The effect of serum lipid levels on peripheral blood hematopoietic stem cell levels.

INTRODUCTION: There are limited numbers of available retrospective studies on various hematological diseases treated with stem cell mobilization therapy. In the present study, we aimed to demonstrate the effects of serum lipid levels on peripheral blood CD34+ (PBCD34+) cell counts as well as the changes in serum lipid levels during stem cell mobilization process. METHOD: PBCD34+ cell counts were compared between hypercholesterolemic patients and healthy individuals. Additionally, total cholesterol (TChol), LDL-cholesterol (LDL-C), HDL-cholesterol (HDL-C), and triglyceride (TG) levels were measured from healthy donors who underwent stem cell mobilization, at different time points (prior to filgrastim [phase 1], prior to apheresis [phase II], and the first week following apheresis [phase III]. RESULTS: In the hypercholesterolemia group, the PBCD34+ cell count was found to be higher among patients with elevated LDL-C (2.6 ± 0.35/µL vs. 1.7 ± 0.17/µL, p = 0.003) and TChol (2.6 ± 0.34/µL vs. 1.7 ± 0.14/µL, p = 0.006) in comparison to the healthy controls. In the mobilization group, phase II HDL-C levels (35.3 ± 2.8 mg/dL) were found to be lower than both phase I (45.6 ± 2.1 mg/dL) and phase III (44.5 ± 2.6 mg/dL) (p = 0.007). Phase II TChol levels (183.5 ± 10.0 mg/dL) were lower than both phase I (216.8 ± 8.5 mg/dL) and phase III (212.2 ± 8.4 mg/dL) (p = 0.02). At phase II, there was an inverse correlation between PBCD34+ cell count and HDL-C (r = - 0.57, p = 0.003). DISCUSSION: Our results indicate that, while increased LDL-C level is the determinant of baseline PBCD34+ cell count, reduced HDL-C is the determinant of PBCD34+ cell count during mobilization process.

The Turkish experience with therapeutic plasma exchange: A national survey.

Therapeutic plasma exchange (TPE) is used to treat more than 60 diseases worldwide and has drawn growing interest. Little is known about the current situation of TPE activity in Turkey, so we developed a survey to obtain information about this timely topic. We collected data on TPE from 28 apheresis units throughout Turkey. We performed a total of 24,912 TPE procedures with 3203 patients over the past decade. Twenty years ago, the majority of procedures were performed for neurological and hematological disorders, and today, most TPE procedures are done for the same reasons. The only historical change has been an increase in TPE procedures in renal conditions. Currently, renal conditions were more frequently an indication for TPE than rheumatic conditions. Fresh frozen plasma was the most frequently used replacement fluid, followed by 5% albumin, used in 57.9% and 34.6% of procedures, respectively. The most frequently used anticoagulants in TPE were ACD-A and heparin/ACD-A, used with 1671 (52.2%) and 1164 (36.4%) patients, respectively. The frequency of adverse events (AEs) was 12.6%. The most common AEs were hypocalcemia-related symptoms, hypotension, and urticaria. We encountered no severe AEs that led to severe morbidity and mortality. Overall, more than two thirds of the patients showed improvement in the underlying disease. Here, we report on a nationwide survey on TPE activity in Turkey. We conclude that there has been a great increase in apheresis science, and the number of TPE procedures conducted in Turkey has increased steadily over time. Finally, we would like to point out that our past experiences and published international guidelines were the most important tools in gaining expertise regarding TPE.

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.

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 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.

The relationship between red cell distribution width and prognostic scores in myelodysplastic syndrome.

INTRODUCTION: The myelodysplastic syndrome (MDS) represents a group of hematopoietic neoplasms that is characterized by clonal hematopoiesis, cytopenia and abnormal cellular maturation. Red cell distribution width (RDW) refers to the variation degree of erythrocyte size and it is a reflection of anisocytosis. Higher values have been linked to adverse outcomes, such as increased mortality, vascular events, kidney and liver disease and demonstrated to harbor poor prognosis in solid and hematological malignancies. The RDW value can be used as a contributing parameter for MDS diagnosis, as well as its prognosis. In this study, we essentially aimed to demonstrate the correlation between the RDW and MDS prognostic indexes. MATERIALS AND METHODS: Ninety-four MDS patients at the Aydin Adnan Menderes University Hematology Division were included in the study. The correlations between the RDW and laboratory values (either lactate dehydrogenase, albumin, globulin or ferritin) and the RDW prognostic scoring indexes (IPSS, WPSS, IPSS-R and LR-PSS) were investigated. The PASW for Windows, version 21.0 (SPSS Inc., Chicago, IL, USA), was used for statistical assessment. A p-value below 0.05 was the cut-off for the statistical significance. RESULTS: The mean age of all the patients was 73 ±â€¯10 years. Patients were observed for 41.88 ±â€¯25 months. The mean RDW value for all cases was 15.5 ±â€¯2.39. We found a statistically significant difference of survival between RDW values below and above 15.5% (p = 0.016). A significant difference was also observed according to the prognostic scoring indexes (see below). CONCLUSION: An increase in RDW is probably related to dysplasia in the MDS and this constitutes a possible explanation for the poor outcome. Prognostic indexes might incorporate the RDW as a parameter in the future.

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.

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.

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).

The protection of the myocardium by amifostine against mitoxantrone-induced acute cardiotoxicity in rats.

