2020 CiteScore: 2.6
Ardeshir Ghavamzadeh, MD.
Vol 11, No 4 (2017)
Background:Thalassemia syndromes are among prevalent hereditary disorders imposing high expenses on health-care system worldwide and in Iran. Organ failure represents a life-threatening challenge in transfusion- dependent β-thalassemia (TDT) patients. The purpose of the present study was to determine the frequency of organ dysfunctions among TDT patients in Sistan and Baluchistan province in South-East of Iran.
Materilas and Methods: Laboratory and clinical data were extracted from medical records as well as by interviews. Standard criteria were applied to recognize cardiac, gonadal, endocrine and renal dysfunctions. The collected data were analyzed using the SPSS statistics software (Ver.19).
Results: A total of 613 TDT patients (54.3% males and 45.7% females) were included in this study. The mean age of patients was 13.3± 7.7 years old. Cardiac events comprised the most encountered complications (76.4%), following by hypogonadism (46.8%), parathyroid dysfunction (22%), thyroid abnormalities (8.3%), diabetes (7.8%) and renal disease (1.8%). Hypogonadism comprised the most identified complication in patient <15 years old, while the cardiac complications were the most frequent sequela in patients >15 years old (P<0.01).
Conclusion: As cardiac events are significantly more common among TDT patients, close monitoring of the heart function is recommended for identifying patients with cardiac problems.
Background: Endocrinopathies and diabetes mellitus are prevalent in patients with beta-thalassemia major
Recently some studies demonstrate a link between low levels of serum zinc level and higher prevalence of diabetes. The aim of this study was to evaluate the glucose tolerance in patients suffered from beta-thalassemia major and determine the association of Homeostasis Model Assessment (HOMA) parameters with zinc status among these patients.
Materials and Methods: In this cross sectional study, clinical data of patients who were suffered from thalassemia major, aged≥10 years were collected. Serum ferritin concentration, fasting blood sugar, fasting blood insulin and serum zinc level were assessed after overnight fasting. Moreover, oral glucose tolerance test was performed. Homeostasis Model Assessment (HOMA-2) was used for calculating beta-cell function, insulin resistance and sensitivity for normoglycemic and pre-diabetic subjects.
Results: of the 163 patients diagnosed with beta-thalassemia major, 10%, 53% and 37% were diabetic, pre-diabetic and normal, respectively. Mean serum zinc concentration was equal to 18.90±10.93µg/dl, and it was not significantly different across diabetic, pre-diabetic and normal groups. Pre-diabetic patients had significantly lower beta-cell function compared to normal subjects (P=0.0001). An inverse relation was documented between beta-cell function on one hand and total units of blood transfusion and ferritin level on the other hand (r=-0.29, P=0.004 and r=-0.27, P=0.03, respectively). The analysis adjusted for multiple possible confounders showed that there is no significant association between HOMA parameters and serum zinc level.
Conclusion: Impaired glucose metabolism and low serum zinc level were quite common among our study participants. The findings of the study also signifies the substantial role of follow-up in early detection and appropriate treatment.
Peripheral T cell lymphomas are a heterogeneous group of post-thymic, mature lymphoid malignancies, accounting for approximately 10-15% of all non-Hodgkin's lymphomas. Hepatosplenic T-cell lymphoma (HSGDTCL) is a rare entity, which is characterized by primary extra nodal disease with typical sinusoidal or sinusal infiltration of the liver and the spleen, respectively by expression of the T cell receptor chain, and by a number of other frequent clinicopathologic features, including aggressive course of disease. Secondary involvement of liver by hematopoietic malignancies is much more common as compared to primary liver involvement. Primary involvement of liver by non- Hodgkin’s lymphoma (NHL) is documented and mostly DLBCL (diffuse large B cell lymphoma) type. But, T cell lymphoma primarily arising from liver is very rare. It occurred commonly in immunocompromised patients and prognosis is very poor. Here, we present two case reports of Hepatosplenic gamma-delta T-cell lymphoma (HSGDTCL) and both are immunocompetent patients. Liver biopsy from the mass and subsequent IHC (immunohistochemistry) were performed for the purpose of diagnosis, which were positive for LCA (leukocyte common antigen), CD2 and negative for CD5, CD20 and CD79a. First patient was a 63-year-old female with hepatitis C virus seropositivity presented with liver mass simulating hepatocellular carcinoma. Second patient was a 60-year- old male, chronic alcoholic patient, presented with liver mass and lytic bony lesion in pelvis. Both patients were managed with conventional CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisolone) and showed complete response after 4 cycles of chemotherapy. After completion of 6 cycles of chemotherapy, both patients remained under 6-month surveillance period for any recurrence of the disease.
