Current practice of autologous hematopoietic progenitor cell mobilization in adult patients with multiple myeloma and lymphoma: The results of a survey from Turkish hematology research and education group (ThREG).

Autologous hematopoietic cell transplantation (AHCT) is an established treatment option for adult patients presenting with multiple myeloma (MM), Hodgkin lymphoma (HL) and various subtypes of non-Hodgkin lymphoma (NHL) in upfront and/or relapsed/refractory disease settings. Although there are recently published consensus guidelines addressing critical issues regarding autologous hematopoietic progenitor cell mobilization (HPCM), mobilization strategies of transplant centers show high variability in terms of routine practice. In order to understand the current institutional policies regarding HPCM in Turkey and to obtain the required basic data for preparation of a national positional statement on this issue, Turkish Hematology Research and Education Group (ThREG) conducted a web-based HPCM survey. The survey was designed to include multiple-choice questions regarding institutional practice of HPCM in adults presenting MM, HL, and NHL. The representatives of 27 adult HCT centers participated to the study. Here we report the results of this survey shedding light on the real-world experience in Turkey in terms of autologous HPCM mobilization strategies in patients presenting with MM and lymphoma.

Cholesterol Levels in Patients with Chronic Lymphocytic Leukemia.

Low cholesterol levels may be accompanied by solid tumors or hematological malignancies such as multiple myeloma. Decreased cholesterol levels have been reported in some experimental studies about chronic lymphocytic leukemia (CLL). It may be associated with tumoral cell metabolism. Herein, we examine blood lipid profiles of patients with newly diagnosed CLL (284 male, 276 female, mean age 64 ± 11 years) as defined by National Cancer Institute criteria. The control group consisted of 71 healthy subjects with mean age 55 ± 9 years (28 male, 43 females). 60% of patients with Binet A, while 25% were Binet C. Decreased levels of total cholesterol, high density lipoprotein (HDL) and low density lipoprotein (LDL) were observed in patients with CLL than control group (p < 0,001). There was no statistical significance between CLL and control group for triglycerides (TG) and very low density lipoprotein (VLDL), also between HDL-C, VLDL, TG and grades. Cholesterol may metabolized by abnormal lymphocytes in CLL patients.

H. pylori positivity and various pathological, endoscopic and clinical features correlated with each other.

OBJECTIVE: To investigate the relationship between dyspepsia symptom scores and endoscopic appearances, and histopathological findings and helicobacter pylori positivity in patients having dyspepsia symptom. METHODS: The study was conducted at the gastroenterology outpatient clinic of Adnan Menderes University, School of Medicine, Aydin, Turkey from April 2012 to July 2012 and comprised patients between 18-65 years of age who were admitted with dyspepsia. Glasgow dyspepsia severity scoring was done with questions posed orally to the patients. In histopathological evaluation of biopsy specimens according to Sydney criteria, chronic inflammation, activity, atrophy, intestinal metaplasia and helicobacter pylori parameters were used. Total number of eosinophils and number of mast cells were recorded. RESULTS: Of the 60 patients with dyspepsia, 38(63.3%) were female and 22(36.7%) were male. The degree of activation and severity of inflammation increased significantly with increasing helicobacter pylori positivity(r=0.459'p<0.0001; r=0.475'p<0.0001). A significant relationship was found between inflammation, activation and the number of mast cells (p<0.05).There was no relationship between helicobacter pylori intensity and the eosinophil count (r=0.171; p=0.093). There was also a statistically significant correlation between severity of inflammation and activation and the number of eosinophils (r=0.313;p=0.002;r=0.245;p=0.016). CONCLUSIONS: Mast cell density was seen to have a role in the inflammatory processes of helicobacter pylori infection.

Serum Bcl-2 Levels in Patients with ß-Thalassemia Minor: A Pilot Study.

