Vol 13, No 1 (2019)

Published: 2019-01-02

Editorial

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    Iran is a member of the International Society for Blood Transfusion (ISBT) working party on rare donors since 20102. Recognition of individuals with Rhnull phenotype was reported to the working party in order to be added to the list of countries with Rhnull donors.
    Iranian database of rare blood groups is followed by international researchers and is important for the Rare Donor Working Party of the International Society of Blood Transfusion (ISBT). Iran is a country with a relatively high prevalence of rare phenotypes and further new blood groups were newly identified.
    In our published paper, we reported a case study of siblings (a 43-year-old female and her brother) who were strongly suspicious of presenting a rare Rhnull phenotype. Their extended RBC phenotyping showed that they were negative for D, C, E, c, e antigens. We now found out that the brother passed away of unknown causes a few months ago. This means that now we have only one person with this very rare blood group, which raised our concern for supplying compatible blood at the time of need, since the deceased brother was the only donor for his sister. There are two units of frozen RBC donated previously by him in stock. We are almost sure that blood transfusion services might face challenges to provide compatible blood to this individual who lacks all Rh antigens.
    In conclusion, at present, there is only one individual (a female) patient with the rare Rhnull phenotype in Iran.

Case Report(s)

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    T-Cell Rich B-Cell Lymphoma (TCRBCL) is relatively a new entity, lately classified as a morphologic variant of Diffuse Large B-cell lymphomas (DLBCL). It consists (1-3) % of all B-cell lymphomas. The rate is far less when describing cases of primary splenic involvement with TCRBCL. Pathologically, TCRBCL is described as a limited number of scattered, large, atypical b-cells embedded in a background of abundant t-cells and frequently histiocytes. The similarity of this malignancy with other types makes it difficult to distinguish between them. Thus, it needs expertise in both clinical and pathological fields to make the right diagnosis.
    Here, we present a case of an adult male patient whose first presentation and previous medical history of renal colic misguided the initial diagnosis and suggested another colic episode as the underlying ailment. However, further physical, radiological and histopathological investigations uncovered the presence of primary TCRBCL within spleen with no involvement of other sites. Moreover, unusual pathologic finding of CD3 positivity was proved by immunohistochemistry.

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    Background: Autologous stem cell transplantation is considered a standard of care treatment in eligible patients with multiple myeloma, but puts the patient at high risk for infections and bleeding complications. Acquired von-Willebrand's disease (AVWD) and acquired platelet dysfunction are rare bleeding disorders that are associated with lymphoproliferative disorders such as multiple myeloma. Patients with acquired bleeding disorders who are planned for ASCT to treat the underlying condition are considered at highest risk for bleeding complications, and optimal treatment strategies are not known.
    Materials and Methods: We summarized the diagnostic and therapeutic approach to a patient affected by AVWD and acquired platelet disorder related to multiple myeloma. The patient who was planned for ASCT presented with moderate to severe bleeding symptoms.
    Results: Acute bleeding episodes were successfully controlled and prevented during induction and consolidation therapy with immunoglobulins, whereas replacement of plasma-derived VW factor showed no clinical improvement. High-dose melphalan-based consolidation therapy supported with autologous stem-cell transplantation led to an immediate and sustainable rise of von-Willebrand antigen and activity and a subsequent normalization of platelet aggregation activity. After a follow-up of 40 weeks, the patient maintained normalized VW levels and platelet aggregation capacity. There were no further signs or symptoms of bleeding.
    Conclusion: This case report highlights the necessity for combined supportive and causal treatment in patients with AVWD and paraproteinemic PD. High-dose melphalan with autologous stem cell support may function as a treatment option in patients with myeloma-related AVWD.

Original Article(s)

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    Background: Lifestyle is a key issue in the concept of health promotion. Lifestyle includes all activities that encourage optimum physical, spiritual, and mental functions. The aim of this study was to determine the effect of a training program based on PRECEDE-PROCEED Model on lifestyle of adolescents with beta thalassemia.
    Materials and Methods: In this clinical trial study, 64 adolescents (age 16-20) who referred to the Thalassemia Center of Ahvaz (2015) were selected and randomly divided into two groups: experimental and control group. The components of the PRECEDE-PROCEED Model were used for planning, implementation and evaluation of the program. Changes in predisposing, reinforcing, enabling factors and lifestyle were immediately and a month after the intervention were assessed by a questionnaire based on PRECEDE-PROCEED Model and the Health-Promoting Lifestyle Profile.
    Results: The intervention had significantly positive effect on predisposing, enabling and reinforcing factors immediately and a month after the intervention (P < 0.05). Repeated measures analysis of variance showed a significant positive increase in the six dimensions of lifestyle score in the experimental group from baseline to one-month follow-up (P < 0.05).
    Conclusion: This study showed that the theory-based training program in adolescents suffered from beta thalassemia disease could improve the adolescent’s awareness and attitude of healthy lifestyle.