Background: Development of locally-derived hematological reference intervals is necessary for improving the quality of health care and clinical trials. However hematological reference intervals are affected by several variables including age, gender and environmental factors. Therefore this study was conducted to determine the gender and age-specific hematological reference intervals of healthy Iranian blood donors.
Materials and Methods: Selected hematological indices of 394 healthy blood donor volunteers, donating blood at Tehran Blood Transfusion Center were analyzed. Hematological reference intervals, stratified by age and gender were compared. The results of current study were also compared with those of US population.
Results: There were significant gender-related differences for mean values of hematological indices, with males having higher mean values of RBC, HCG, HCT and MCV than females. While the mean of PLT and MCH were higher in women. Age-related differences for mean values of RBC and MCH were also significant. The comparison of reference intervals, stratified by both gender and age showed that RBC, HGB and HCT values were higher in males than females in all age groups. But MCH values of females in all age groups and WBC and PLT counts in females older than 30 years were higher compared to the males in the same age group. The results of this study showed some similarity with US population, with narrower intervals.
Conclusion: This study suggests that gender and age-specific, locally derived hematological reference intervals should be referred to, before interpretation of any laboratory test result.
Background: Patients with haematological malignancies are often hospitalized in protective isolation until full neutrophil recovery in order to prevent infections. This descriptive pilot study evaluate the level of isolation-related distress and the use of free time in a sample of Italian onco-haematological patients who were hospitalized in protective isolation.
Materials and Methods: Participants were 18 patients hospitalized in hematologic ward to receive induction therapy (n=12) or autologous stem cell transplant (n=6). They completed a self-report questionnaire before discharge.
Results: Participants reported a moderate level of isolation-related distress, anxiety, and boredom: the more the anxiety and the boredom, the more the distress (r=.77; P<.001), (r=.79; P<.001), respectively. The activities performed during isolation were: watching TV (72.2%), reading (55.6%), thinking (33.3%), surfing in Internet or using PC (33.3%), and playing games or making cross-words (16.7%). Participants who reported pessimistic thinking had higher isolation-related distress (P=.004) as well as anxiety (P<.001) and boredom (P=.001).
Conclusion: Haematology Units should support isolated patients in spending their time in recreational activities, allowing more contacts with immediate relatives and friends, providing free TV and Wi-Fi connection inside the room. In addition, patients should have to keep themselves physically active. Isolation-related distress could also be reduced by providing psychological support.
Acute myeloid leukemia (AML) is a group of hematological diseases, phenotypic and genetically heterogeneous, characterized by abnormal accumulation of blast cells in the bone marrows and peripheral blood. Its incidence rate is approximately 1.5 per 100,000 in infants younger than 1 year of age and 25 per 100,000 persons in octogenarians. Traditionally, cytogenetic markers are used to stratify patients in three risk categories: favorable, intermediate and unfavorable. However, the forecast stratification and the treatment decision for patients with normal karyotype shows difficulties due to the high clinical heterogeneity. The identification of several genetic mutations additional to classical molecular markers has been useful in identifying new entities. Nowadays, many different mutations and epigenetic aberrations have been implicated in the diagnostic, prognostic and treatment of AML. This review is focused on describing the most important molecular markers with implications for clinical practice.
Background: The aim of this study was to evaluate the protective role of short hydration against nephrotoxicity induced by cisplatin (CDDP).
Materials and Methods: Twenty-two patients (13 men and 9 women) under CDDP therapy were enrolled in this retrospective study between 2009 and 2014. The CDDP was given in 500 ml of isotonic solution, and before and after CDDP administration, the patients received 10mEq potassium chloride15% and 1gr magnesium sulfate in 1000 ml isotonic saline. Renal parameters were evaluated on the first day of each cycle of CDDP therapy.
Results: Median cumulative CDDP dose was 465 mg/m2. Based on renal parameters, the prevalence of CDDP-induced nephrotoxicity (CIN) was 22.7%, while no hypokalemia and hypomagnesemia were observed.
