REVIEW | |
1. | Fertility-Preserving Treatment Options in Patients with Malignant Hematological Diseases Mert Küçük, Ali Zahit Bolaman, Irfan Yavaşoğlu, Gürhan Kadıköylü doi: 10.5505/tjh.2012.72681 Pages 207 - 216 The number of patients of reproductive age diagnosed with various malignant hematological diseases increases every year. These patients undergo chemotherapy, radiotherapy, and various other treatments that may have gonadotoxic effects. The life expectancy of these patients is increasing rapidly due to the variety of treatment options. As such, an increasing number of patients—as well as their parents and spouses—express their concerns about the patient’s fertility post treatment. In the present review it was aimed to provide an overview of current fertility-preserving treatment options and the future of fertility preservation. |
RESEARCH ARTICLE | |
2. | Pulmonary Radiological Findings in Patients with Acute Myeloid Leukemia and Their Relationship to Chemotherapy and Prognosis: A Single-Center Retrospective Study Mehmet S Buğdacı, Halil Yanardağ, M. Cem Ar, Teoman Soysal, Süleyman Coşkun, Sabriye Demirci doi: 10.5152/tjh.2011.77 Pages 217 - 222 OBJECTIVE: Acute myeloid leukemia (AML) is the most common acute leukemia in adults. Pulmonary events are one of the commonest causes of mortality in AML. In this single center retrospective study aimed to evaluate the relationship between radiological findings of pulmonary at presentation and post chemotherapy on prognosis and clinical outcome in a group of AML patients. METHODS: The study included 278 AML patients. Clinical and radiological findings, laboratory findings, and microbiological culture results were evaluated. Pulmonary complications at presentation and post chemotherapy were compared. RESULTS: Pulmonary complications were observed in 53 of the patients (19%). Mean age of the patients with and without pulmonary complications was 43.1 ± 15.2 years and 38.8 ± 16.3 years, respectively (P < 0.001). Pulmonary complications were not correlated with gender, AML subtype, or the serum lactate dehydrogenase (LDH) level. The most common cause of pulmonary complications was infection. Pulmonary complications were observed in 29% and 71% of the patients at presentation and post chemotherapy, respectively. CONCLUSION: Pulmonary complications were observed more frequently at presentation in neutropenic AML patients of advanced age. The mortality rate was higher among the AML patients that had pulmonary complications at presentation. |
3. | Commentary Selami K. Toprak doi: 10.5505/tjh.2012.16013 Pages 223 - 224 Abstract | |
4. | Analysis of Chromosomal Aberrations and FLT3 gene Mutations in Childhood Acute Myelogenous Leukemia Patients Ender Coşkunpınar, Sema Anak, Leyla Ağaoğlu, Ayşegül Ünüvar, Ömer Devecioğlu, Gönül Aydoğan, Çetin Timur, Ahmet Faik Öner, Yıldız Yıldırmak, Tiraje Celkan, İnci Yıldız, Nazan Sarper, Uğur Özbek doi: 10.5505/tjh.2012.24392 Pages 225 - 232 OBJECTIVE: To identify the well-known common translocations and FLT3 mutations in childhood acute myelogenous leukemia (AML) patients in Turkey. METHODS: The study included 50 newly diagnosed patients in which t(15;17), t(8;21), and inv(16) chromosomal translocations were identified using real-time PCR and FLT3 gene mutations were identified via direct PCR amplification PCR-RE analysis. RESULTS: In all, t(15;17) chromosomal aberrations were observed in 4 patients (8.0%), t(8;21) chromosomal aberrations were observed in 12 patients (24.0%), inv(16) chromosomal aberrations were observed in 3 patients (6.0%), and FLT3-ITD mutations were observed in 2 patients (4.0%); FLT3-D835 point mutation heterozygosity was observed in only 1 patient (2.0%) patient. CONCLUSION: Despite of the known literature, our case shows a better survival and this might be due to the complementation effect of the t(15;17) translocation. The lower mutation rate (4%) of FLT3 gene seems to be one of the first results for Turkish population. |