OBJECTIVE: Anti-apoptotic proteins such as Bcl-2 and Bcl-xL may play a role in the survival of erythroid progenitor cells. Information about these proteins in patients with ß-thalassemia minor is limited. We aimed to determine the levels of serum Bcl-2 in patients with ß-thalassemia minor. MATERIALS AND METHODS: Ninety-seven patients (60 females and 37 males with mean age of 29±21 years) with ß-thalassemia minor were enrolled in this study. The diagnosis of ß-thalassemia minor was based on whole blood counts, family history, and HbA2 levels estimated by high-performance liquid chromatography. The control group comprised 23 healthy adults (17 females and 6 males with mean age of 58±9 years) without anemia. The levels of serum Bcl-2 were measured by enzyme-linked immunosorbent assay. Mann-Whitney U tests were used in statistical evaluation and p<0.05 was accepted as statistically significant. RESULTS: Although there was no statistically significant difference between patients with ß-thalassemia minor and the control group for the level of serum Bcl-2 (p>0.05), these levels were higher in ß-thalassemia minor patients than controls. CONCLUSION: There are damaged beta chains in ß-thalassemia minor. Therefore, it is expected that premature death of red blood cells may occur due to apoptosis. The mean age of the control group was higher than that of the ß-thalassemia minor group; this may be why Bcl-2 levels were higher in the ß-thalassemia minor group. It is known that older age constitutes a risk for increased apoptosis. Other proteins (Bad, Bax, etc.) and pathways [CD95 (Fas) ligand] associated with apoptosis should be evaluated in future studies including more patients.

The effect of insulin resistance on inflammatory response and oxidative stress in acute cerebral ischemia.

OBJECTIVE: Insulin resistance (IR) has effects on inflammation and oxidative stress which have importance in acute stroke. Our aim was to investigate the relationships between IR, inflammation, oxidative stress and stroke severity in acute ischemic stroke patients. METHODS: We examined the relationships between inflammation, oxidative stress and stroke severity in 75 acute stroke patients with and without IR. Serum levels of oxidative stress markers (nitric oxide (NO), malondialdehyde (MDA), glutathione (GSH)) were measured as well as the cytokines interleukin-6 (IL-6) and interleukin-10 (IL-10). RESULTS: The levels of IL-10 (13.7±19.11 vs 51.20±89.32 pg/ml, p<0.00) in IR group were significantly reduced. Patients with IR had higher levels of NO (30.26±17.63 vs 22.57±14.5 µmol/L, p=0.04) and IL6 (27.44±57.13 vs 8.68±11.8 pg/ml, p<0.00) and higher NIHSS scores (11.40±5.35 vs 8.81±5.76, p=0.04) when compared with noninsulin resistant group. IL-10 was found negatively correlated with HOMA. Additionally, the parameters with positive correlations with HOMA were NIHSS, IL-6 and NO. CONCLUSIONS: Inflammation and oxidative stress are more evident in acute stoke patients with insulin resistance which may cause worse stroke severity. Our data also suggest that IL-10 as an antiinflammatory cytokine can be much lower in insulin resistance in acute phase of ischemic stroke. However it can be elevated as an adaptive mechanism in metabolic syndrome as a chronic condition.

Knowledge, attitudes, and practices of hematologists regarding fertility preservation in Turkey.

OBJECTIVE: Fertility preservation stands before us as an issue of quality of life for cancer patients and their partners and families. Therefore, the object of the present study was to determine the extent of the knowledge that hematologists have about fertility preservation and to understand their attitudes and practices regarding this matter. MATERIALS AND METHODS: A total of 25 hematologists participated in a survey. The questionnaire included questions on sociodemographic characteristics and awareness concerning the subject of fertility preservation, as well as questions designed to determine the extent of the knowledge that hematologists had on the subject and to understand their attitudes and practices in this context. RESULTS: Of the participants in the study, all expressed their awareness of the adverse effects that the various treatments they were prescribing could have on fertility; 2 (8%) revealed that they had never heard of the concept of fertility preservation. Of the participants, 19 (76%) indicated that they did not have adequate knowledge about fertility preservation, but 22 (88%) fortunately expressed a need for acquiring more knowledge about the subject. Of the respondents, 23 (92%) said that they did not have any brochures or published resources on this subject and stated their belief that if hematologists did have such documents, they would have more opportunity to discuss the various fertility preservation options with patients. All of the participants in the survey supported the idea of the Turkish Society of Hematology publishing a guidebook on this subject and organizing a session on fertility preservation in their regular congress. CONCLUSION: Meeting the needs of hematologists for training and knowledge in the subject of fertility preservation and ensuring the development of appropriate attitudes and practices in this area is an important issue. The Turkish Society of Hematology may play a significant key role. CONFLICT OF INTEREST: None declared.

The single center registry for therapeutic apheresis in Turkey: 11-year activity.

Therapeutic apheresis (TA) is used as primary and adjunctive therapy in the treatment of several diseases and syndromes. We retrospectively evaluated the results of therapeutic apheresis (TA) including therapeutic plasma-exchange (TPE), double filtration plasmapheresis (DFPP), therapeutic thrombocytapheresis and leukocytapheresis as 11-year activity during 2000-2011. A total of 845 TA procedures were performed in 114 patients (67 male and 47 female, with mean age 51±17 years). Adverse events (AE) were seen in 8.6% of procedures. None of the patients died from any complication. TA is safely carried out in our center in several diseases which are similar to previous reports.

