2020 CiteScore: 2.6
Ardeshir Ghavamzadeh, MD.
Vol 12, No 4 (2018)
Background: Pleomorphic xanthoastrocytoma is a rare tumour of children and young adults, particularly for those with features of anaplasia.
Materials and Methods: This retrospective study comprises five cases of anaplastic pleomorphic xanthoastrocytomas diagnosed over a period of 4 years in a tertiary care institute. A detailed linicopathological and immunohistochemical profile of the tumours were noted from the hospital database.
Results: Five cases of anaplastic pleomorphic xanthoastrocytomas were evaluated for their clinicoradiological, histomorphological as well as immunohistochemical findings, which included 3 females and 2 males, with age range of 11-40 years and a mean age at presentation of 22 years. Histologically a solid cystic biphasic tumour with moderate to high cellularity, spindled pleomorphic astrocytes, hyperchromatic nuclei showing moderate to marked atypia, intranuclear inclusions, ≥5 mitoses per 10 high power fields, with evidence of necrosis and atypical mitoses was noted. One of the cases showed transformation into glioblastoma with evidence of spinal metastasis on follow-up. The tumours expressed both glial as well as neuronal markers with expression of CD34 with increased Ki 67 ranging between 5-20%.
Conclusion: It was concluded that PXA, a low-grade glioneuronal tumour, can show odd site presentation, marked pleomorphism, increased mitosis, atypical mitoses and increased Ki 67 when associated with features of anaplasia. An appropriate panel of immunohistochemical markers in conjunction with a detailed evaluation of histomorphological features and clinicoradiological information are useful for its diagnosis.
Background: Consanguineous marriage is defined as inbreeding between second cousins or closer. In such families there will be a potential increase in the autosomal recessive traits with its lethal effect, with an increased risk of morbidity and mortality in the new generation. Inherited bleeding disorders (InBDs) are rare complicated diseases, difficult and expensive to treat, the defect usually due to quantitative or qualitative deficiency of clotting factors, platelets or fibrinolysis. This study attempts to assess the diversity, the frequency and the clinical features of inherited bleeding disorders (InBDs) in central part of Iraq and to determine the state of consanguineous marriage.
Materials and Methods: This is a prospective cross-sectional study conducted in the National Center of Hematology NCH, Baghdad, Iraq between June2014 and June 2017. In total, 256 pediatrics and adult patients were included. Full bleeding history, family history, drug history and consanguineous marriage were recorded and followed by medical examination. First-line laboratory tests were performed and then were followed by further tests included mixing study, lupus anticoagulant testing, clotting factor activity assay, von Willebrand Antigen (VW: Ag), Ristocetin co factor vWF: RiCoF activity and platelet function test.
Results: The range of age was from 1 month to 57 years, with mean age 8.424±8.623 years and median age of 6.5years. The male to female ratio was 1.1:1. The most common age group was in the range of 1-10 years (46.45%). Family history was positive in 55.07% of patients (P >0.05). The consanguinity was found in 76.95% of the families studied (P <0.0001). The most prevalent InBD was von Willebrand disease (42.98%) with majority type 3VWD (86.4%). The second most prevalent was thrombasthenia (36.71%) and the majority had Glanzmann’s thrombosthenia (86.2%). Rare bleeding disorders (RBDs) were observed in 6.25% of patients and the most common factor deficiency was FVII.
Conclusion: Consanguinity is high in patients with inherited bleeding disorders in Iraq, leading to emergence of life-threatening autosomal recessive inherited diseases. Genetic counselling is recommended besides education and awareness to minimize such rare illnesses in the community.
Background: The present study was conducted to determine the response to treatment in patients with GTN, the survival rate and to investigate the outcomes of first pregnancy after chemotherapy.
Materials and Methods: The treatment protocol was based on the FIGO Staging of GTN and the Modified WHO Prognostic Scoring.
Results: Complete remission was achieved with MTX in 100% of the low-risk patients and with combination therapy in 91% of the high-risk cases. Out of 27 low-risk patients, 21 had no metastasis 6 had lung metastasis, 18 preserved their fertility and conceived in the first year following the chemotherapy. Out of 3 patients who had developed invasive moles, 1 got pregnant after chemotherapy. Four of the patients with choriocarcinoma conceived in the first year following the chemotherapy. In the patient with placental site trophoblastic tumors, there was no pregnancy due to hysterectomy.
Conclusion: GTN was found to be a chemosensitive condition, but more effective therapeutic protocols are therefore required.
Background Primary tracheal cancers (PTCs) are rare and current evidence-based understanding is limited to retrospective reports and national databases. We present single institutional study of a historical cohort of PTC from Canadian provincial cancer registry database.