5. | Comparison of Stored Umbilical Cord Blood and Adult Donor Blood: Transfusion Feasibility Rola Sahyoun-tokan, Saadet Arsan, Ömer Erdeve, Nuri Solaz, Aslıhan Avcı, Serenay Elgün-ülkar, Elif Gülyapar, Zeynep Üstünyurt, Zeynep Bıyıklı, Sabri Kemahlı doi: 10.5505/tjh.2012.94547 Pages 233 - 241 OBJECTIVE: This study aimed to compare the storage properties of red blood cell (RBC) concentrates of umbilical cord blood (UCB) and adult donor blood (ADB), and to evaluate the feasibility of UCB-RBC concentrate as an autologous source for blood transfusion in very low birth weight (VLBW) preterm neonates. METHODS: In all, 30 newborn (10 preterm, 20 full term) UCB and 31 ADB units were collected. RBC concentrates were stored and compared with regard to pH, potassium (K+), 2,3-biphosphoglycerate (2-3-BPG), adenosine tri-phosphate (ATP), plasma Hb, and bacterial contamination on d 1, 21, and 35 of storage. RESULTS: The K+ level increased with time and differed significantly between storage d 1 and 21, and between storage d 1 and 35 in both the UCB and ADB units. Initial and d 21 K+ levels were higher in the UCB units than in the ADB units. The 2,3-BPG level did not differ significantly between the UCB-PRC and ADB-PRC samples. After 35 d of storage both UCB-PRC and ADB-PRC samples exhibited significant differences from the initial free Hb, intracellular ATP, and pH values. Significant differences in intracellular ATP and pH were also observed between the UCB-PRC and ADB-PRC samples. CONCLUSION: The volume of harvested and prepared UCB-PRC can be used for some of the blood transfusions required during the neonatal period and thus may decrease the number of allogeneic transfusions, especially in preterm newborns. The hematological and biochemical changes that occurred in UCB during storage were comparable with those observed in ADB, and do not pose a risk to the immature metabolism of neonates. UCB-RPC prepared and stored under standard conditions can be a safe alternative RBC source for transfusions in VLBW newborns. |
6. | The Association Between JAK2V617F Mutation and Bone Marrow Fibrosis at Diagnosis in Patients with Philadelphia-Negative Chronic Myeloproliferative Neoplasms M. Cem Ar, Deram Büyüktaş, A. Emre Eşkazan, Şeniz Öngören, Eda Tanrıkulu, Zafer Başlar, A. Nur Buyru, Burhan Ferhanoğlu, Yıldız Aydın, Nükhet Tüzüner, Teoman Soysal doi: 10.5505/tjh.2012.58751 Pages 242 - 247 OBJECTIVE: Bone marrow fibrosis is the second most common complication causing morbidity and mortality in patients with Philadelphia negative myeloproliferative neoplasms (MPNs). The aim of this study was to investigate the association of JAK2V617F mutation with the bone marrow (BM) fibrosis at diagnosis in patients with MPNs. METHODS: In total, 149 patients with MPNs were retrospectively evaluated to determine if there was an association between the histological grade of bone marrow fibrosis and JAK2V617F mutation. RESULTS: In all, 67.7% of the patients carried the mutated JAK2 gene. The presence of JAK2V617F mutation was not associated with the occurrence of bone marrow fibrosis (P = 0.552) or its grade at diagnosis (P = 0.65). CONCLUSION: Molecular mechanisms or genetic defects other than JAK2V617F may underlie the occurrence of bone marrow fibrosis in patients with MPNs. |
7. | Does Reactive Thrombocytosis Observed in Iron Deficiency Anemia Affect Plasma Viscosity? Selami K. Toprak, İbrahim Tek, Sema Karakuş, Nihat Gök, Nazmiye Kurşun doi: 10.5505/tjh.2012.13008 Pages 248 - 253 OBJECTIVE: The accompanying thrombocytosis is referred to as the major factor associated with thromboembolism in iron deficiency anemia (IDA). Increased viscosity may increase the risk of thrombosis. We hypothesized that increased platelet count -with reactive thrombocytosis- might also affect plasma viscosity. We planned to evaluate the influence of normal and high platelet count on plasma viscosity in IDA patients. METHODS: The patient population consisted of fifty-three newly diagnosed and untreated women aged between 18 and 62 years with IDA. Group 1 consisted of 33 patients, platelet levels below 400 x 109/L. Group 2 consisted of 20 patients, platelet levels above 400 x 109/L. Measurements of plasma viscosity were performed using Brookfield viscometer. RESULTS: Mean plasma viscosity was found as 1.05 ± 0.08 mPa.s. in Group 1, and 1.03 ± 0.06 mPa.s. in Group 2. Mean plasma viscosity was not statistically different. White blood cell count was significantly higher in Group 2. Vitamin B12 levels were significantly higher in Group 2, while folic acid levels were higher in Group 1 (p=0.011 and p=0.033). Plasma