Thrombocytosis in rheumatoid arthritis: JAK2V617F-positive essential thrombocythemia.

Thrombocytosis is an important laboratory finding in rheumatoid arthritis (RA) and it has a correlation with disease activity. Janus kinase 2 valin 617 phenylalanine (JAK2V617F) mutation has gained importance in the diagnosis of myeloproliferative diseases recently. There is no published report in literature on the association between RA and JAK2V617F-positive essential thrombocythemia (ET). In this report, we present a JAK2V617F-positive ET case that had RA. A 57-year-old male patient was diagnosed with RA according to the criteria of American College of Rheumatology (ACR), whose complaint was of pain in the hands and morning stiffness lasting for about 2 h. The patient was evaluated for thrombocytosis because he was in remission and suffering persistent thrombocytosis under treatment. After excluding the causes of secondary thrombocytosis, bone marrow aspiration and biopsy was performed. On peripheral blood and bone marrow PCR examination, the patient was detected to be JAK2V617F positive heterozygously and diagnosed with ET. As a conclusion, mild-moderate thrombocytosis is frequent in RA; however, ET can be diagnosed by JAK2V617F evaluation in peripheral blood in thrombocytosis, especially when platelet count is more than 1 million/ml and when persisting thrombocytosis is detected in RA remission.

[Blastoschizomyces capitatus fungemia: three case reports and review of the literature]. / Blastoschizomyces capitatus'un Etken Oldugu Fungemi: Üç Olgu Sunumu ve Literatürün Gözden Geçirilmesi.

Blastoschizomyces capitatus is a rare fungal pathogen that may lead to fatal infections especially in immunosuppressive individuals. In this report, three cases of B.capitatus were presented. The patients were under treatment for acute myeloid leukemia and their blood cultures yielded B.capitatus. The patients clinical conditions deteriorated and they died despite amphotericin B treatment. The isolates were identified by conventional mycological methods and API 20C AUX (Bio-Mérieux, France) system. Antifungal susceptibility test of the strains was performed with Sensititre Yeast One Panel (Trek Diagnostic Systems, USA) and minimum inhibitory concentration (MIC) ranges for amphotericin B, caspofungin, fluconazole, itraconazole, voriconazole, posaconazole, and flucytosine were found as 0.5-1; > 16; 8-16; 0.5; 0.25; 0.5-1 and 0.06-0.25 µg/ml, respectively. Isolated strains were genotyped with RAPD-PCR (Random Amplified Polymorphic DNA-Polymerase Chain Reaction) using Cnd-3, Cnd-4, OPE-03, OPE-18 primers. The strains isolated from the first two cases were found to be genotypically identical, while the strain isolated from the third case was different. Genotypically identical isolates belonged to two patients who were admitted to the hospital with approximately 18 months interval. The other strain with a unique genotype, was isolated from a patient who was admitted to the hospital about two years later than the other two patients. In conclusion, B.capitatus should be considered as an important opportunistic pathogen especially in patients with hematologic malignancies. The data of this study demonstrated that the lowest MIC values for B.capitatus strains were with voriconazole.

Fertility-preserving treatment options in patients with malignant hematological diseases.

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.

Plasmacytoid dendritic cell tumor: A case report.

A 62-year-old man presented with a painless eruption on his arms and trunk. Physical examination showed 2 well-demarcated erythematous plaques on the anterior trunk and 6 purple-red papules on the back and upper extremities. Blood chemistry and computed tomography results were normal. Herein we describe a patient with plasmacytoid dendritic cell neoplasm in the absence of systemic symptoms.

Investigation of demographic features, performance, comorbidity status and mortality causes among multiple myeloma patients: real-life data.

BACKGROUND: The demographic characteristics, performance status, frequency of comorbidities and survival rate of patients with multiple myeloma (MM) show variability geographically and different risk scoring systems have been used to assess this population. Here, we present data from a Turkish cohort, focusing on identifying similarities and differences, relative to other reports in the literature. RESEARCH DESIGN AND METHODS: A total of 310 patients diagnosed with MM were enrolled. Their demographic characteristics were investigated retrospectively. For performance assessment; the ECOG-IMWG Myeloma Frailty Score, R-MCI and HCT-SCI scoring indexes were used. PFS and OS periods, as well as the causes of deaths, were determined. RESULTS: The mean age of all study participants was 65 ± 10 years. The mean PFS and OS periods were 24.14± 26.11 and 65.3 ± 4.4 months, respectively. The median R-MCI, CCI and HCT-CI scores were five, four and three points, respectively. Myeloma-related complications were the leading cause of death, with a frequency of 51%. CONCLUSION: Among the scoring systems utilised, R-MCI was more convenient to apply due to its ease of use and practicality. Our study supports the heterogeneous course of myeloma and highlights geographic differences including comorbidities, causes of death and overall survival.

