Vol 15, No 3 (2021)

Original Article(s)

  • XML | PDF | downloads: 271 | views: 469 | pages: 139-144

    Background: Immune thrombocytopenic purpura (ITP) is defined as a bleeding disorder in which the number and production of platelets are reduced by the immune system; however, the destruction of peripheral blood platelets also occurs. Although its exact etiology and pathogenesis have not already known, several studies have shown that Epstein-Barr virus (EBV) and cytomegalovirus (CMV) are known as possible causative agents of ITP. This investigation aims to evaluate the presence of CMV and EBV in two groups of case and control by polymerase chain reaction (PCR).

    Materials and Methods: we considered the presence of CMV and EBV in 48 acute ITP patients and 48 healthy people. Study participants were recruited from Ahvaz Shafa Hospital between 2017 and 2018 and the presence of two viruses was investigated by (PCR).

    Results: Out of 48 acute ITP patients, the CMV DNA was detected from the blood of 12 (25%) patients and the EBV DNA from the blood of 2 (4.2%) other patients. Also, only one patient was (2.1%) co-infected with CMV and EBV. In contrast, in 48 healthy subjects, 3 (6.6%) had CMV and none of the control group was infected with EBV.

    Conclusion: Due to the presence of both EBV and CMV in the acute ITP patients in Ahvaz, they can be considered as factors in the progression of this disease. Therefore, consideration of the methods of elimination and treatment of these two viruses in these patients may be used as a treatment strategy in ITP patients in the future.

  • XML | PDF | downloads: 188 | views: 262 | pages: 145-151

    Background: Overall five-year survival rate of Wilm’s Tumor (WT) in developing countries is still poor. Delayed diagnosis is one of the contributing factors, whereas early diagnosis is an important thing for the outcome. It is caused by the WT burden in developing countries that was not comparable with the number of facilities for diagnosis and treatment. Ultrasonography (USG) is the mandatory first-line imaging modality in children with a suspected abdominal mass and an overall sensitivity of76%. Additionally, it can be found in many health facilities at a lower cost, quick, non-invasive, and carries no risk of radiation.   Therefore, the relationship between USG and histopathology should be measured.

    Materials and Methods: A cross-sectional study with an analytical approach was performed in pediatric (0 untill 18 year of age)  renal malignancy and neuroblastoma that admitted to Dr. Hasan Sadikin Hospital, Bandung between 2015-2018. Data were collected from medical records. Statistical analyses using Fisher exact test were done to determine the significance of the relationship between USG and histopathology.

    Results: Forty-three samples were obtained based on inclusion criteria, such as  WT (n=33), neuroblastoma (n=6), renal clear cell carcinoma (n=2), and no specific type of renal malignancy (n=2). Fisher exact test revealed the no-significant relationship between USG and histopathology with a p-value > 0.05

    Conclusion: There is no significant relationship between USG and histopathology. Therefore, centralized unity for USG interpretation is recommended.

  • XML | PDF | downloads: 217 | views: 296 | pages: 152-159

    Background: Multiple myeloma is a hematologic malignancy manifested by the secretion of abnormal immunoglobulin. Different methods have been described for diagnosis and patient response to management. Serum free light-chain assay is recently approved in the diagnosis of multiple myeloma patients.

    This study aimed to evaluate the diagnostic accuracy of serum free light-chain assay and its agreement to bone marrow findings.

    Materials and Methods: Forty-six patients with the diagnosis of multiple myeloma were enrolled in the study. The patients were grouped into newly diagnosed cases (22 patients,47.8%) and known cases who were under treatment (24 patients,52.2%). Bone marrow study was done and percentage and clonal status of plasma cells were evaluated by a combination of immunohistochemistry and flow cytometry. Free light-chain assay was done in all patients and sensitivity, specificity, positive predictive value, and negative predictive value were analyzed.