viscosity was correlated with erythrocyte sedimentation rate (r=0.512 p=0.002) in Group 1 and inversely correlated with vitamin B12 (r=−0.480 p=0.032) in Group 2. CONCLUSION: Despite the significant difference between groups in terms of platelet count, no significant difference was detected in plasma viscosity and this finding could be explained as the following; 1-These platelets were not thrombocythemic platelets; 2-Similar to the theory about leukocytes, higher platelet counts – even non-thrombocythemic – may increase plasma viscosity; 3-Evaluating platelet count alone is not sufficient and the associating red-cell deformability should also be taken into account; and 4-Although other diseases that could affect viscosity are excluded, some definitely proven literature criteria such as fibrinogen, hyperlipidemia, and the inflammatory process should also be evaluated by laboratory and clinical measures. |
8. | Secondary Infections in Cancer Patients with Febrile Neutropenia Alpay Azap, Gülden Yılmaz Bozkurt, Meltem Kurt Yüksel, Hakan Kutlu, Pervin Topçuoğlu, Adalet Aypak, Hamdi Akan doi: 10.5152/tjh.2011.75 Pages 254 - 258 OBJECTIVE: Patients with neutropenia due to cancer chemotherapy are prone to severe infections. Cancer patients can experience >1 infectious episode during the same period of neutropenia. This study aimed to determine the etiological and clinical characteristics of secondary infectious episodes in cancer patients with febrile neutropenia and to identify the factors associated with the risk of secondary infectious episodes. METHODS: All cancer patients that received antineoplastic chemotherapy at Ankara University, School of Medicine, Department of Hematology between May 2004 and May 2005 and developed neutropenia were included in the study. Data were collected using survey forms that were completed during routine infectious diseases consultation visits. Categorical data were analyzed using the chi-square test, whereas Student’s t-test was used for continuous variables. Multivariate logistic regression analysis was performed to identify independent predictors of secondary infections (SIs). RESULTS: SIs were observed during 138 (53%) of 259 febrile neutropenic episodes. Of the 138 episodes, 89 (64.5%) occurred in male patients with a mean age of 40.9 years (range: 17-76 years). In total, 80% of the SIs were clinically or microbiologically documented. Factors on d 4 of the initial febrile episode were analyzed via a logistic regression model. The presence of a central intravenous catheter (OR: 3.01; P < 0.001), acute myeloid leukemia (AML) as the underlying disease (OR: 2.12; P = 0.008), diarrhea (OR: 4.59; P = 0.005), and invasive aspergillosis (IA) during the initial febrile episode (OR: 3.96; P = 0.009) were statistically significant risk factors for SIs. CONCLUSION: Among the cancer patients with neutropenia in the present study, AML as the underlying disease, the presence of a central venous catheter, diarrhea, and IA during the initial febrile episode were risk factors for the development of SIs. |
9. | Psychiatric Problems in Children and Adolescents with Sickle cell Disease, Based on Parent and Teacher Reports Özalp Ekinci, Tanju Çelik, Şule Ünal, Cahit Özer doi: 10.5505/tjh.2012.15986 Pages 259 - 264 OBJECTIVE: This study aimed to investigate the occurrence of psychiatric problems in children and adolescents with sickle cell disease (SCD). METHODS: The Child Behavior Checklist for ages 4-18 years (CBCL/4-18), Conners’ Parent Rating Scale (CPRS), Conners’ Teacher Rating Scale (CTRS-R), and The Turgay DSM-IV Based Child and Adolescent Behavior Disorders Screening and Rating Scale, clinician and parent forms (T-DSM-IV-S) were given to the caregivers and teachers of 31 children with SCD aged between 7-18 years and the caregivers and teachers of 34 age matched controls with irondeficiency anemia. RESULTS: The SCD patients had higher scores on all 4 of scales. Among the subscales, internalizing problems, and attention problems were more prominent in the SCD patients. CONCLUSION: Children and adolescents with SCD appear to have an increased risk for psychiatric problems. Regular psychological evaluation and referral to child and adolescent psychiatry clinics may facilitate timely diagnosis and effective treatment of at-risk children and adolescents. |