OBJECTIVE: Amifostine (AMI) has been used for the prevention of doxorubicin-induced cardiotoxicity in several experimental and a few clinical studies. The aim of this study was to investigate the effects of AMI on lipid peroxidation, protective enzymes, and mitoxantrone (MITO)-induced acute cardiotoxicity in the rat heart using biochemical tests and histopathological examinations. METHODS: Thirty-six rats were divided into six groups (n=6 in each). Control rats were given intraperitoneal (i.p.) serum saline and AMI group rats were given 200 mg/kg AMI i.p. Rats received MITO-2.5 and 5 mg/kg i.p. in the MITO-2.5 and MITO-5 groups. AMI 200 mg/kg i.p. was administered 30 min. before the same doses of MITO in the MITO-2.5+AMI and MITO-5+AMI groups. RESULTS: The levels of cardiac enzymes such as creatinine phosphokinase-myocardial band and cardiac troponin T did not change. Malondialdehyde (MDA) levels increased in MITO groups compared to controls. Catalase and glutathione (GSH) levels in the MITO and MITO+AMI groups were higher than in controls. Superoxide dismutase and glutathione peroxidase levels were not different between MITO groups and controls. There was no difference in MDA levels between MITO+AMI groups and controls. Calcium deposition was not detected. The scores of fibrosis, apoptosis, inflammation, and degeneration in MITO groups were higher than in controls. The scores of fibrosis, degeneration and inflammation in MITO+AMI groups were lower. CONCLUSION: MITO caused lipid peroxidation and myocardial damage, and the myocardium increased catalase and GSH levels to prevent this damage. AMI can protect against MITO-induced acute cardiotoxicity, decreasing myocardial damage and lipid peroxidation.

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.

The spectrum of diseases causing fever of unknown origin in Turkey: a multicenter study.

OBJECTIVE: The purpose of this trial was to determine the spectrum of diseases with fever of unknown origin (FUO) in Turkey. METHODS: A prospective multicenter study of 154 patients with FUO in twelve Turkish tertiary-care hospitals was conducted. RESULTS: The mean age of the patients was 42+/-17 years (range 17-75). Fifty-three (34.4%) had infectious diseases (ID), 47 (30.5%) had non-infectious inflammatory diseases (NIID), 22 (14.3%) had malignant diseases (MD), and eight (5.2%) had miscellaneous diseases (Mi). In 24 (15.6%) of the cases, the reason for high fever could not be determined despite intensive efforts. The most common ID etiologies were tuberculosis (13.6%) and cytomegalovirus (CMV) infection (3.2%). Adult Still's disease was the most common NIID (13.6%) and hematological malignancy was the most common MD (7.8%). In patients with NIID, the mean duration of reaching a definite diagnosis (37+/-23 days) was significantly longer compared to the patients with ID (25+/-12 days) (p=0.007). In patients with MD, the mean duration of fever (51+/-35 days) was longer compared to patients with ID (37+/-38 days) (p=0.052). CONCLUSIONS: Although infection remains the most common cause of FUO, with the highest percentage for tuberculosis, non-infectious etiologies seem to have increased when compared with previous studies.

Chronic myeloid leukemia following the treatment of rectal adenocarcinoma.

Solid tumors may occur in 3% of the patients with chronic myeloid leukemia (CML). Philadelphia (Ph)-positive CML was diagnosed in a 66-year-old man upon a white blood cell count of 58.1 x 10(9)/L. He had no symptoms and physical findings 3 years after treatment for rectal adenocarcinoma. Imatinib mesylate 400 mg/day was started and white blood cell count was lowered to 5.7 x 10(9)/L in 1 month. The patient had received several chemotherapeutic agents such as 5-fluorouracil, irinotecan, raltitrexed, capecitabine, and oxaliplatin. In the literature, there are two reports on CML after the treatment of colorectal carcinoma. The possibility of a relationship between oxaliplatin and/or irinotecan and CML may not be fully excluded. In conclusion, hematological disorders such as CML may emerge in colorectal carcinoma and whole blood counts should be carefully checked. The possibility of a relationship between CML and the chemotherapeutic agents in colorectal carcinoma should be further evaluated.

Therapeutic plasma exchange for hypertriglyceridemia induced acut pancreatitis: the 33 cases experience from a tertiary reference center in Turkey.

BACKGROUND/AIMS: Hypertriglyceridemia (HTG) is the third most common cause of acute pancreatitis. In patients with severe HTG (TG level>1000 mg/dL), it may be beneficial to immediately lower the levels of triglyceride (TG) and chylomicrons. In this study, we present one of the largest case series on the use of therapeutic plasma exchange (TPE) for hypertriglyceridemia-induced acute pancreatitis (HTG-AP). MATERIALS AND METHODS: Overall, 33 patients who were admitted to our clinic for HTG-AP and underwent TPE between January 2007 and July 2017 were included in the study. Clinical data and outcomes and the reduction of triglyceride levels were examined retrospectively. RESULTS: The TG level decreased by 54.4%, and the total cholesterol level decreased by 52.1% after one TPE session. The TG decrease after the second TPE session was found to be 79.4%. There were 20 (60.6%) patients with mild acute pancreatitis, 10 (30.3%) patients with moderetaly severe acute pancreatitis, and 3 (9.1%) patients with severe acute pancreatitis based on the categorization according to the revised Atlanta criteria. Regarding local complications, the acute peripancreatic fluid collection was observed in 13 (39.4%) patients, acute necrotic collection was observed in 1 (3%) patient, walled-off necrosis was observed in 1 (3%) patient, and pancreatic pseudocyst was not observed in any patient. Mortality was not determined in patients with mild and moderately severe acute pancreatitis, and its rate was 33.3% in patients with severe acute pancreatitis. The overall mortality rate was 3%. No significant complications related to TPE were noted. CONCLUSION: TPE is a safe and helpful therapeutic treatment method for patients with HTG-AP and may be considered particularly in patients with severe acute pancreatitis.