Materials and Methods: After institutional research ethics board approval, all PTC patients diagnosed from 1980 to 2014 were identified through the Canadian provincial cancer registry. Demographic and tumor related factors were evaluated using descriptive statistics. Survival rates were estimated using the Kaplan-Meier method and cox hazard regression analyses were performed to identify predictors of disease-free survival (DFS) and overall survival (OS).
Results: A total of 30 patients were included in the study. At presentation, 10 patients (33%) had only local disease, 14 patients (47%) had locoregional disease and the remaining 4 patients (13%) had distant metastasis. The majority of patients underwent primary radiation treatment. The overall survival rate was 30% at 2 years and 16% at 5 years. Patients receiving radical-intent therapy had better 2-year DFS and OS compared to patients managed with palliative radiotherapy and best supportive care (46%, 17% and 0%) (p=<0.001) and (50%, 23% and 0%) (p=<0.001), respectively. Radiotherapy resulted in a better 2-year OS and DFS (32% versus 14%) (p=<0.03) and (32% versus 0%) (p=<0.001), respectively.
Conclusion: PTC is an uncommon neoplasm making the study of the disease technically and logistically challenging. Radical radiotherapy alone is curative option in inoperable PTC. Intent of treatment and radiotherapy were associated with superior survival outcomes.
Background: Toll-like receptors (TLRs) are a family of transmembrane pattern-recognition receptors that play a crucial role in the realization of innate and adaptive immune response. TLRs may play a role in tumor development and growth because of expression or up-regulation of functional TLRs in some tumors and tumor cell lines. The participation of TLRs in the pathogenesis of acute myeloid leukemia (AML) remains unspecified. This study aimed to investigate the effect of TLR2 and TLR4 expression in peripheral blood mononuclear cells of AML patients in response to induction chemotherapy.
Materials and Methods: Eighty- five patients with newly diagnosed AML were evaluated. Using quantitative reverse transcriptase PCR, the mRNA expression of genes TLR2 and TLR4 was measured before starting and after induction chemotherapy. The differences in the mean expression levels of TLR2 and TLR4 before and after chemotherapy were compared using a paired t-test. The mean expression levels of TLR2 and TLR4 regarding laboratory data were analyzed by one-way ANOVA and Chi-square test.
Results: We found that the mRNA expression of TLR2 after induction chemotherapy was significantly lower as compared to before treatment (p=0.001). Also, we found a lower TLR4 gene expression level after chemotherapy as compared to before chemotherapy, albeit it was not statistically significant (p=0.21). Moreover, we observed significantly higher expression of TLR2 and TLR4 in AML-M3 cases compared to non-M3 AML patients.
Conclusion: The decreased expression of TLR4 in leukemic samples after induction chemotherapy might indicate a novel potential prognostic role for this receptor, particularly in AML-M3 cases.
Background: Cancer affects the physical, psychological, and social aspects of the patients’ life. Cancer-related fatigue (CRF) is the most common and severe condition among cancer patients. Ginseng has long been used as an efficient treatment for CRF and improvement of quality of life (QOL). The present study aims to assess the efficacy of Panax Ginseng (PG) in reducing CRF in patients with non-metastatic cancer. In addition, the safety of the medication is evaluated.
Materials and Methods: This was a prospective clinical trial conducted on the patients (n=113) suffering from non-metastatic colon cancer (age range: 20-70 years old) referring to the Shafa Hospital, Ahvaz, Iran for chemotherapy treatment. After the chemotherapy sessions, the patients were randomly divided into two groups. The first group received daily dose of 100 mg PG for 30 days and the second group received placebo medication. The demographic information and clinical parameters of the patients including age, sex, weight, symptoms of fatigue, depression, sleep disturbances, and pain were measured pre and post intervention. Afterwards, the variables were compared in each group and between the groups.
Results: Results of study showed that the ginseng improved the quality of life and mood in the subjects. (P<0.0001) and no difference was observed in the placebo group (P=0.887).
Conclusion: The use of ginseng may can effective on reducing CRF and the associated symptoms in the patients with cancer, but further studies should be conducted for the evaluation of comprehensive therapeutic efficacy.
Background: Cancer-related fatigue (CRF) is a very prominent complaint and disabling symptom in cancer patients probably influenced by endogenous cytokines. But, the published data on this subject are limited. We explored the relationship of cytokines such as tumor necrosis factor (TNF-α) and interleukin-6 (IL-6) with fatigue in patients with AML.
Materials and Methods: This study was performed on 45 patients (25 men, 20 women) with newly diagnosed AML. We examined fatigue in these patients with validated questionnaire. Simultaneously, blood samples were obtained for quantitative measurement of IL-6 and TNF-α.
Results: Our results showed a positive correlation between fatigue and circulating levels of IL-6 (P=0.004, R=0.416).
