Development of Acute Promyelocytic Leukemia in a Patient With Gouty Arthritis on Long Term Colchicine

Colchicine is a frequently used drug in rheumatological diseases. Acute promyelocytic leukemia developed in a patient who used colchicine for gouty arthritis since 10 years is presented and the possible relation between the long term use of colchicine and hematological malignancies is discussed

Alpha-Thalassemia Mutations in Adana Province, Southern Turkey: Genotype-Phenotype Correlation

To look over the distribution of the mutations in a large series from Adana province, Southern Turkey, and determine the genotype-phenotype correlation of the frequent mutations. Among the 2500 individuals with mild or moderate anemia, microcytosis, and normal iron levels that were referred to our Genetic Diagnosis Center, a population consisting of 539 individuals were included in the study and tested for alpha-thalassemia mutations by using reverse dot blot hybridization technique. Twelve different mutations were detected in 539 patients. Among the 12 different mutations found, the most frequent mutations were the -alpha(3.7) (63.3 %), --(MED) (11.7 %), --(20.5) (10.7 %), alpha 2(IVS1(-5nt)) (3.9 %), and alpha 2(polyA-2) (3.5 %). The most frequent genotypes were -alpha(3.7)/alpha alpha (35.8 %), -alpha(3.7)/-alpha(3.7)(18.9 %), -(20.5)/alpha alpha (11.5 %), and --(MED)/alpha alpha (10.4 %), respectively. There were statistically significant differences in hematological findings between -alpha(3.7)/-alpha(3.7) and --(MED)/alpha alpha, even though both have two mutated genes in the genotype. Our results show that alpha-thalassemia mutations are highly heterogeneous as well as deletional and -alpha(3.7) single gene deletion is particularly prevalent at Adana province in agreement to other studies from Turkey

Effects of bone marrow fibrosis and angiogenetic structure on autologous hematopoietic stem cell engraftment

Purpose: Hematopoietic stem cell (HSC) engraftment is influenced by many factors. We investigated the effects of bone marrow fibrosis and angiogenetic structure on engraftment in patients with hematological malignancies. Materials and Methods: Data were collected from 34 patients (20 males and 14 females) who underwent autologous HSC transplantation. Bone marrow myelofibrosis was graded from 0 to 3, angiogenesis was quantified using a stereological method in the most recent bone marrow biopsy before the transplantation. Patients were categorized into two groups according to intensity of angiogenesis parameters. Results: Half of the patients had fibrosis and majority had multiple myeloma (73.5%). Eleven patients had grade 1, six had grade 2 myelofibrosis. The engraftment day (ED) for platelets and erythrocytes was significantly different between the grade 2 fibrosis and non-fibrosis groups. VSD and NVES levels were significantly higher in the grades 1 and 2 fibrosis groups than the no fibrosis group. While the overall survival time was shorter in the grade 2 fibrosis group than the others, the difference was not statistically significant. Conclusion: Bone marrow fibrosis was found to be independent risk factor. It may have a negative effect on platelet and erythrocyte engraftment time of autologous transplantation process but this effect does not influence survival

The Effect of Fc gamma RIIIA Gene Polymorphism on the Treatment of Diffuse Large B-cell Non-Hodgkin Lymphoma: A Multicenter Prospective Observational Study

Objective: The curative treatment approach for diffuse large B-cell lymphoma (DLBCL) is controversial even in the rituximab (R) era. The aim of this study was to examine the Fc gamma RIIIA gene polymorphism distribution of DLBCL patients who had been treated with R-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. Furthermore, we investigated the impact of Fc gamma RIIIA gene polymorphism on the overall response rate (ORR) and overall survival (OS). Materials and Methods: Patients from 3 centers in the Aegean region of Turkey who had newly diagnosed CD20-positive DLBCL were enrolled in the study. The single nucleotide polymorphisms of the Fc gamma RIIIA gene were analyzed by real time-PCR. The response to treatment was determined in the middle and at the end of the protocol. During 2 years of follow-up, the patients were clinically and radiologically evaluated for disease status every 3 months. Results: Thirty-six patients were included in the study and the distributions of F/F, V/F, and V/V types of alleles of Fc gamma RIIIA were 25%, 50%, and 25%, respectively. Twenty-seven patients were considered as evaluable according to ORR and OS. The patients' ORR was 87.5%, 100%, and 50% in the F/F, V/F, and V/V allele groups, respectively. We did not establish any statistically significant differences among the 3 alleles groups in respect to ORR (p=0.93). The OS within 2 years in the F/F, V/F, and V/V allele groups was 62.5%, 100%, and 100%, respectively. The OS in the F/F allele group was found to be lower than in the other 2 allele groups (p=0.01). Conclusion: The distribution of gene polymorphisms in our study group was similar to those of previous studies. While ORR was similar between the groups, our results highlight a lower OS in F/F patients compared to other allele groups of Fc gamma RIIIA

Red blood cell alloimmunization in patients with sickle cell disease in Turkey: a single center retrospective cohort study