    Results: Thirty of 46 patients showed monoclonal plasma cell infiltration and 16 patients showed polyclonal plasma cell infiltration based on bone marrow findings. An abnormal κ/λ ratio was seen in 15(68.18%) of new cases and 16(66.6%) of known cases. Sensitivity, specificity, PPV and NPV for κ⁄λ ratio were 72.73%, 46.15%, 71%, and 50%, respectively.

    Conclusion: In conclusion, due to high false positive and false negative results, the presence of an abnormal serum FLC ratio was not equal to the presence of monoclonal gammopathy, and observation of a normal ratio does not exclude the presence of monoclonal gammopathy.

  • XML | PDF | downloads: 145 | views: 260 | pages: 160-169

    Background: Chronic lymphocytic leukemia (CLL) is the most common leukemia in adults. The MDM2 and p53 are interacting proteins that play crucial roles in cell biology. Genetic variations of p53 and MDM2 (p53 codon 72 and MDM2 SNP30) have been identified in many cancers including CLL.

    Materials and Methods: In this study, we sought to find the impact of two SNPs of p53 and MDM2 in the pathogenesis of CLL. A total of 100 CLL patients and 102 healthy controls were recruited. Genomic DNA was extracted, and genotyping was performed using the PCR-RFLP method. The allele and genotype associations were analyzed using the χ2 test. The gene-gene interaction analysis was studied using GMDR v0.9.

    Results: Our study found the absence of a significant difference between CLL patients and controls related to the allelic frequencies or genotypic distributions for both MDM2 SNP309 and p53 codon72. A significantly higher frequency of p53 C allele was found in patients with a disease duration of more than 36 compared to those less than 36 months. However, GMDR analysis suggests genetic interaction between the genes under study.

    Conclusion: Our findings indicated each polymorphism of p53 codon72 and MDM2 (SNP309) was not a risk factor for CLL but the p53 C allele could be associated with the disease duration. Besides, the interaction between p53/MDM2 genotypes may confer susceptibility to CLL. Our study could be useful in genetic association studies of CLL and the role of gene-gene interactions in the susceptibility to the disease.

     

  • XML | PDF | downloads: 173 | views: 350 | pages: 170-177

    Background: Autologous HCT in multiple myeloma is done as upfront treatment in newly diagnosed transplant eligible patients after induction chemotherapy. In addition, it is standard for relapsed, aggressive non-Hodgkin lymphoma (NHL) and classical Hodgkin lymphoma (HL), and is curative in ~40% to 45% of patients. Over a decade many efforts were made to find helpful parameters to predict an optimal time for initiating an efficient peripheral blood stem cell collection so that adequate stem cells are collected.  It has been well accepted that CD34+ cell count in peripheral blood before leukapheresis is the best parameter to predict CD34 cell yield. However, white blood cell count, mononuclear cell count, and other easily obtained parameters are still used to guide the clinical practice of peripheral blood stem cell mobilization and collection. 

    Materials and Methods: In the present study, we analyzed the correlation between peripheral blood MNC and Apheresis CD34 levels and also between peripheral blood CD34 by flow cytometry and apheresis CD34 levels.

    Results: We found that there was a statistically insignificant weak correlation between peripheral MNC and apheresis CD34. There was a statistically significant strong correlation between peripheral CD34 and apheresis CD34.

    Conclusion: The results show that peripheral blood MNC was analogous indicating that no reliable prediction can be done for CD34 cells collected in apheresis while peripheral CD34 by flow cytometry is the strongest predictor for initiating stem cell collection.

  • XML | PDF | downloads: 192 | views: 426 | pages: 178-191

    Background: Fresh stem cell exosomes are usually obtained and reused in the same individual. It cannot be kept viable for a long period of time regardless of the lengthy preparation time. Freezing is typically used to preserve the viability of perishable materials and increase their lifetime. Regrettably, normal freezing of biomaterials leads to cell damage. Therefore, a cryoprotectant can save the cells from the conventional cryodamage. Sodium carboxymethylcellulose (NA-CMC) is a powdery substance that is used to manufacture bio-safe hydrofilm gels because of its high viscosity, cytocompatibility, and non-allergenic nature.

