Fikri Baslamisli

The frequency of anaemia, iron deficiency, hemoglobin S and beta thalassemia in the South of Turkey

A survey on the prevalence of anemia, iron deficiency, and hemoglobinopathies (Hb S and beta thalassemia) was carried out in an ethnically mixed and carefully registered population of 45,000 living in an area in the Southern Turkey. A total of 1223 subjects (representing the population with respect to sex and age group) were surveyed. The rate of anemia was found to be 16.9% overall with a highest prevalence of 18.3% in the children (0–2 ages) and 16.3% in the 14+ age group. The prevalence of iron deficiency using low transferrin saturation as criteria was found to be 17.2% overall, being 48.0% in the infant group, 19.6% in the children, and 14.7% in the 14+ age group. The rate of Hb S using the sickling test was found to be 3.9% in the entire population without separating for ethnic groups. It was more prevalent (9.6%) in the Arabic speaking and not found at all in the Kurdish speaking people. The rate of beta thalassemia with a high HbA2 was 3.4% overall and highest (7.1%) in the Kurdish speaking people.

Methylenetetrahydrofolate reductase gene C677T mutation and plasma homocysteine level in Behçet's disease

ObjectiveThe aim of this study was to assess whether homozygosity for the 5, 10-methylenetetrahydrofolate reductase (MTHFR) C677T mutation and plasma homocysteine concentration are related to deep vein thrombosis in Behçets disease (BD) patients.MethodsForty BD patients (23 males, 17 females; mean age 40.2±8.4 years) and 60 healthy controls (HC) (34 males, 26 females; mean age 41.6±6.9 years) were included in the study. Fourteen of the BD patients had a history of deep venous thrombosis (DVT), as confirmed by Doppler ultrasound.ResultsThe rates of homozygosity for the MTHFR C677T mutation in the BD and HC groups were 7.5% and 10%, respectively. The distribution of MTHFR genotypes was similar in the two groups (p>0.05), and analysis showed that homozygosity for the mutation was not a risk factor for DVT. The mean plasma homocysteine levels were 13.4±4.2 µmol/l for the overall BD patients and 12.6±3.8 µmol/l for HC (p>0.05). However, the mean plasma homocysteine level in the BD patients with DVT history (15.9±4.6 µmol/l) was significantly higher than the level in the BD patients with no DVT history (12.1±3.3 µmol/l) (p=0.013) and the level in the HC group (12.6±3.8 µmol/l) (p=0.025).ConclusionThe study results suggest that elevated plasma homocysteine level may play a role in the pathogenesis of venous thrombosis in BD.

Tissue doppler imaging and NT-proBNP levels show the early impairment of ventricular function in patients with beta-thalassaemia major.

ß-thalassaemia major is a chronic haemolytic anaemia, and congestive heart failure (CHF) is the most common cause of death in this disease. N terminal pro B type natriuretic peptide (NT-proBNP) increases with the severity of CHF and predicts the prognosis. The aim of this study was to investigate the relation between left ventricular systolic and diastolic function determined by standard pulsed wave Doppler (PWD), tissue Doppler imaging (TDI) and NT-proBNP in patients with ß-thalassaemia major. Thirty-four patients with ß-thalassaemia major and 34 healthy individuals were included in the study. Blood samples were taken for NT-proBNP. All patients and controls underwent echocardiographic examination. All cardiac chambers were significantly increased in the patient group. Left and right ventricular (LV, RV) ejection fractions and all diastolic parameters were normal in the patients and controls.Tissue Doppler imaging (TDI) showed a significant decrease in LV and RV Sm velocities in patients compared to the controls. NT-proBNP levels were also significantly higher in the patient group. There was a negative correlation between serum NT-proBNP levels and LV Sm and RV Sm velocities in patients (r=-0.426, P=0.006 and r=-0.409, P=0.009, respectively). Linear regression analysis showed that LV Sm and RV Sm were independent predictors for NT-proBNP. Our findings suggest that although iron overload in patients with ß-thalassaemia major impairs the systolic and diastolic functions of both ventricles, it impairs the systolic function earlier than diastolic function. Tissue Doppler imaging is an easy and reliable method in the early determination of ventricular dysfunction in these patients.