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    Background: Acute lymphoblastic leukemia (ALL) is resulted from the infiltration of high amount of non-differentiated cells in bone marrow. Differentiation of the hematopoietic stem cells into specific cell lineage occurs through a highly regulated pathway which is mainly monitored during transcription step. Expression level and pattern of transcription factors e.g. PU.1 determine fate and developmental phases in this pathway. This study was performed to evaluate the expression level of the PU.1 gene in a group of children suffering from ALL.
    Materials and Methods: The mRNA expression level of the PU.1 gene was compared between 30 children diagnosed as new cases of ALL and 30 sex- and gender-matched healthy children in the present case-control study. The quantitative real time PCR (qRT-PCR) was used to determine the level of PU.1 gene expression. The data were analyzed using Graph Pad Prism statistical software.
    Results: The mRNA level of the PU.1 gene was significantly lower in the blood samples of the ALL patients compared to the controls (p= 0.002).
    Conclusion: The results of the study indicated that the PU.1 gene seemed to have key roles in the differentiation pathway of blood cells.

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    Background: Semaphorins play prominent roles in physiological and pathological processes such as vascular development, tumor growth and immune responses. Semaphorins have different roles in various kinds of cancers, but there is no study concerning their expression in the chronic lymphocytic leukemia (CLL). This study aimed to assess the SEMA3A, SEMA4A and SEMA4D expression in patients with CLL. 
    Materials and Methods: Peripheral blood specimens were collected from 30 newly-diagnosed untreated patients with CLL and 30 healthy subjects as a control group. The SEMA3A, SEMA4A and SEMA4D expression was determined by real-time PCR method. 
    Results: The fold change expression of SEMA3A and SEMA4D was 7.58 ± 2.66 and 3.20 ± 0.99 in patients with CLL, and was 1.01 ± 0.31 and 1.00 ± 0.27 in healthy subjects, respectively. The CLL patients expressed higher amounts of SEMA3A and SEMA4D in comparison with healthy subjects (P<0.02 and P<0.03, respectively). The fold change expression of SEMA3A in patients with stage II (11.12 ± 5.35) was also higher than patients with stage I (4.49 ± 1.61, P<0.05). No significant difference was also observed in the expression of SEMA4A and SEMA4D between patients with stage I and stage II CLL. In both CLL and control groups, the fold change expression of SEMA3A was higher in men than in women (P<0.03 and P<0.02, respectively).
    Conclusion: The results of the study indicated elevated expression of the SEMA3A and SEMA4D in patients with CLL. The SEMA3A expression was influenced by tumor stage and gender of participants. 

     

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    Background: Sickle cell disease (SCD) is a hereditary disorder characterized by hemolytic anemia with different clinical manifestations. Patients with SCD exhibit a chronic inflammatory state and reduced length and quality of life. Interleukin-1 β (IL-1β) is important in acute and chronic diseases; and its single nucleotide polymorphisms (SNP) have been considered as predictors of prognosis in several inflammatory conditions. This study aimed at exploring IL-1β (+3954C/T) SNP as a potential genetic modifier and/or predictor of SCD clinical and laboratory phenotypes.
    Materials and Methods: This cross-sectional study involved 50 SCD patients and 50 age, sex and ethnicity-matched healthy individuals. IL-1β (+3954C/T) SNP was identified by PCR-RFLP. Associations between IL-1β (+3954 C/T) SNP and the clinical and laboratory profiles of patients with SCD were studied.
    Results: It was found that the homozygous mutant genotype TT was significantly higher in cases compared to controls [13(26%) vs. 3(6%) respectively; p=0.006, OR (95%CI): 5.505(1.460-20.756)]. The homozygous mutant genotype TT was associated with a higher mean pulmonary arterial pressure when compared to the CC and CT genotype (42.62 vs. 33.49 mmHg, p<0.001).
    Conclusion: There is an increased prevalence of the mutant genotype of IL-1β +3954 SNP in Egyptian SCD patients. Regarding disease complications, the mutant genotype was more prevalent in cases complicated by pulmonary hypertension. These findings point to the possible role of IL-1β +3954 SNP in the pathophysiology of SCD and its manifestations.

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    Background: Thalassemia patients need repeated transfusion that lead to increased blood ferritin level and iron overload in the heart and liver. Because the roles of hepcidin antimicrobial peptide (HAMP) and hemocromatosis protein (HFE) in iron metabolism have been confirmed, this study investigated the effects of these gene's polymorphisms on blood ferritin levels and iron overload in the heart and liver in patients with beta thalassemia major
    Materials and Methods: This cross-sectional study was conducted on 91 patients referring to the Hajar Hospital in Shahrekord, Iran in 2015. After the blood samples were collected, the ferritin levels were measured, DNA was extracted from the blood cells, and the types of polymorphisms were determined using PCR-RFLP. Data of MRI T2* in the heart and liver were drawn from the patients' medical files. Data analysis was conducted by t-test, chi-square test, Fisher's exact test, and Pearson correlation coefficient.
    Results: There was no significant correlation between blood ferritin level and c.-582 A>G polymorphisms of hepcidin gene (p=0.58), and H63D of HFE gene (p=0.818). In addition, there was no significant association between the polymorphisms and heart and liver MRI, but there was a significant association between blood ferritin level and qualitative heart and liver MRI (r=-0.34, p=0.035 and r=-0.001, p=0.609, respectively).
    Conclusion: In patients with β-thalassemia major, the presence of c.-582A>G HAMP and H63D HFE polymorphisms is not effective on blood ferritin level and iron overload in the heart and liver in the studied region.