The effect of breathing exercises on chemotherapy-induced nausea and vomiting in autologous hematopoietic stem cell transplantation patients: A randomized controlled trial.

PURPOSE: This research was conducted as a randomized controlled study to examine the effect of breathing exercises on managing chemotherapy-related nausea and vomiting in patients who underwent autologous hematopoietic stem cell transplantation. METHODS: A randomized controlled trial design was used, including an intervention group doing breathing exercises and a control group receiving standard care for chemotherapy-induced nausea and vomiting. The sample was selected from patients hospitalized in the bone marrow transplantation unit. A total of 70 autologous hematopoietic stem cell transplantation patients (35 intervention and 35 control participants). Several variables were evaluated, including the frequence and degree of nausea and vomiting, the patient's nutritional status, and the need for antiemetics. Data were analyzed and interpreted using Mann Whitney U, Pearson chi-square test, Independent t-test, Mann Whitney U, and multiple linear regression. RESULTS: It was found that there was a negative relationship between the number of breathing exercises, the number of nausea and vomiting and the severity of nausea. As the number of breathing exercises increased, the number nausea and vomiting, and severity of nausea decreased significantly (p < 0.05). It was found that the use of antiemetics decreased in the intervention group. Furthermore, it was found that the intervention group had higher daily food consumption on the 3rd, 7th, and 9th days (p < 0.05). CONCLUSION: Breathing exercises were a practical approach to managing chemotherapy-related nausea and vomiting.

A 52-Year-Old Man With Progressive Weakness and Incontinence.

Here we report a challenging case of a 52-year-old man presenting with subacute constipation, urinary retention, impotence, absent Achilles reflexes, and hypoesthesia in S2-S5 dermatomes. We review the clinical decision-making as the symptoms evolved and diagnostic testing changed over time. Once the diagnosis is settled, we discuss the sign and symptoms, additional diagnostic tools, treatment options and prognosis.

Upregulation of T-Cell-Specific Transcription Factor Expression in Pediatric T-Cell Acute Lymphoblastic Leukemia (T-ALL).

OBJECTIVE: T-cell acute lymphoblastic leukemia (T-ALL) is associated with recurrent chromosomal aberrations andabnormal ectopic gene expression during T-cell development. In order to gain insight into the pathogenesis of T-ALLthis study aimed to measure the level of expression of 7 T-cell oncogenes (LMO2, LYL1, TAL1, TLX1, TLX3, BMI1, andCALM-AF10) in pediatric T-ALL patients Material and Methods: LMO2, LYL1, TLX1, TLX3, BMI1, TAL1, and CALM-AF10 expression was measured usingquantitative real-time PCR in 43 pediatric T-ALL patients. RESULTS: A high level of expression of LMO2, LYL1, TAL1, and BMI1 genes was observed in a large group of T-ALL.Several gene expression signatures indicative of leukemic arrest at specific stages of normal thymocyte development(LYL1 and LMO2) were highly expressed during the cortical and mature stages of T-cell development. Furthermore,upregulated TAL1 and BMI1 expression was observed in all phenotypic subgroups. In all, 6 of the patients had TLX1and TLX3 proto-oncogene expression, which does not occur in normal cells, and none of the patients had CALM-AF10fusion gene transcription. Expression of LYL1 alone and LMO2-LYL1 co-expression were associated with mediastinalinvolvement; however, high-level oncogene expression was not predictive of outcome in the present pediatric T-ALLpatient group, but there was a trend towards a poor prognostic impact of TAL1 and/or LMO2 and/or LYL1 protooncogeneexpression. CONCLUSION: Poor prognostic impact of TAL1 and/or LMO2 and/or LYL1 proto-oncogene expression indicate the needfor extensive study on oncogenic rearrangement and immunophenotypic markers in T-ALL, and their relationship totreatment outcome. CONFLICT OF INTEREST: None declared.

Prognostic significance of NOTCH1 and FBXW7 mutations in pediatric T-ALL.

The NOTCH signaling pathway plays important role in the development of multicellular organisms, as it regulates cell proliferation, survival, and differentiation. In adults, it is essential for the T- or B-lymphocyte lineage commitment. NOTCH1 and FBXW7 mutations both lead the activation of the NOTCH1 pathway and are found in the majority of T-ALL patients. In this study, the mutation analysis of NOTCH1 and FBXW7 genes was performed in 87 pediatric T-ALLs who were treated on the ALL-BFM protocols. In 19 patients (22%), activating NOTCH1 mutations were observed either in the heterodimerization domain or in the PEST domain and 7 cases (10%) demonstrated FBXW7 mutations (2 cases had both NOTCH1 and FBXW7 mutations). We also analyzed the relationship of the mutation data between the clinical and biological data of the patients. NOTCH1 and FBXW7, NOTCH1 alone were found correlated with lower initial leucocyte counts which was independent from the sex and T- cell immunophenotype. However, NOTCH1 and FBXW7 mutations were not predictive of outcome in the overall cohort of pediatric T-ALLs.

Minimal Residual Disease (MRD) Detection with Translocations and T-Cell Receptor and Immunoglobulin Gene Rearrangements in Adult Acute Lymphoblastic Leukaemia Patients: A Pilot Study.

