فهرست مطالب

International Journal of Hematology-Oncology and Stem Cell Research
Volume:7 Issue: 2, Apr 2013

  • تاریخ انتشار: 1392/02/24
  • تعداد عناوین: 8
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  • Mehrdad Payandeh, Zohreh Rahimi, Atefeh Nasir Kansestani, Shahrooz Hemmati, Mahnaz Aleyasin, Mohammad Erfan Zare, Zohreh Nouri, Amir Hossein Hashemian, Farzad Gohardehi Pages 1-5
    Menorrhagia is the most common symptom that is experienced by women with bleeding disorders. Von Willebrand disease (VWD) is the most common congenital human bleeding disorder that is manifested as a quantitative deficiency in Von Willebrand factor (VWF) or dysfunction of this factor. The frequency of VWD is similar in both men and women. However, VWD is more readily detected in women due to the presence of severe bleeding associated with menstrual cycles and childbirth. The present study was carried out to find the frequency of VWD, its types, and clinical features of the disease among women with menorrhagia who referred to the Hematology Clinic of the Kermanshah University of Medical Sciences. The study comprised 482 women with menorrhagia. After excluding patients with confounding factors, 56 (11.6%) patients were evaluated for inherited bleeding disorders. We detected 31 (55.3%) patients with VWD. Type 3 of VWD was the most frequent subtype (45.1%) followed in frequency by type 2, (32.2%), and type 1 (22.5%). In conclusion, our study indicated that menorrhagia can be the first symptom of VWD. Therefore, rare coagulation disorders should be considered in women with idiopathic menorrhagia, particularly in regions with high rates of consanguinity.
  • Roshanak Hasheminasab Zavarreh, Mohammad-Mehdi Zahmatkesh, Masood Vakili, Ali Shahriari-Ahmadi, Mohammad Ali Zohal, Mohsen Arabi, Alireza Mahmoudian, Abbas Gheisuri, Abdolhamed Kian, Ali Fahimi Pages 6-10
    Background And Aim
    Chronic obstructive pulmonary disease (COPD) is one of the major causes of morbidity and mortality in adults. Anemia is known as comorbidity in many chronic diseases that can increase morbidity and mortality of COPD. Recent studies have shown that anemia may be more prevalent than expected in COPD patients and can increase disabilities of COPD. In this study we have evaluated the correlation between anemia and the severity of COPD in patients referred to teaching hospitals of the Tehran University of Medical Sciences (TUMS), Tehran, Iran.
    Material And Method
    In this cross-sectional study the severity of COPD in 760 patients with dyspnea who referred to teaching hospitals of Tehran University of Medical Sciences and 96 stable COPD patients were categorize using a GOLD criteria from mild to moderate, severe and very severe. Anemia was determined as hemoglobin <13 g/dL in men and <12 g/dL in women, respectively. Demographic characteristics, spirometry parameters and laboratory findings were compared between anemic and non-anemic groups using Student t-test and regression tests (SPSS v.18 software).
    Results
    The Mean age of patients was 65 ± 13.07 years (59.4% male). Overall prevalence of anemia was 27% and there was no correlation between severity of COPD and anemia. Anemic patients were significantly older than non-anemic patients (71.1 ± 8.5 years vs. 65.4± 12.8 years; p=0.030). RBC count of anemic patients were significantly lower than non-anemic group (4.3 ± 0.5 vs. 5.02± 0.8 ×106/µL; p<0.001). Erythropoietin levels in anemic group was significantly higher than non-anemic group (16.33±2.43 vs. 10.22 ± 2.67 mu/ml; p<0.001) and there was a significant inverse correlation of hemoglobin vs erythropoietin (r= -0.8).
    Conclusion
    there was a high prevalence of anemia in COPD patients. Anemia can increase disabilities of COPD. Thus, treatment of anemia may improve quality of life in these patients. Further comprehensive studies are needed for determination of exact prevalence of anemia and its physiologic effects in COPD.
  • Mehrdad Payandeh, Mohammad Erfan Zare, Atefeh Nasir Kansestani, Shirin Falah Pakdel, Firuzeh Jahanpour, Hoshang Yousefi, Farzaneh Soleimanian Pages 11-16
    Background
    Transfusion services rely on transfusion reaction reporting to provide patient care and protect the blood supply. Unnecessary discontinuation of blood is a major wastage of scarce blood, as well as man, hours and funds. The aim of the present study was to describe the main characteristics of acute transfusion reactions reported in the 4 hospital of Kermanshah University of Medical Sciences (KUMS), Kermanshah, Iran.
    Material And Methods
    The study was carried out at 4 teaching hospital of Kermanshah University of Medical Sciences, Kermanshah, Iran over18 months from April 2010. All adult patients on admission in the hospitals who required blood transfusion and had establish diagnosis and consented were included in the study.
    Results
    In the year 2010 until 2012, a total of 6238 units of blood components were transfused. A total of 59 (0.94%) cases of transfusion reaction were reported within this 3 years period. The most frequent reactions were allergic reactions which presented with various skin manifestations such as urticarial, rashes and pruritus 29 (49.1%) followed by increase in body temperature of > 1◦C from baseline which was reported as febrile non-hemolytic transfusion reaction (FNHTR) 22 (37.2%). There were four patients who had pain at the transfusion site (6.7%) and hypotension (6.7%).
