessed Inheritance from God)”

This paper discusses the expansion of Oceania through a Marshallese indigenous lens as a focal point. It explains that decolonizing methodologies allows reclaiming of space for mental liberation and reassurement of constitutional rights. It highlights similar occurrences of decolonization practices meeting resistance in the 21st century all while strengthening the human right argument that no human deserves any less than their fellow human brothers and sisters. It argues that an indigenous imagery can only be viewed through an indigenous lens where the researches’ level of purity is retained and unfiltered. It nevertheless argues that Marshallese ethnolinguistics reveal the same cultural practices in America, Judeo-Christianity, and Oceania thus dictating the reality that “we are the same not withstanding one stays here and one there (Bedbedjin Bedbedjen, Bedbedjinma wot Kwe)”. It further explains the importance in these similarities and how Marshallese spirituality predates introduced American Judeo-Christianity despite the latter attempting to marginalize the former. It concludes by stating that Marshallese contributions on the global stage are rooted in that culture of love (IaKwe) which is echoed by the custom(s) revealing the significance of Marshallese validation academically, spiritually, economically, & socially to prevent institutionalized discrimination. This paper ends stating that the agency to know one’s self and how one should fit in the world, is a human right in itself and Marshallese are entitled to this sense of self worth through knowing thy self by thy self where real thinking takes place in one’s own mind as we all live our own lives

Review of therapeutic options and the management of patients with myelodysplastic syndromes

Myelodysplastic syndromes (MDS) are a poorly understood group of disorders caused by one or more genetic aberrations in the bone marrow-derived cell line responsible for hematopoiesis. Recent advances in genetic medicine have offered new insights into the epigenesis as well as the prognosis of MDS, but have not resulted in new or improved curative treatment options. Bone marrow transplantation, introduced before the advent of genetic medicine, is still the only potential cure. Advances in other medical and pharmaceutical areas have broadened the scope of supportive care and disease-modifying therapies, and treating physicians now have a broad range of disease management options depending on a patient's likely prognosis. There is now clear evidence that appropriate supportive care and therapeutic intervention can improve progression-free and overall survival of MDS patients

Behçet Hastalığında Hormon Düzeylerinin Değerlendirilmesi

Objectives: This study aims to evaluate the basal serum levels of the hypophysial, thyroidal, adrenal, and gonadal hormones and the disease activity in patients with Behcet's disease (BD). Patients and methods: Forty-three patients (22 males, 21 females; mean age 34 years; range 21 to 46 years) were divided into the following four groups according to the disease activity and presence of ocular involvement: Active BD (n=27), inactive BD (n=16), those with ocular involvement (n=14), and those without ocular involvement (n=29). A group of 40 (18 male, 22 female) healthy subjects matched according to age and gender were used as the negative control group. A positive control group (two males and 14 females) consisted of 16 patients with systemic lupus erythematosus. Triiodothyronin, thyroxin, the thyroid-stimulating hormone, the follicle-stimulating hormone, the luteinizing hormone, the growth hormone (GH), dihydroepiandrosterone sulphate (DHEAS), estradiol, testosterone, basal cortisol, prolactin (PRL), and the adrenocorticotropic hormone were evaluated in all subjects. Results: No clinically significant differences were found between the BD groups and negative control group in terms of the basal serum levels of the hypophysial, thyroidal, adrenal, and gonadal hormones. When the hormonal values were evaluated with regard to disease activity and ocular involvement, the BD patients with ocular involvement showed significantly higher PRL and GH values in both genders and significantly lower DHEAS levels in female patients. Conclusion: Despite being a vasculitis-related disease, we can conclude that BD has no major influence on the endocrine system. Also, further, large-scale studies are needed to evaluate the correlations between PRL, GH, and DHEAS values and ocular involvement.WoSScopu

Nasal Natural Killer/T-Cell Lymphoma With Skin, Eye, And Peroneal Nerve Involvement

Nasal-type natural killer (NK)/T-cell lymphoma (NKTL) is a rare disease strongly associated with Epstein-Barr virus and is often localized to the upper aerodigestive tract at presentation. Extranodal NKTL may involve any extranodal site and disease beyond the nasal cavity is highly aggressive, with short survival time and poor response to therapy. Herein we present a 57-year-old male that had been treated with systemic chemotherapy and cranial radiotherapy for nasaltype NKTL in the palate with skin, right eye, and right peroneal nerve involvement. He was given salvage chemotherapy consisting of 3 cycles of ICE and his response to the therapy was satisfactory, except for persistent right drop foot. About 6 weeks later, the patient presented with bilateral total loss of vision and proptosis; therefore, DHAP chemotherapy was started. Unfortunately, after 1 cycle of the second salvage chemotherapy, he died due to severe fungal infection of the hard palate. , Despite the fact that involvement of any extranodal site is possible, concurrent involvement of many systems in NKTL patients is unusual. Nasal-type NKTL has a poor prognosis, despite local radiotherapy and systemic chemotherapy. Physicians should be aware of this rare disorder than can only be diagnosed after extensive immunohistochemical studies., Conflict of interest:None declared.PubMedWoSScopu