Monitoring minimal residual disease has become increasingly important in clinical practice of ALL management. Break-point fusion regions of leukaemia related chromosomal aberrations and rearranged immunoglobulin (Ig) and T cell-receptor (TCR) genes, which can be detected by polymerase chain reaction (PCR), are used as leukaemia specific markers in genetic studies of MRD. A total of 31 consecutive patients with newly diagnosed ALL were screened for eligibility criteria. Of those 26 were included in the study. One patient with partial response following induction therapy and four patients who were lost to follow-up after induction were excluded from the study; thus 21 patients were evaluated for MRD. Chromosomal aberrations were detected in 5 (24%) of the patients and were used for MRD monitoring. Three patients had t(9;22) translocation, the other 2 had t(4;11) and t(1;19). MRD-based risk stratification of the 16 patients analysed for Ig/TCR rearrangements revealed 3 low-risk, 11 intermediate-risk and 2 high-risk patients. MRD monitoring is progressively getting to be a more important predictive factor in adult ALL patients. As reported by others confirmed by our limited data there is a good correlation between MRD status and clinical outcome in patients receiving chemotherapy. The pilot-study presented here is the first that systematically and consecutively performs a molecular MRD monitoring of ALL patients in Turkey.

Alemtuzumab in Sézary syndrome: efficient but not innocent.

Mycosis fungoides is the most common form of cutaneous T-cell lymphomas. The related Sézary syndrome is a more aggressive form in which the skin is diffusely affected and the peripheral blood is involved. Although easily managed during its early phases, late-stage mycosis fungoides/Sézary syndrome is usually difficult to treat and becomes refractory to chemotherapy. Recently, promising case-based results have been obtained with alemtuzumab, a humanized immunoglobulin G1 monoclonal antibody that binds to CD52 cell surface antigens, in the treatment of advanced stage mycosis fungoides/Sézary syndrome. We report a case of Sézary syndrome treated successfully with alemtuzumab but who died of treatment-related infection.

The SOCS-1 gene methylation in chronic myeloid leukemia patients.

SOCS-1, an important protein in the JAK/STAT pathway, has a role in the down stream of BCR-ABL protein kinase. We investigated 56 CML patients and 16 controls for the methylation status of SOCS-1 gene promoter and Exon 2 regions. Exon 2 was found to be methylated in 58.9% of the patients and 93.8% of the controls [P = 0.020, OR = 0.121(0.015-0.957)%95CI]. The promoter region was found unmethylated in all patient samples and controls. Although previous studies revealed a relation between SOCS1 gene Exon-2 hypermethylation and CML development or progression, the results of this study showed no such correlation. On the contrary, our results might be indicating hypomethylation in CML patients, this hypothesis need to be studied in larger study population.

Acute renal failure in a patient with severe hemolysis.

Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal disorder of bone marrow. It is characterized by blood cells lacking membrane proteins that are normally attached by the glycosylphosphatidylinositol (GPI) anchor. The cellular defect arises in a hematopoetic stem cell and is due to somatic mutation of the Phosphatidylinositol-glycan protein-A gene (PIG-A gene), encoding a protein needed for the biosynthesis of the anchor GPI. Paroxysmal nocturnal hemoglobinuria is presented by intravascular hemolysis, cytopenias, frequent infections, bone marrow hypoplasia, and a high incidence of life threatening venous thrombosis. Kidney involvement is usually benign and secondary to chronic tubular deposition of hemosiderin. Acute renal failure may occur in association with a hemolytic crisis. Here we report a case of 40-year-old woman with hematuria, pancytopenia, and acute renal failure due to PNH.

Hematopoetic stem celi transplantation activity at a single center: Cerrahpasa experience.

At our institution 94 transplantations have been performed in an 8 year period up to December 2001. Forty-three females and 49 males with ages ranging from 14 to 61 years received 67 allogeneic (allo) and 27 autologous (auto) transplants; 2 patients were transplanted twice. The diagnosis of allo transplants were AML (27 patients-pts), CML (17 pts), ALL (16 pts) and AA (5 pts); those of auto transplants were NHL (13 pts), HD (10 pts), MM (3 pts) and AML (l pt). Of the patients with acute leukemia 69.7% were in first CR and ali but öne of the patients with CML were in first chronic phase. Source of hematopoetic stem cells were bone marrow (BM) in 61.9% (allo 81.5%, auto 14.8%) and peripheral blood (PB) in 38.1% (allo 18.5%, auto 85.2%). Ail donors were HLA-full matched siblings with öne exception. ConditionIng regimens were BU-CY (31 pts), TBI-CY (28 pts) and Flag-lda (öne pî) for leukemia, CY-ATG for AA, CBV for lymphoma and Mel-200 for MM. Median 2.69 x 108 nucieated celIsAg (BM) and 21.7 x 106 CD34 + celIsAg (PB) were infused to allo transplant recipients; 4 patients faiied to engraft and öne patient was inevaluable due to early death. Acute GVHD was observed in 11 patients (16.9% - grade ll-IV in 10.7%) and chronic GVHD was documented in 18 patients (33.9%- extensive in 9.43%). VOD was seen in 8 patients (12.3%). Early response was CR in 91.6% in patienîs wîth leukemia; patients with lymphoma showed a 73.1% response (CR & PR) rate and 23.1% had resistant disease. So far 27.6% of the patients have relapsed ör showed progression and 45.7% have died (disease-related 27.2%, transplant-related 18.5%). At a median follow-up time of 32 months (range 0.6-96) DFS is 50.8% and OS is 53.9% in the allo transplant group. With a shorter follow-up (median 16 months, range 1-65) the same figures for the auto transplant group are 51.9 and 55.5%, respectively, in this cohort overall, OS and DFS are significantly superior in patients with early-stage disease: 71.7% vs. 26.7% in advanced-stage disease for OS and 71.4% vs. 36.8% for DFS, respectively and this trend applies to both transplant groups (p< 0.001 for ali comparisons).