    Materials and Methods: Sterile CMC hydrogel was prepared, part of which was loaded with exosomal solution derived from MSCs. The gel was kept at −20°C for preservation. Two bilateral full-thickness circular skin wounds of 2-cm diameter were created on the back of experimental dogs. The wounds were at least 2.5 cm apart. Treatment started 24 hours after wound creation. Group I received CMC gel solely, whereas group II received frozen CMC exosomal gel. The gel was applied 4 times, a single application per day with 1-day interval.

    Results: Clinically, the frozen exosomal gel significantly promoted wound healing with no scaring. Histologically, enhanced dermal fibroblasts and organized collagen deposition were seen in the treated group.

    Conclusion: CMC proved to be an efficient cryoprotectant and a suitable vehicle for exosomes. Deep freezing was proven to conserve the viability, extended the preservation, and facilitated the usage of exosomal gel. This technique of preserved cell-free therapy is inexpensive, time-saving, and proficient and seems suitable for treating cutaneous wounds.

  • XML | PDF | downloads: 148 | views: 264 | pages: 192-198

    Background: Hemophilia is a well-known bleeding disorder with worldwide distribution. Replacement therapy, using plasma-derived or recombinant coagulation factors, comprises a gold standard regimen for the treatment. Regardless of the advancements made in viral inactivation methods in the production of plasma-derived coagulation factors, the possibility of transmission of new viral infections remained as a noticeable concern yet. The aim of the current study was to investigate the status of parvovirus 4 (PARV4) in severe hemophilia A, von Willebrand disease (vWD), and healthy control.

    Materials and Methods: In the current case-control study, 76 patients with hemophilia and vWD and 60 individuals from their family members entered the study. Nested PCR used to determine the presence of PARV4 in study subjects (76 cases). To characterize the PARV4 genotype, positive samples subjected to sequencing and phylogenetic analysis.

    Results: PARV4 genome detected in 11 (14.47%) patients with bleeding disorders. Among whom, nine patients (14.75%) were with severe hemophilia A and two (13.33%) patients with vWD. Only five healthy controls (8.33%) were positive for PARV4. All PARV4 sequences were found to be genotype 1.

    Conclusion: PARV4 infection in patients with hemophilia and vWD was higher than the control group. While detection of PARV4 DNA in patients with bleeding disorders may not necessarily reflect a clinical urgency, future investigations are needed to define the clinical significance of PARV4. It seems the detection of the virus immune signature of PARV4 infection, particularly in the context of acute and persistent infections, needs to focus on cellular and tissue targets.

Case Report(s)

  • XML | PDF | downloads: 190 | views: 332 | pages: 199-205

    Hyperdiploid multiple myeloma (MM) is associated with better prognosis and non-hyperdiploid subtype is associated with variable to adverse prognosis based on the nature of karyotype abnormality.  Rarely exceptions to this hyperdiploid and non-hyperdiploid divisions do exist in a minority. We report an adult male MM patient who showed hyperdiploid karyotype with few novel complex abnormalities and who showed poor clinical outcome. Conventional cytogenetic analysis carried out in 22 GTG banded metaphases showed 53,Y,der(X)t(X;22)(q27;q11.2),+3,+5,+6,+9,+11,+15,der(17)ins(17;1;3)(q11.2;?;?),der(17)ins(17;1;3)(q11.2;?;?),+19,-22 karyotype pattern in 15 metaphases whereas 7 metaphases showed 46,XY karyotype  pattern.  Interphase FISH revealed biallelic del(13q14) and del(17p13) but no translocations involving the 14q32 region. Through Spectral karyotyping FISH, the origin of complex abnormalities involving der(17) chromosome,  translocation t(X;22), and marker chromosome could be clearly delineated. Although the present case showed hyperdiploid karyotype, he showed an adverse prognosis probably due to the co-existence of complex abnormalities and expired 5 months after initial diagnosis despite standard treatment given.