Eculizumab Before and After Allogeneic Hematopoietic Stem Cell Transplantation in a Patient With Paroxysmal Nocturnal Hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by the triad of intravascular hemolysis, venous thrombosis, and cytopenia. Treatment of PNH is generally supportive. Bone marrow transplantation is the only curative therapy for PNH, but is associated with significant morbidity and mortality. Herein, we present a patient with PNH that received eculizumab, a humanized monoclonal antibody that blocks activation of the terminal complement at C5, before and immediately following allogeneic peripheral stem cell transplantation. Prior to hematopoietic stem cell transplantation eculizumab treatment markedly reduced hemolysis and transfusion requirement; however, 1 d post transplantation a hemolytic episode occured, which was successfully stopped with eculizumab re-treatment. Afterwards the patient did not require additional transfusions. The results of this study indicate that early administration of eculizumab may be a safe and effective therapy for hemolytic episodes associated with allogeneic peripheral stem cell transplantation in patients with PNH. (Turk J Hematol 2011; 28:223-7)WoSScopu

Unclassifiable non-CML classical myeloproliferative diseases with microcytosis

Background/aim Polycythemia Vera (PV) is a myeloproliferative disorder characterized by overproduction of morphologically normal red blood cells (RBCs), granulocytes, and platelets, a phenotype that is caused by a mutation (V617F) in Janus kinase 2 (JAK2). However, JAK2 V617F is also found in approximately 50% of patients with essential thrombocytosis and primary myelofibrosis, rendering its presence nonspecific as a diagnostic test. An increased red cell mass is a major criterion for the diagnosis of PV according to World Health Organization (WHO) 2016 criteria. High hemoglobin (Hgb) or Hematocrit (Hct) are universally used as indicators of an increased red cell mass for the diagnosis of PV. However, conditions such as iron deficiency (ID) with decreased mean cell volume may mask the diagnosis due to nonelevated Hct level. The aim of this study was to investigate the clinical characteristics of the patients with unclassifiable non-CML classical myeloproliferative disease with microcytosis (MPD/M) and nonelevated Hgb and Hct levels at diagnosis and to determine if some of these cases could be real PV cases masked due to ID-related microcytosis. Materials and methods There were 23 MPD/M cases among 208 non-CML classical MPD cases (11%). Among 22 patients who had adequate test results related to the cause of microcytosis, ID and beta-thalassemia trait (TT) were the apparent causes of microcytosis in 15 and 1 cases, respectively. Results Clinicopathological correlations revealed consistently positive JAK2 V617F mutation status (20/20, 100%), frequently elevated RBC count (17/23, 73.9%), and PV-compatible bone marrow findings (10/12, 83.3%). These findings are compatible with PV instead of essential thrombocytopenia or primary myelofibrosis. In spite of frequent cytoreductive treatment, 3 patients developed increased Hgb/Htc levels during median 58.2 (279–63) months’ follow-up. Conclusion These data show that the majority of MPD/M cases are PV patients masked due to ID-related microcytosis.PubMedWoSScopu

Allografting for Bosutinib, Imatinib, Nilotinib, Dasatinib, and Interferon Resistant Chronic Myeloid Leukemia without ABL Kinase Mutation

The current treatment of chronic phase chronic myeloid leukemia (CML) consists of oral tyrosine kinase inhibitors (TKIs). However, high-risk CML may present with an aggressive course which may result in blastic crisis or a "difficult-to-manage" state with available treatments. The aim of this paper is to report a patient with complicated CML resistant to treatment and progressed despite the administration of bosutinib, imatinib mesylate, nilotinib, dasatinib, interferon alpha 2a, cytotoxic chemotherapy, and allogeneic hematopoietic stem cell transplantation. The striking point of this case story is that no Abl kinase domain mutation against TKIs has been detected during this very complicated disease course of CML. Meanwhile, challenging cases will always be present despite the hope and progress in CML in the TKI era

Mitoxantrone-Melphalan Conditioning Regimen For Autologous Stem Cell Transplantation In Relapsed/Refractory Lymphoma

Background/aim High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) has become the standard approach for patients with relapsed/refractory Hodgkin’s lymphoma (HL) or non-Hodgkin’s lymphoma (NHL). In this study, we report the outcome of the mitoxantrone-melphalan conditioning regimen for lymphoma. Materials and methods The study group included 53 patients who were relapsed/refractory HL (n = 14) and NHL (n = 39) and received mitoxantrone and melphalan followed by ASCT. The transplant regimen consisted of mitoxantrone (60 mg/m2) and melphalan (180 mg/m2) followed by peripheral blood stem cell infusion (PBSC). Results Prior to high-dose chemotherapy, 37.7% of the patients were in complete remission (CR) and 45.3% were in partial remission (PR), and 17% had stable or progressive disease. After high-dose chemotherapy and PBSC, 44 out of 51 patients achieved CR (86.2%). CR was achieved in 24 out of 33 patients (72.7%) who were transplanted in a marginally active phase of the disease. At a median followup of 25.4 months (1.8–131.3 months) after ASCT, 13 patients relapsed/progressed and 8 patients died. The estimated 2-year overall survival (OS) was 81.9%, and event-free survival (EFS) was 59.3%. Conclusion High-dose chemotherapy followed by ASCT is an effective conditioning regimen in relapsed/refractory lymphoma patients who are undergoing ASCT.PubMedWo

Sole Infrequent Karyotypic Aberration Trisomy 6 In A Patient With Acute Myeloid Leukemia And Breast Cancer In Remission

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