Conclusion: Short hydration accompanied with potassium chloride and magnesium sulfate may decrease the risk of CIN.
Background: There is conflicting data about the increased risk of pulmonary toxicity when granulocyte-stimulating factor (G-CSF) is given in combination with bleomycin. No clear consensus for management of patients with Hodgkin lymphoma (HL) who require G-CSF support exists. Our objective was to evaluate whether there is an increase in pulmonary toxicity in patients who receive bleomycin and G-CSF during treatment for HL.
Materials and Methods: We conducted a single-center retrospective analysis of patients with Hodgkin Lymphoma from January 2003 until July 2015. All patients who received at least 1 dose of bleomycin and followed at our institution were included. Patients were evaluated for pulmonary toxicity starting from the day of first dose of bleomycin until 1 year after initiation of bleomycin. Data on pre-identified risk factors for pulmonary toxicity were also collected.
Results: Fifty-four patients met inclusion criteria. Twenty-one patients received bleomycin alone, and 33 patients received bleomycin and G-CSF. There was no statistically significant (p = 0.50) difference in the development of pulmonary toxicity between the two groups. Crude hazard ratio for development of pulmonary toxicity in the bleomycin and G-CSF cohort was 1.58 (95% confidence interval, CI: 0.41-6.12). On multivariate analysis, the hazard ratio for development of pulmonary toxicity was 1.71 (95% CI: 0.43-6.81).
Conclusion: This study does not find evidence that the combination of bleomycin and G-CSF increases the risk for bleomycin- induced pulmonary toxicity. We recommend G-CSF use in HL patients receiving bleomycin when needed to maintain dose intensity.
Blood is considered to be precious because it is the basic necessity for health; our body needs a steady provision of oxygen, supplied via blood, to reach billions of tissues and cells. Hematopoiesis is the process that generates blood cells of all lineages. However, platelets are the smallest blood component formed in the bone marrow and play a fundamental role in thrombosis and hemostasis. Platelets contribute their hemostatic capacity via adhesion, activation and aggregation, which are triggered upon tissue injury, and these actions stimulate the coagulation factors and other mediators to achieve hemostasis. In addition, these coordinated series of events are the vital biological processes for wound healing phases. The aim of this review is to summarize and highlight the important pathways involved in achieving hemostasis that are ruled by platelets. In addition, this review also describes the mechanism action of platelets, including adhesion, activation, aggregation, and coagulation, as well as the factors that contribute to hemostasis and wound healing.
Extramedullary plasmacytoma as a mode of relapse in multiple myeloma (MM) is unusual. Current recommendations do not incorporate the routine use of 18-fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET/CT) imaging prior to haematopoietic stem cell transplant (HSCT) in MM. We report a case of relapsed MM with complete remission as per IMWG criteria. In the interim period, before the HSCT, the patient had localizing neurological signs and symptoms attributed to multiple extramedullary plasmacytomas. The uniqueness of this case is that this patient after complete marrow remission with no obvious external masses had unexpected, symptomatic multiple extramedullary plasmacytomas. This case illustrates the need for integration of FDG PET/CT imaging into routine pre-HSCT investigations in relapsed MM to prevent missing any asymptomatic extramedullary plasmacytomas.
Thrombotic thrombocytopenic purpura is a rare thrombotic disease characterized by episodes of thrombocytopenia and microangiopathic hemolytic anemia due to disseminated microvascular thrombosis. Thrombotic thrombocytopenic purpura was first described in 1924 by Moschowitz as a disease presenting with a pentad of signs and symptoms (anemia, thrombocytopenia, fever, hemiparesis and hematuria). Previous studies have described atypical manifestations of thrombotic thrombocytopenic purpura such as hemolysis, anemia and thrombosis.
Nowadays, the prevalence of Multiple Myeloma (MM) seems to have been increasing among young females. Here, we report that thalidomide is contraindicated in pregnant women diagnosed with MM and those desirous of subsequent pregnancy. In this case report, we compared the clinical response of Thalidomide-Dexamethasone therapy in a post-abortive woman with persistently elevated β-hCG levels due to retained products of conception, undergoing hysterectomy later. This case report underlines the clinical significance of age, the effect of Thalidomide-Dexamethasone therapy even after initial discontinuation and the response to high β-hCG levels.
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