CASE REPORT | |
10. | Familial Hemophagocytic Lymphohistiocytosis with A665 G Perforin Gene Mutation: A Case Report Idil Yenicesu, Geneviève De Saint Basile, Hamdi Cihan Emeksiz, Buket Dalgıç doi: 10.5505/tjh.2012.62134 Pages 265 - 269 Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically heterogeneous disease. Presentation of the disease such as primarily fever, hepatosplenomegaly, and cytopenia, which are the results of functional degradation in cytotoxic T-lymphocytes and natural killer cells, activation of macrophages and T-lymphocytes, over production of proinflammatory cytokines, and hemophagocytosis. In all, 5 genetic loci have been identified in FHL, and all known affected genes encode critical components of the granule exocytosis pathway, which is essential for the release of cytotoxic granules and proteases that are necessary for targeted cell death. Herein we present an FHL patient with a severe clinical course and a very rare perforin gene mutation. The patient was homozygous for A665G mutation. However, the child died in a short period of time. Prenatal diagnosis was performed in the family and the fetus was found to be heterozygous for the mutation. |
11. | Treatment of Priapism with Automated Red Cell Exchange and Hyperbaric Oxygen in an 11-year-old Patient with Sickle Cell Disease Fatih Mehmet Azık, Avni Atay, Ahmet Emin Kürekçi, Hakan Ay, Yusuf Kibar, Okan Özcan doi: 10.5505/tjh.2012.78553 Pages 270 - 273 Priapism affects up to 50% of all males with sickle cell disease, and there is no standard treatment. Delayed and unsuccessful treatment leads to corporal fibrosis and impotence. It is therefore necessary to determine the best treatment methods for this complication in order to offer effective interventions to all affected patients. Herein we report an 11-year-old patient with sickle cell disease that presented with priapism 72 h after onset, and was successfully treated with automated red cell exchange and hyperbaric oxygen following unsuccessful surgical and conventional interventions. |
12. | Primary Breast Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma Transformation to Diffuse Large B-cell Lymphoma: A Case Report Şerife Hülya Arslan, Ümmügül Üyetürk, Emre Tekgündüz, Sultan Çiğdem Irkkan, Meltem Yüksel Kurt, Itır Şirinoğlu Demiriz, Fevzi Altuntaş doi: 10.5152/tjh.2011.80 Pages 274 - 277 Primary non-Hodgkin’s lymphoma (NHL) of the breast constitutes 0.04%-0.53% of all malignancies and 2.2% of extra nodal lymphomas. In total, 7%-8% of all B-cell lymphomas are the mucosa-associated lymphoid tissue (MALT) type, of which up to 50% of primary gastric MALT lymphoma. Herein we present a patient with breast MALT lymphoma that transformed to diffuse large B-cell lymphoma (DLBCL). A 69-year-old female presented with a mass on her left breast. Physical examination showed a 3 × 3-cm mass located 1 cm from the areola on the upper lateral quadrant of the breast at the 1 o’clock position, which was fixed and firm. Excisional biopsy was performed and pathologic examination of the specimen showed MALT lymphoma transformation to DLBCL. The patient was staged as II-EA. The rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) protocol was scheduled as treatment. Following 6 courses of R-CHOP, 2 additional courses of rituximab were administered. Positron emission tomography (PET)-CT was done at the end of the treatment. PET showed that the patient was in complete remission. At the time this report was written, the patient was being followed-up at the outpatient clinic on a regular basis. Lymphoma of the breast is a rarity among malignant tumors of the breast. The most common type of lymphoma is DLBCL. Breast MALT lymphoma is extremely rare. Primary MALT lymphoma of the breast can transform from low grade to high grade and recurrence is possible; therefore, such patients should be monitored carefully for transformation. |