    Conclusion
    It is important that each transfusion of blood components to be monitor carefully. Many transfusion reactions are not recognized, because signs and symptoms mimic other clinical conditions. Any unexpected symptoms in a transfusion recipient should at least be considered as a possible transfusion reaction and be evaluated. Prompt recognition and treatment of acute transfusion reaction are crucial and would help in decreasing transfusion related morbidity and mortality, but prevention is preferable.
  • Ali Dehghanifard, Mohammad Shahjahani, Hamid Galehdari, Fakher Rahim, Fatemeh Hamid, Kaveh Jaseb, Asnafi Ali Amin, Mohammad Ali Jalalifar, Najmaldin Saki Pages 17-22
    Background
    Hemoglobinopathy and thalassemia are prevalent genetic disorders throughout the world. Beta thalassemia is one of these disorders with high prevalence in Iran, especially in Khuzestan province. In this study, the rate of different mutations in β-globin gene for prenatal diagnosis in fetal samples was evaluated.
    Materials And Methods
    In this experimental pilot study, 316 fetal samples (chorionic villus or amniotic fluid) suspicious to hemoglobin disorders were enrolled. Afterwards, DNA was extracted and PCR and DNA sequencing were used for evaluation of different mutations in β-globin gene.
    Results
    Amongst 316 samples evaluated for prenatal diagnosis, 180 cases (56.8%) were carrying at least one mutated gene of β-thalassemia. In addition, results showed that CD 36-37 (- T) and IVS II-1 (G>A) polymorphisms are the most prevalent polymorphisms of β-thalassemia in Ahvaz city with 13.9% and 10.1% rates, respectively.
    Conclusion
    Using molecular tests for prenatal diagnosis is considered an efficient approach for reducing the birth of children with hemoglobinopathy and identification of prevalent mutations in each region.
  • Leila Sayadi, Fateme Jafaraghaee, Alireza Jeddian, Mahboobe Kafaei Atrian, Azam Akbari, Farhood Tootoonchian Pages 23-29
    Background
    Today, hematopoietic stem cells transplantation (HSCT) has been accepted as a therapeutic approach and is widely applied in many patients with disorders of hematopoietic systems or patients with malignancies. Concomitant use of this therapeutic approach with long term chemotherapeutic procedures and hospitalization requires special care. This study was conducted to examine basic needs of patients after HSCT.
    Methods
    In this study, 171 hospitalized patients were selected after transplantation, using convenience sampling method. They completed a questionnaire formulated on the basis of Yura and Walsh Theory of Basic Needs.
    Results
    Most of the needs reported in the areas of vital functions, functional health status, and reaction to functional health status were chills (76.8%), insomnia (68.5%), and dissatisfaction with changes of lifestyle/habits (53.6%), respectively. Furthermore, 94.1% of the patients were aware of their disease.
    Conclusion
    This study identified a broad spectrum of the needs in HSCT patients. Given the importance of determining needs to reach a thorough nursing care, paying attention to the provided list can facilitate the achievement of the goals of the care program for these patients.
  • Mozaffar Aznab, Kaveh Kavianymoghdam Pages 30-33
    Myelodysplastic syndrome is a bone marrow failure in which differentiation and maturity do not happen naturally and dysplasia exists in each of 3 cell categories in Bone marrow. Refractory anemia is one of the major complaints with which the patients come to hematology clinics, which in diagnostic considerations lead to MDS as diagnosis. Often there is no recognized reason for this, so it is called “primary MDS”. In practice, we meet some patients who have MDS criteria however we can also find specific reasons for it; therefore we call it “secondary MDS”. One of the most important reasons for secondary MDS is the side effects of medications used in chemotherapy and radiotherapy in patients who undergo these therapies. We observed 6 patients in this case study during lengthy follow up that were diagnosed as MDS and during follow up period malignancy appeared in 6 cases. Supportive and therapeutic measures in these patients did not considerably improve blood cell count, most patients required blood injection and antibiotics for infection treatment. However align with malignancy treatment such problems are completely resolved both in terms of clinical and laboratory.
  • Majid Vafaie, Jaseb Kaveh, Majid Ghanavat, Mohammad Pedram, Tooran Rahiminia Pages 34-36
    Essential thrombocythemia is a rare myeloproliferative disorder in pediatrics. This myeloproliferative disorder is charactherized by thrombocytosis and hyperplasia of megakaryocytes in the bone marrow. Other cell lines are not involved.JAK2V617Fmutations has been identified in approximately half the patients with this disorder. We describe a 12-year-old boy with essential throbocythemia. The patient had a persistent thrombocytosis over 600x109 /L and the time of diagnosis, his platelet count ranged between 900x109and 2150x109/L. Megakaryocytes in the bone marrow were increased in number.The chromosomal analysis was normal and bcr/abl rearrangement was negative. He remained asymptomatic throughout the follow-up period.
  • Naser Aghamohammadzadeh, Afsaneh Faraji, Farshid Bozorgi, Ismail Faraji, Majid Moghadaszadeh Pages 37-41
    Adrenocortical carcinoma (ACC) is a rare tumor and usually a fatal disease which can develop at any age in either sex. Differential diagnosis between malignant and benign adrenal mass is not easy which leads to significant clinical challenge for optimal treatment. Here we report a 22-year-old woman diagnosed with primary hyper aldostronism initially but disease relapsed during six months and magnetic resonance imaging revealed tumor with metastasis to the liver.