Vascular Endothelial Growth Factor (VEGF) Expression in Plasmacytoma

Vascular endothelial growth factor (VEGF) plays an important role in angiogenesis. Although the role and importance of angiogenic factors such as VEGF have been established in various solid tumors, this has not been widely evaluated in hemopoietic neoplasias. In this trial, VEGF was studied in plasmacytoma and VEGF expression was compared with histopathologic grade. Forty-seven samples have taken from cases with plasmacytoma (Pm) (33 bones and 14 soft tissue plasmacytomas) were used as study material. Pm was the initial presentation in all cases, and bone marrow (BM) involvement was detected in 19 cases with systemic evaluation. Twenty-seven of the cases were male (age range was between 19 and 81 years). Histopathologically 27 cases had mature and 20 cases had immature morphology. Immunohistochemical analysis was used to detect VEGF expression and this was scored according to the percentage of the VEGF stained cells. VEGF expression was detected in 32 cases and in eight cases this expression was strong. In 11 cases expression was moderate and 13 cases showed mild expression. When we compared VEGF expression with pathologic grade, 17 of 20 immature samples showed VEGF expression while 15 of 27 mature samples showed VEGF expression. There was a statistically significant association between immature morphology and VEGF expression (p <0.0264). Additionally all the samples, except one, with strong VEGF expression showed immature morphology. In conclusion two thirds of the cases with Pm showed VEGF expression and this was associated with immature morphology. Increased expression of VEGF was seen in plasmacytomas, and additional studies are needed to determine whether this translates to increased microvasculature or increased risk of progression to myeloma.

The Relationship between the VEGF Levels and VEGF mRNA Expression and Clinical Course in Different Glomerulonephritis

In this study, serum and urinary VEGF levels and VEGF expression in PBMNC were correlated with daily proteinuria, renal function tests, and renal histopathologic findings in untreated patients with different glomerulonephritis and with the course of renal function and proteinuria for one year. Forty-five untreated patients with different glomerulonephritis and 11 healthy persons comprised the study and control groups, respectively. VEGF mRNA expression was detected by RT- PCR in peripheral blood mononuclear cells (PBMNC), and VEGF levels were measured by ELISA in serum and urine samples simultaneously. Male/female ratio was 24/21 and mean ages were 34.49 ± 14.98. Serum and urinary VEGF levels, VEGF expressions in PBMNC, and the ratios of urine VEGF/urine creatinine were found to be similar in patients and controls. There were important correlations between urinary VEGF levels and baseline serum Cr (p = 0.035) and ESR (p = 0.022). There was also a marginal correlation between urinary VEGF levels and baseline CCr (p = 0.072). There was no correlation between serum and urinary VEGF levels and PBMNC mRNA expression and pathological findings such as with or without glomerular sclerosis, tubulointerstitial fibrosis (TIF), periglomerular fibrosis, and mesangial cell proliferation in renal biopsy. Serum and urinary VEGF levels or VEGF expression in PBMNC in patients with renal amyloidosis or proliferative or nonproliferative glomerulonephritis were similar with that of healthy controls and each other. Serum and urinary VEGF levels and PBMNC VEGF mRNA expression in untreated patients with different glomerulonephritis and controls were similar. We found only one important correlation, that between urinary VEGF levels and baseline serum creatinine levels in patients with different glomerulonephritis. Urinary VEGF can be an important pathogenesis of glomerular disease or a simple proteinuria. Serum and urinary VEGF levels and PBMNC VEGFmRNA did not change by periglomerular sclerosis, periglomerular fibrosis, or tubulointerstitial fibrosis on renal biopsy. PBMNC VEGF mRNA expression decreased in patients undergoing remission. In addition to the important correlation between urinary VEGF and serum creatinine, we also found an important correlation between erythrocyte sedimentation rate and urinary VEGF. This finding was interesting because we could not find a similar conclusion in other studies.