13. | Myeloid Sarcoma: An Unusual Presentation of Acute Promyelocytic Leukemia Causing Spinal Cord Compression Tay Za Kyaw, Jayaranee A.s. Maniam, Ping Chong Bee, Edmund Fui Min Chin, Veera Sekaran Nadarajan, Hemalatha Shanmugam, Khairul Azmi Abd Kadir doi: 10.5505/tjh.2012.94809 Pages 278 - 282 Acute promyelocytic leukemia with concurrent myeloid sarcoma is a rare clinical event. Herein we describe a patient that presented with back pain and bilateral leg weakness caused by spinal cord compression due to extramedullary deposition of leukemic cells. Acute promyelocytic leukemia was suspected based on immunophenotypic findings of malignant cells in bone marrow aspirate. The diagnosis was confirmed by the presence of PML-RARα fusion copies. MRI showed multiple hyperintense changes on the vertebral bodies, together with intraspinal masses causing spinal cord compression. The patient immediately underwent radiotherapy, and was treated with all-trans retinoic acid and idarubicin. Reassessment MRI showed complete resolution of all intraspinal masses and the disappearance of most of the bony lesions. Post-treatment bone marrow aspirate showed complete hematological and molecular remission. The motor power of his legs fully recovered from 0/5 to 5/5; however, sensory loss below the T4 level persisted. |
14. | Hodgkin’s Lymphoma with Leptomeningeal Involvement Rahşan Yıldırım, Mehmet Gündoğdu, Fuat Erdem, İlhami Kiki, Betül Gündoğdu doi: 10.5152/tjh.2011.17 Pages 283 - 286 A 31-year-old male patient with Hodgkin’s lymphoma (HL) and leptomeningeal involvement presented to at Hematology Departments of Atatürk University. The patient developed dizziness, limb weakness, involuntary contractions of the hands and feet, incontinence, headache, fever, nausea, and vomiting following the first cycle of treatment with the ABVD protocol (adriamycin 25 mg m⎯2, bleomycin 10 mg m⎯2, vinblastin 6 mg m⎯2, and dacarbazine 375 mg m⎯2 on d 1 and 15 of a 28-d cycle). Magnetic resonance imaging (MRI) showed that there were regions with infiltration in particularly the thoracic vertebrae (T3, T4, T9, and T10-T12). In the lumbar region leptomeningeal involvement beginning at the end of the medulla spinalis that gave rise to a separation of nerves in the cauda equine was observed. Contrast agent uptake was observed, especially at the dural sac, with edema in the nerve roots. |
LETTER TO EDITOR | |
15. | First Observation of Hemoglobin M Saskatoon (ß63 (E7) His>Tyr(C-T)) in the Iraqi Population Nejat Akar, Çiğdem Arslan, Emin Kürekçi doi: 10.5505/tjh.2012.71542 Pages 287 - 288 Abstract | |
16. | HbA2-Yokoshima (delta 25(B7)Gly >Asp) and Hb A2-Yialousa (delta 27(B9)Ala>Ser) in Turkey Aylin Köseler, Ayfer Atalay, Erol Ömer Atalay doi: 10.5505/tjh.2012.50470 Pages 289 - 290 Abstract | |
17. | Spontaneous Subdural Hematoma Associated with Kasabach-Merritt Syndrome: A Case Report Ufuk Emre, Ayla Gökmen, Banu Özen, Enes Demiryürek, Şanser Gül, Dilvin Gökçe doi: 10.5505/tjh.2012.78736 Pages 291 - 292 Abstract | |
18. | Erythema Annulare Centrifugum and Relapsing Polychondritis Associated with Acute Myeloid Leukemia: A Case Report Selami K. Toprak, Sema Karakuş, Aysun Halaçoğlu, Efe Hasdemir, Halit Üner doi: 10.5505/tjh.2012.66563 Pages 293 - 296 Abstract | |
19. | Mirtazapine-Induced Thrombocytopenia and Neutropenia Selami K Toprak, Elçin Erdoğan, Özlem Kurt Azap doi: 10.5505/tjh.2012.72325 Pages 297 - 298 Abstract | |
20. | Non-secretory Multiple Myeloma in a Hemodialysis Patient with Hypercalcemia Fatih Mehmet Erdur, Yasemin Usul Soyoral, Habib Emre, Hüseyin Beğenik, Mehmet Taşdemir, Reha Erkoç doi: 10.5505/tjh.2012.02438 Pages 299 - 300 Abstract | |
21. | Bortezomib-Associated Severe Orthostatic Hypotension and Hyponatremia Elif Suyanı, Zeynep Akı, Zeynep Arzu Yeğin, Gülsan Türköz Sucak doi: 10.5505/tjh.2012.90377 Pages 301 - 302 Abstract | |
22. | Autoimmune Hemolytic Anemia Şinasi Özsoylu doi: 10.5505/tjh.2012.48568 Pages 303 - 304 Abstract | |
23. | Oral Findings and Inherited Disorders of Platelet Function Beuy Joob, Viroj Wiwanitkit doi: 10.5505/tjh.2012.23922 Pages 305 - 306 Abstract | |
24. | Extramedullary Plasmacytoma Presenting as a Mediastinal Mass: A Comment Selami K. Toprak, Sema Karakuş, Pervin Topçuoğlu doi: 10.5505/tjh.2012.24382 Pages 307 - 308 Abstract | |
25. | Megadose Methylprednisolone (MDMP) for the Treatment of Steroid Refractory Patient with Diamond-Blackfan (D-B) Anemia Şinasi Özsoylu doi: 10.5505/tjh.2012.24865 Pages 309 - 310 Abstract | |
IMAGES IN HEMATOLOGY | |
26. | EDTA-Dependent Pseudothrombocytopenia Aysenur Bahadır, Erol Erduran, Beril Dilber, Nalan Üçüncü doi: 10.5505/tjh.2012.60973 Pages 311 - 312 Abstract | |