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12th Congress of the European Hematology ... - Haematologica

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ly. Conclusions. 1. Combined intensive chemo<strong>the</strong>rapy (C–D, ESHAP or<br />

dexa-BEAM) for PMLBCL allow to achieve complete remission in most<br />

cases. 2. Surgical treatment is effective in local residual diseases eradicating.<br />

3. The radio<strong>the</strong>rapy should be conducted in purpose <strong>of</strong> consolidation<br />

<strong>of</strong> complete remission.<br />

1388<br />

VALUE OF GLYCOSYLATED HEMOGLOBIN (HBA1C) DETECTION ANALYSIS AS SCREENING<br />

METHOD FOR HEMOGLOBINOPATHIES<br />

S. de la Iglesia, J. López, R. Martin, P. Martin, M.T. Gomez Casares,<br />

C.E. Lopez Jorge, N. Navarro, T. Molero<br />

Hospital Negrin, LAS PALMAS DE G.C., Spain<br />

Backround. HbA1c determination is a usual way to control diabetic<br />

patients, because it provides a way to check <strong>the</strong>rapeutic adherence in <strong>the</strong><br />

last three months. This analysis is performed by high resolution liquid<br />

chromatography (HPLC), which in some cases shows peaks belonging<br />

to pathologic hemoglobins. Aim. The aim <strong>of</strong> this study was to review<br />

graphics obtained through HPLC to determine Hb1Ac in diabetic<br />

patients, in order to detect abnormal peaks which would be <strong>the</strong>n crosschecked<br />

by a HPLC hemoglobinopathies specific program, and see if<br />

<strong>the</strong>y belong to known pathologic hemoglobins. Materials and Methods.<br />

Chromatograms belonging to 1500 diabetic patients were checked. HA-<br />

8160 (A. Menarini Diagnostics) analyzer was used for HbA1c detection.<br />

This analyzer uses reversed-phase cation exchange chromatography column,<br />

and dual-wavelength colorimetry (415-500 nm) to carry out its<br />

measurements. Toge<strong>the</strong>r with HbA1c, this analyzer can also provide <strong>the</strong><br />

value for HbA0, HbF, and o<strong>the</strong>r abnormal peaks belonging to o<strong>the</strong>r fractions.<br />

The s<strong>of</strong>tware used is not able to correctly separate HbA0 from<br />

HbA2. All samples, extracted in EDTA tubes, that showed abnormal<br />

crests or >2% HbF values, were analyzed by HPLC with specific s<strong>of</strong>tware<br />

for <strong>the</strong> detection <strong>of</strong> β-thalassemias as well as o<strong>the</strong>r hemoglobinopathies<br />

(Variant. Bio-Rad. Beta Short Program). Results. From <strong>the</strong><br />

1500 analyzed chromatograms, 12 ( 0.8%) showed abnormal peaks,<br />

being <strong>the</strong>n cross-checked by VARIANT detecting; 1 HbD, high HbF in<br />

4 patients, 2 HbS, and 1 HbC. None <strong>of</strong> <strong>the</strong>se patients showed mycrocytosis<br />

or were previously known by <strong>the</strong> hematology department at our<br />

hospital. Conclusions. According to our results, it appears interesting to<br />

check chromatograms belonging to diabetic patients, because without<br />

any added cost, structural hemoglobinopathies without mycrocytosis<br />

could be detected. Although <strong>the</strong> diabetic populations is not <strong>the</strong> most adequate<br />

because <strong>of</strong> its median age for <strong>the</strong> screening <strong>of</strong> hemoglobinopathies,<br />

it could be very interesting to check pathologic chromatograms from<br />

<strong>the</strong>se patients in order to detect possible previously unknown hemoglobinopathies<br />

in order to perform family studies and genetic counseling<br />

when necessary.<br />

1389<br />

FAILURE OF PUBERTY IN EGYPTIAN β THALASSEMIC PATIENTS: EXPERIENCE IN NORTH<br />

EAST REGION - DAKAHLIA PROVINCE<br />

N. Abdelrazik, 1 H. Ghanem2 1 Mansoura University Children Hospital, MANSOURA, Egypt; 2 Mansoura<br />

Faculty <strong>of</strong> Medicine, MANSOURA, Egypt<br />

Background. Endocrine complications in thalassaemia major (TM) are<br />

classically considered to be <strong>the</strong> result <strong>of</strong> iron deposition in <strong>the</strong> endocrine<br />

glands. Hypogonatotrophic hypogonadism, which still remains <strong>the</strong> commonest<br />

endocrinopathy in patients with TM, has been proven to be <strong>the</strong><br />

result <strong>of</strong> hemosiderosis <strong>of</strong> <strong>the</strong> gonadotroph cells <strong>of</strong> <strong>the</strong> pituitary gland.<br />