Budd-Chiari syndrome associated with visceral leishmaniasis and factor V Leiden mutation.

We here report a case of subacute Budd-Chiari syndrome (BCS) related to Factor V Leiden (FVL) mutation in the presence of visceral leishmaniasis. A 17-year-old man was admitted to hospital because of abdominal pain, pretibial edema and fever. The clinical picture of BCS had been developed within several months. BCS was diagnosed by radiographic examination. On DNA analysis, a heterozygote Arg506Gln mutation in the factor V gene was found. Histological examination of the bone marrow showed intracellular leishmania amastigotes. Despite appropriate treatment patient’s clinical condition deteriorated rapidly and died with multiorgan failure. FVL mutation is the most common procoagulant disorder and account for many cases of BCS. This case report demonstrates that in addition to duration and severity of the disease accompanying conditions including infections are prognostically significant for the outcome of this potentially lethal disease.

Late Onset Epstein Barr Virus Seropositive Posttransplant Lymphoproliferative Disorder in Two Renal Transplant Receivers

Posttransplant malignancy is one of the most important complications of organ transplantation. Immunosuppressive drugs, viral infections such as human herpes virus 8 or Epstein-Barr virus, exposure to carcinogenic factors such as sun, and host factors can be etiologic factors in the development of malignant disease. In this paper we report 2 cases of late posttransplant lymphoproliferative disorder with malign behavior. Conflict of interest:None declared.

Color doppler ultrasonography of renal vascular resistance in sickle cell disease: Clinical implications

The major complications in sickle cell disease (SCD) are microcirculation lesions and impairment of renal function. The aim of this study was to evaluate renal vascular resistance by means of color and duplex ultrasonography (CDU) and its relationships with hematological and clinical features in patients with SCD. Forty patients with SCD (mean age 22.4±7.0) and 14 age- and sex-matched healthy subjects (mean age 25.7±9.5) were included in the study. CDU of the main renal, segmental, and interlobar arteries was performed in both kidneys in all patients and controls. Peak systolic, end diastolic, and mean velocities through the entire cardiac cycle were obtained with further calculation of resistive (RI) and pulsatile (PI) indices. All the patients were investigated in stable state condition and had not been transfused within a month before investigation. Patients had a 6-month follow-up. Patients with SCD had higher RI and PI values than control subjects (p<0.0001,p<0.0001, respectively). Patients with high RI values (RI>0.70) had more pronounced disturbances of blood parameters (allp<0.05) than patients with normal RI values (<0.70). The significant positive correlation between RI and ISC number, MCHC level (r=0.52,p<0.001 and r=0.42,p<0.01), as well as RBC and Hgb level, correlated inversely with RIs (r=−0.39,p<0.01 and r=−0.42,p<0.01). During the follow-up, in nine patients (33.3%) with high RIs and one (5.5%) with normal RIs developed painful crises. In conclusion, renal vascular resistance, assessed by CDU is raised in SCD patients as compared with age-matched, apparently healthy persons. These changes are more pronounced in those with more severe manifestations of disease and correlated with hematological and clinical features of sickle cell disease.

Bencyclane as an anti‐sickling agent

A vasodilating Ca2+ channel blocker, bencyclane, was used in 18 patients with homozygous sickle cell anaemia (SCD) to test the possible anti‐sickling effect. With bencylane intervention the Na+‐K+ ATPase activity increased from 256±29 to 331±37 nmol Pi/mg protein/h (P<0.0001) and the Ca2+‐Mg2+ ATPase level increased from 172±12 to 222±44 nmol Pi/mg protein/h (P<0.0001). The intracytoplasmic Ca2+ concentration reduced from 3.5±0.6 to 2.7±0.25 μmol/l (P<0.0001). The patient’s blood contained fewer irreversibly sickled cells (ISCs) (a reduction from 21.4% to 14.4%) (P<0.05). At the same time MCHC of the erythrocytes decreased from 34.5 to 33.0 g/dl (P<0.05). Bencyclane appears to be a promising anti‐sickling agent that can be used orally in SCD.