Emergent therapy with therapeutic plasma exchange in acute recurrent pancreatitis due to severe hypertriglyceridemia.

Hypertriglyceridemia causes acute pancreatitis in 1.3-3.8% of patients. We report here on two cases with severe (triglyceride level >1000 mg/dL) hypertriglyceridemia-induced acute recurrent pancreatitis. Both patients had uncontrolled hypertriglyceridemia and suffered from acute pancreatitis. No cause of secondary hypertriglyceridemia was detected. While stage E pancreatitis (Ranson's score: 2) was diagnosed in the first case, stage D pancreatitis with a null Ranson's score was detected in the second case. Both patients were treated with classical treatment with fluid replacement, analgesic, antibiotics and discontinuation of oral intake. Therapeutic plasma exchange (TPE) with fresh frozen plasma was performed consecutively and with two procedures on the 2nd and 3rd day in the first case. After TPE, while the triglyceride levels decreased from 4408 to 302 mg/dL, the amylase levels dropped from 4234 to 171 IU/L. In the second case, TPE was performed once daily. After TPE, the levels of triglyceride and amylase decreased from 2210 mg/dL and 1618 IU/L to 154 mg/dL and 110 IU/L, respectively. Local and systemic complications due to acute pancreatitis were not observed. Clinical signs and laboratory values improved. At the two-year follow-up of both patients, acute pancreatitis had not recurred with regular fenofibrate treatment. Hypertriglyceridemia should be considered in patients with acute recurrent pancreatitis. Although there is no definitive evidence for early application of TPE in severe hypertriglyceridemia-induced acute pancreatitis yet, therapy with TPE may be of benefit, improving the clinical course.

Thrombotic thrombocytopenic purpura as the first manifestation of metastatic adenocarcinoma in a young woman.

Thrombotic microangiopathy occurs in 5-10% of patients with mucin-producing disseminated adenocarcinoma. A 28-year-old woman complained of fatigue, bone pain, and weight loss. There were pallor, icterus, and tenderness in the bones on physical examination. Microangiopathic hemolytic anemia, leukoerythroblastic picture, thrombocytopenia, and normal coagulation tests were detected. Thrombotic thrombocytopenic purpura (TTP) was diagnosed and therapeutic plasma exchange was performed on the patient. On day 5 a laparotomy had to be performed because of acute abdomen due to the rupture of a corpus hemorrhagicum follicle of an ovary. Signet ring cell adenocarcinoma stained with cytokeratin 7 and mucicarmine was seen on ovaries and bone marrow, after the pathological examination. The primary site of tumor could not be investigated, because of the patient's refusal. Although chemotherapy including cis-platinum, infusional 5-fluorouracil, and calcium leucovorin were administered in two courses, she died from respiratory failure. In conclusion, malignancy and bone marrow involvement should be considered when associated with leukoerythroblastic picture and TTP.

The relationship between insulin resistance and hypercoagulability in acute ischemic stroke.

AIM: Insulin resistance has effects on the coagulation system, which is important in the acute phase of infarct. We examined the relationships between insulin resistance, hemostatic markers and stroke severity in acute ischemic stroke patients. METHODS: Protein C (PC), protein S (PS), fibrinogen, von Willebrand factor and antithrombin III (AT III) were studied in 75 acute ischemic stroke patients with and without insulin resistance. RESULTS: The PC and PS levels of insulin-resistant patients were significantly lower than those of non-insulin-resistant patients (PC: 87 ± 19.23 vs. 97.89 ± 13.3%, p = 0.007; PS: 84.75 ± 15.72 vs. 93.21 ± 15.02%, p = 0.02), and both of the anticoagulants were correlated with the homeostasis model assessment (HOMA; r = -0.339, p = 0.003 and r = -0.481, p = 0.000, respectively). Additionally, the NIH Stroke Scale (NIHSS) score correlated negatively with PS (r = -0.329, p = 0.004) and AT III levels (r = -0.235, p = 0.04). The parameters with positive correlations with NIHSS were fibrinogen (r = 0.270, p = 0.019), fasting glucose (r = 0.358, p = 0.008) and HOMA (r = 0.286, p = 0.013). CONCLUSIONS: The significant associations between insulin resistance and hemostatic markers may be relevant to stroke severity by causing a procoagulant tendency in acute ischemic stroke.