The aim <strong>of</strong> <strong>the</strong> study. to evaluate <strong>the</strong> prevalence <strong>of</strong> delayed puberty and<br />

hypogonadotropic hypogonadism in transfusion-dependent patients<br />

with β-thalassemia major. Patient and Methods. Growth and sexual development<br />

<strong>of</strong> 40 patients with thalassemia major (20 males, 20 females)<br />

aged 12-22 years were evaluated. The following parameters were measured<br />

in every patient: age, sex, height, weight, Body Mass Index (BMI)<br />

and Tanner’s pubertal staging. For all patients, <strong>the</strong> following investigations<br />

were done: opthalomogical evaluation, audiograms, skeletal survey,<br />

echocardiography, serum ferritin, liver function tests, hepatitis pr<strong>of</strong>ile,<br />

serum calcium, phosphorus and blood sugar. Thyroid, parathyroid hormones,<br />

serum follicule stimulating hormone (FSH), luteinising hormone<br />

(LH), testosterone (T), and estradiol (E2) hormone were also measured.<br />

Results. Failure <strong>of</strong> puberty was present in 80% <strong>of</strong> boys and 75% <strong>of</strong> girls<br />

aged 12-22 years. Gonadotropin insufficiency was found in most <strong>of</strong> <strong>the</strong><br />

patients with lack <strong>of</strong> puberty. Arrested puberty was noted in five boys<br />

(25%) and six girls (30%). Ten girls (50%) did not menstruate, two (10%)<br />

had regular menstrual cycles, one (5%) had irregular menstrual cycles,<br />

12 th <strong>Congress</strong> <strong>of</strong> <strong>the</strong> <strong>European</strong> <strong>Hematology</strong> Association<br />

and two (10%) developed secondary amenorrhea. Using univariate<br />

analyses and stepwise logistic regression analysis after adjustment for<br />

confounding factors, serum ferritin at <strong>the</strong> time <strong>of</strong> <strong>the</strong> study was identified<br />

as an independent risk factor for hypogonadotropic hypogonadism,<br />

with an odds ratio <strong>of</strong> 28.40 (95% confidence interval 3.25-245.15), p=<br />

0.003 with a B value <strong>of</strong> 3.24 (standard error, 1.12). Conclusions. We conclude<br />

that failure <strong>of</strong> puberty are very common in our thalassemic patients<br />

which necessitates newer protocols <strong>of</strong> treatment, correct blood transfusion<br />

and chelation <strong>the</strong>rapy.<br />

1390<br />

THE EFFECTS OF LOSARTAN ON PLATELET AGGREGATION AND HEMATOLOGICAL PARA-<br />

METERS IN PATIENTS WITH NEWLY DIAGNOSED HYPERTENSION: (A CLINICAL STUDY)<br />

I. Yavasoglu, M. Unubol, G. Kadikoylu, Z. Bolaman<br />

Adnan Menderes University, AYDIN, Turkey<br />

Background. Angiotensin II receptor blockers have antiproliferative,<br />

antihypertensive and preventive effects from a<strong>the</strong>rosclerosis. There are<br />

controversial in vitro effects <strong>of</strong> angiotensin II receptor blockers on platelet<br />

aggregation in several experimental studies. In only one study, losartan<br />

decreased in vivo platelet aggregation with ADP, and ristocetin after 3<br />

weeks. Aim. To evaluate in vivo effects <strong>of</strong> losartan on platelet aggregation<br />

with ADP, collagen, epinephrine, ristocetin and o<strong>the</strong>r hematological<br />

parameters. Methods. Nineteen (13 patients female and 6 male, mean<br />

aged <strong>of</strong> 55±7 years) with newly diagnosed hypertension were enrolled<br />

to this study. They treated with 100mg/day losartan. Before <strong>the</strong> treatment<br />

and after 2 months, we analyzed complete blood cells parameters<br />

(Beckman Coulter) and platelet aggregation with collagen, epinephrine,<br />

ristocetin, and ADP (Chrono-log, 570 blood aggregation systems, 2 West<br />

Park Road, Haverton,PA, 19083-4691, USA). The values were analyzed<br />

using two-paired t test. Results. Before <strong>the</strong> treatment platelet aggregation<br />

with ADP, collagen, epinefrin, ristocetin, were 82±20%, 69±24%,<br />

93±15%, and 80±18.4%, respectively. After 2 months, <strong>the</strong>se rates were<br />