Conclusion: Many patients with AML experienced severe fatigue before the onset of treatment, which is not related to their hemoglobin (Hb) levels. Cytokine levels may be beneficial markers in resistance to fatigue, but further studies are needed before considering targeted therapies as a treatment for CRF.
Background: Cardiac muscle possesses a limited capacity to regenerate its tissue on its own. It is less likely to reverse the altered cardiac functioning to its normal physiological state after a major myocardial infarction. Stem cell transplantation provided a unique therapeutic approach in managing such injuries. There has been a substantial debate about the complexity, scope and medical application of stem cell transplantation in past few years.
Materials and Methods: An extensive review of medical literature was conducted to establish the consensus about the possible mechanism of cell renewal, associated complications and risks of failure of this technique. Twenty cases of mammalian animals and twenty-four cases of stem cell transplantation in human subjects were reviewed.
Results: Most common associated complication was re-stenosis of coronary artery. Few clinical trials reported the failure in improving cardiac functioning. The success rate of stem cell transplantation was remarkable in the literature related to experimental animal subjects.
Conclusion: It was concluded that renewal of the cardiac cell is a result of induction of angiogenesis and prolonged cell survival. This topic still requires an immense amount of research to fill the gap in adequate knowledge.
Hematopoietic stem cell transplantation (HSCT) is a useful treatment. In contrast to solid organ transplantations, the use of ABO blood group mismatch is acceptable in HSCT. Immediate or late hemolytic reactions, pure red cell aplasia, delayed red blood cell recovery, and graft-versus -host disease are the results of this situation. This review shows the consequences of ABO-mismatched HSCT and its impacts on HSCT parameters, as well as providing clinical guides in this situation.
Adrenal involvement in the course of malignant lymphoma occurs in about 4% of patients, but primary adrenal lymphoma (PAL) is extremely rare. To the best of our knowledge, only one case study reported the combination of PAL with pheochromocytoma. In the current study, we present the second case who was a 63-year-old man admitted to our hospital with hematuria and abdominal discomfort. Ultrasound imaging indicated the presence of a lesion, 5×4×3 cm in size, in the upper pole of his right kidney. Histopathologic study confirmed a collision tumor composed of pheochromocytoma and diffuse large B-cell lymphoma (DLBCL).
TAFRO syndrome is a rare subtype of the Castleman’s disease which has been described over the last years. The name of TAFRO syndrome comes from thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. We report a young Turkish male patient presented with fever, night sweats, fatigue, nausea, bilateral pretibial pitting edema, abdominal pain and watery diarrhea. PET/CT revealed multiple lymphadenopathies in cervical, axillary, mediastinal, paraaortic, mesenteric and inguinal lymph nodes. Excisional lymph node biopsy showed atretic germinal centers and expanded interfollicular areas, containing sheets of plasma cells. The R-CHOP regimen was started, and his signs and symptoms improved after the treatment. The current case confirms the unique presentation of this syndrome, helping to understand its clinical course and treatment strategy.
The 5q35.2-q35.3 duplication phenotype is characterized by growth delay, microcephaly, mental retardation and delayed bone aging. However, there has been no reports on the occurrence of pancytopenia as a consequence of 5q35.2-q35.3 duplication. A 42-year-old male visited the emergency room due to multiple trauma. He had been diagnosed with mental retardation in the past. No previous history of severe bleeding symptom was also reported . Complete blood cell counts were leukocyte 3.51×109/L, neutrophil 0.19×109/L, hemoglobin 8.3 g/dL, hematocrit 25.0%, and platelet 4.0×109/L. There was no relevant history of any medication intake and there were no other haematological parameters leading to the persistent pancytopenia. A bone marrow biopsy revealed hypercellular marrow with increased trilineage hematopoiesis. The uptake of fluorodeoxyglucose was increased in multiple lymph nodes, bone and spleen in positron emission tomography–computed tomography. A biopsy of the right axillary lymph node was performed and histologic findings were unremarkable. The chromosomal microarray revealed a 3.46 Mb microduplication at the 5q35.2-q35.3 site including NSD1. The patient had distinctive features related to atypical pancytopenia. Various managements for pancytopenia had no effect on the patient. However, there were no complications such as massive bleeding or serious infection compared to the severity of pancytopenia during a follow-up of 3 months. In addition, periodic patterns of deterioration and improvement in pancytopenia appeared spontaneously. Since it is rare for these distinctive features of pancytopenia and chromosomal abnormality to coexist, it is important to investigate the association. In the current study, we describe the first case of 5q35.2-q35.3 microduplication encompassing NSD1 with unexplained pancytopenia.
|All the work in this journal are licensed under a Creative Commons Attribution-NonCommercial 4.0 International License, which permits any non-commercial use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source.|