Purpose: We aimed to investigate erythrocyte alloimmunization frequency and related factors in our region where SCD is common Material and Methods: This study was planned as a single center, cross-sectional and retrospective cohort study. A total of 216 patients who had been followed up due to SCD [Hemoglobin (Hb) SS, Hb S-beta thalassemia, Hb S-alpha thalassemia] were included in this study. Patients were divided to two groups according to amount of transfusion. The patients who had received less than 6 transfusions per year and who did not have the history of erythropheresis were allocated to Group 1, and the patients who had received 6 or more simple transfusion per year or who had undergone erythrocyte exchange were allocated to Group 2 Results: Of 216 SCD patients included in the study. Alloimmunization was detected in 67 (31.0%) out of 216 patients who underwent transfusion, and in 17 (30.4%) out of 56 patients in Group 1 and in 50 (31.3%) out of 160 patients in Group 2. When the patients were analyzed according to alloimmunization development, our study revealed that neither SCD complications are a risk factor for alloimmunization nor alloimmunization increases mortality rates Conclusion: High alloimmunization frequency found in our study suggests the insufficient adherence of alloimmunization-prevention policies in RBC transfusions performed except experienced institutions. Therefore alloimmunization may be reduced or prevented through performing extended red cell typing among SCD patient

A Comparison of the BEAM and MITO/MEL Conditioning Regimens for Autologous Hematopoietic Stem Cell Transplantation in Hodgkin Lymphoma: An Analysis of Efficiency and Treatment-Related Toxicity

In this multicenter retrospective study, we compared the efficacy and toxicity of BEAM (BCNU, etoposide, cytarabine, and melphalan) and MITO/MEL (mitoxantrone, melphalan) preparation regimens. The 3-year expected overall survival for the MIT/MEL and BEAM were 86.1% and 91.3%, respectively. The MITO/MEL seems to be as effective as the BEAM but has better tolerability in terms of pulmonary toxicity and may be used as an alternative option

Anorectal Complications During Neutropenic Period in Patients with Hematologic Diseases

Background: Neutropenic patients are susceptible to any anorectal disease, and symptomatic anorectal disease afflicts 2-32% of oncology patients. Perianal infections are the most feared complication, considering the lack of natural defense against infectious microorganisms. When septic complications develop, the anorectal disease is potentially fatal, especially in neutropenic patients in whom mortality rates range between 11-57%. Although anorectal diseases are a frequent complication with potentially fatal outcomes among patients with hematologic diseases, sufficient data are not available in the literature. In this study, we aimed to investigate the anorectal complications developing during the neutropenic period in patients with hematologic diseases. Methods: A total of 79 patients whose neutropenic period (absolute neutrophil count < 500/mcL) continued for 7 days, or longer were included in the study. Results: A total of 34 patients out of 79 (43%) were detected to develop anorectal complications, of them 6 (7.6%) developed an anorectal infection. The patients were characterized according to the hematological disease and its status (active or not), the type of treatment and the presence of a history of an anorectal pathology before the onset of the hematologic disease. Nineteen (24.1%) patients had the history of anorectal disturbances before diagnosis of the hematologic disease, and recurrence of an anorectal pathology was found in 14 out of 19 patients(73.7%). In addition, the overall mortality rate was higher among the patients who developed anorectal complications compared to another group (41.2% vs. 22.2%, p= 0.059). Conclusion: Anorectal pathology is a common complication with high recurrence rate in neutropenic patients. Perianal infections are important as they can cause life-threatening outcomes although they are relatively rare among all anorectal complications. Therefore perianal signs and symptoms should be meticulously evaluated concerning early diagnosis and treatment

The clinicopathologic features of multiple primary malignancies in hematology: A cross sectional descriptive study

In Turkish literature there are very few studies regarding multiple primary malignancies (MPM). The aim of this study was to analyze the synchronous and the metachronous malignancies that occurred with a hematologic malignancy. All the patients with a hematologic malignancy were enrolled in this cross-sectional, definitive retrospective study. Data were obtained from the medial records. Patients characteristics including demographic features, treatment protocols and overall survival (OS) were recorded. Among 663 patients with a hematologic malignancy, there were 26 patients with MPMs (3.9%). Synchronous malignancies constitute 0.9% and metachronous malignancies were present in 3%. In men diffuse large B-cell lymphoma (DLBCL) and non-small cell lung carcinoma (NSCLC) and in women breast and acute myeloid leukemia were the most common primary and secondary MPMs respectively. The mean cumulative OS of all patients with MPMs was 246.3±33.4 months and the 5 years-OS was 91.3%. In synchronous MPMs the most frequent concomitant tumors were DLBCL and NSCLC. In metachronous tumors the median time interval between first and second malignancies was 69.5 months (range: 31-312). In four patients there were three MPMs. After radiotherapy three patients developed breast, thyroid and skin cancers and in one patient who received radioiodine for the treatment of thyroid carcinoma, DLBCL had developed. The chemotherapeutic agents applied for the primary malignancies consisted of alkylating agents, antimetabolites, anthracyclines, topoisomerase II inhibitors, monoclonal antibodies and mitotic inhibitors. In 75% of the patients with DLBCL who had received R-CHOP chemotherapy regimen, NSCLC had developed during the follow-up period. In conclusion secondary malignancies with hematologic malignancies are not rare and the clinicians should keep the possibility of secondary malignancies in mind and be suspicious during diagnostic evaluations. Warning with regard to the risk of development of secondary malignancies due to the primary treatment should be given to any patient with a hematologic malignancy. [Med-Science 2020; 9(1.000): 94-9