79.8±13%, 62.5±20%, 92±14%, and 63±25%. Although all parameters<br />

decreased, decrease in ristocetin was significant. (p=0.042). The levels <strong>of</strong><br />

hemoglobin (before 14±1.6 g/dL, after 13.4±1.3 g/dL) and hematocrit<br />

(before 40.2±5%, after 38.5±3.7%, p0.05). Conclusions Losartan treatment decreased<br />

platelet aggregation with ristocetin and <strong>the</strong> levels <strong>of</strong> hemoglobin and<br />

hematocrit. Losartan with <strong>the</strong>se hematological effects may be useful in<br />

<strong>the</strong> a<strong>the</strong>rosclerosis, inhibiting platelet aggregation and decreasing viscosity,<br />

in addition to antihypertensive effect.<br />

1391<br />

SREENING OF HB S IN THE REGIONAL HOSPITAL OF MALABO (EQUATORIAL GUINEA)<br />

P. Ropero, 1 L. Molina Esteban, 2 F.A. González, 1 M. Polo, 1 M. Mateo, 1<br />

C. Benavente, 1 R. Sánchez-Dominguez, 1 R. Paúl, 1 J. Raso, 2 A. Villegas1 1 Hospital Clínico San Carlos, MADRID, Spain; 2 Control de endemias de<br />

Guinea Ecuatorial, ISCIII, Spain<br />

Background. The sickle cell anemia is one <strong>of</strong> <strong>the</strong> genetic diseases more<br />

important in <strong>the</strong> world, it appears mainly in <strong>the</strong> black race constituting<br />

in a disease <strong>of</strong> high prevalence in some countries and originating serious<br />

problems <strong>of</strong> public health. The greater prevalence <strong>of</strong> <strong>the</strong> sickle trait is in<br />

Equatorial Africa in where until 40% <strong>of</strong> <strong>the</strong> population are carrying,<br />

being in direct relation with <strong>the</strong> zones in which falciparum malaria has<br />

been rampant; <strong>the</strong> disease reaches a prevalence <strong>of</strong> to 2-3%. Aims. To<br />

determine <strong>the</strong> incidence <strong>of</strong> Hb S in <strong>the</strong> population <strong>of</strong> Malabo (Equatorial<br />

Guinea). Methods. A total <strong>of</strong> 328 blood samples <strong>of</strong> umbilical cord and<br />

placenta were compiled in <strong>the</strong> regional hospital <strong>of</strong> Malabo during <strong>the</strong><br />

months <strong>of</strong> February and June <strong>of</strong> 2005. The conserved samples were ga<strong>the</strong>red<br />

on paper <strong>of</strong> filter and in methanol to 70% to 4º C. In <strong>the</strong> Service <strong>of</strong><br />

<strong>Hematology</strong> <strong>of</strong> <strong>the</strong> Hospital Clinico San Carlos <strong>of</strong> Madrid (Spain), <strong>the</strong><br />

samples were analyzed in <strong>the</strong> months <strong>of</strong> October <strong>of</strong> 2005 and February<br />

<strong>of</strong> 2006, being used for <strong>the</strong> analysis a VARIANT (Bio-Rad) with <strong>the</strong> program<br />

Sickle Cell Short Program. Results. Of <strong>the</strong> 328 compiled samples<br />

only 273 could be analyzed <strong>of</strong> which 208 (76.2%) were normal; 14,2%<br />

(39) displayed <strong>the</strong> sickle trait (Hb S/Hb A); 8 (2.9%) only have Hb S (Hb<br />

S/Hb S); 18 (6.6%) were carrying <strong>of</strong> some o<strong>the</strong>r hemoglobinopatia non<br />

S (Hb X/Hb A) and 55 samples (16.8%) were degraded not being able to<br />

be studied. Summary/Conclusions. Equatorial Guinea is a country located<br />

in <strong>the</strong> coast <strong>the</strong> west <strong>of</strong> Central Africa in <strong>the</strong> denominated zone <strong>of</strong> high<br />

malaria prevalence. It is divided in a continental part on <strong>the</strong> gulf <strong>of</strong><br />

Guinea and ano<strong>the</strong>r insular one. In <strong>the</strong> total population it is <strong>of</strong> 504,000<br />

inhabitants. The capital is Malabo in <strong>the</strong> island <strong>of</strong> Bioko. Considering<br />

haematologica/<strong>the</strong> hematology journal | 2007; 92(s1) | 499

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