Successful treatment of early relapse of ocular myeloma with bortezomib and steroid after autologous stem cell transplantation.

Autologous stem cell transplantation (ASCT) can prolong remission duration, overall and progression free-survival in multiple myeloma (MM). Ocular relapse is rare in MM. Here we present a patient with only ocular relaps and without evidence of bone marrow progression after ASCT. Ig A kappa myeloma, stage IIIA was diagnosed in a 53-year-old man, according to Kyle-Greipp and Durie Salmon. He was treated with three courses of VAD therapy. Then he received high dose melphalan (200 mg/m2), followed by the ASCT. After two months from ASCT, he had bilateral blurry vision, pain, redness in both eyes and diplopia. We detected 5 mm of right-sided proptosis by Hertel exophthalmometry (base 110, 20 mm right eye, 15 mm left eye). Ocular motility of oculus dexter (OD) was restricted in up and lateral gaze. He has diplopia in up gaze. His color vision was 7 of 12 in the right eye and 10 of 12 in the left eye with Ishihara plates. Best corrected visual acuity was 6/10 in the right eye and 7/10 in the left eye. Intraocular pressures were 19 mmHg for OD and 18 mmHg for oculus sinister (OS). Slit lamp biomicroscopy revealed subconjunctival hemorrhages superiorly and temporally in the right eye and bilateral conjunctival hyperemia with chemosis. Fundus examinations of both eyes were unremarkable. Computed tomography and magnetic resonance imaging of orbita revealed a right intraorbital extraconal soft tissue density mass that involved the lacrimal gland and lateral rectus muscle. Prednisolon 1mg/kg/day and bortezomib 1.3 mg/m2 were started (1, 4, 8, 11 days). Eye findings were recovered after one month. Ocular relapse should be considered if there are ocular findings after ASCT for MM. Bortezomib and steroid may be useful for ocular extramedullary relapse of MM (Fig. 2, Ref. 8).

Hereditary thrombophilic factors in stroke due to cerebral infarct.

BACKGROUND: The stroke is the third most common cause of all deaths. In new studies, the importance of hereditary thrombophilic factors on stroke is emphasized. The aim of this study is to determine the role of hereditary thrombophilic factors including factor V Leiden A1691G (FVL), prothrombin G20210A, and methylenetetrahydrofolate reductase (MTHFR) C677T gene mutations in patients with stroke because of cerebral infarct. METHODS: Twenty-four patients with stroke and 53 controls with risk factor for stroke were enrolled. Polymerase chain reaction was used to detect these mutations. RESULTS: Heterozygote FVL mutation in 2 (8.3%) patients and MTHFR mutation in 10 (41.7%) patients were detected. In the control group, there were 2 (3.8%) patients with heterozygote FVL mutation and 15 (28.3%) patients with MTHR mutation. Both FVL and MTHFR gene mutations were detected in 1 patient and 2 controls, respectively. Prothrombin gene mutation was not found in 2 groups. There were not statistically significant differences for all 3 mutations in-between 2 groups (P > 0.05). Odds ratios were 0.431 (0.074-2.504, 95% CI) for FVL mutation and 0.553 (0.221-1.381, 95% CI) for MTHFR mutation, respectively. CONCLUSION: Although our study group was small, hereditary thrombophilic factors might not be risk factors for stroke because of cerebral infarct.

Is Blastocystis hominis a new etiologic factor or a coincidence in iron deficiency anemia?

Iron deficiency anemia (IDA) is a frequent health problem. Gut parasites such as N. americanus and A. duodenale are known to cause blood loss, but the role of Blastocystis hominis is uncertain. In this study, 212 patients (193 female, 19 male, mean age 41 SD 15 yrs) with IDA were enrolled and 90 persons without IDA (78 female, 12 male, mean age 45 SD 17 yrs). Microscopic examination of stools for B. hominis using the native lugol method was done three times on each subject. If any specimen contained five or more cysts per x400 field, the person was considered positive. B. hominis was found in 48 out of 212 subjects with IDA (22.6%) and in five of 90 (5.6%) subjects without IDA. This difference is highly statistically significant (P < 0.001). Few subjects had other gut parasites and there was no statistical difference in the ir frequencies between IDA and non-IDA subjects. Blastocystis hominis may play a role in the development of IDA either on its own or in conjunction with some other agent.