Alloimmunisation during pregnancy in Greece: need for nationwide HDFN prevention programme.

AIM/OBJECTIVES: To access the incidence and specificity of maternal red blood cells alloimmunisation and its relevant clinical impact in Greece. BACKGROUND: The rate of alloimmunisation in pregnant women in Greece is unknown. MATERIALS/METHODS: We performed a 4-year study in two tertiary hospitals in Greece. Demographics, transfusion and obstetric history were analysed. Maternal alloimmunisation was detected with indirect anti-globulin test. RESULTS: We investigated 4368 pregnant women. Of which 3292 (75·37%) were Greek and 1076 (24·63%) were migrants. In 39 alloimmunised women, 41 alloantibodies were detected (0·89%). The incidence of alloimmunisation was 0·66% (22/3292) in Greeks and 1·76% (17/1076) in migrants (P = 0·01). Anti-D was the most frequent alloantibody (0·18%). Anti-D was more frequent in migrants; 5·76% compared to 0·56% in Greek RhD negative women (P = 0·002). Other antibody specificities in declining frequency rank were anti-K, anti-E, anti-Lea, anti-M, anti-c, anti-Ce, anti-Jka, anti-Jkb and anti-C. Primiparae vs para >2 and past history of blood transfusion were significantly associated with alloimmunisation during pregnancy (P = 0·0088, P < 0·0001, respectively). CONCLUSIONS: Our results depict differences in the delivery of health care between migrants and Greek women, as well as the heterogeneity in practices for the prevention of haemolytic disease of foetus and newborn in Greece and highlight the need for the implementation of nationwide guidelines.

Unusual development of an abdominal neoplasm in a patient with Maffucci's syndrome.

A case of Maffucci's syndrome complicated by a mixed abdominal tumor with ascites in a 23-year-old female patient is described. As far as we know, this is the first such case in the literature.

The effect of homocysteine on the clinical outcomes of ventilated patients with severe sepsis.

BACKGROUND: There is considerable evidence that elevated plasma homocysteine levels are associated with a prothrombotic milieu, whereas activation of the coagulation cascade is an important component of the pathogenesis of sepsis. The protein C pathway has been reported to play a central role both in the propagation of sepsis and a hyperhomocysteinemia-induced hypercoagulable state. Our primary aim was to measure plasma homocysteine levels in mechanically ventilated patients with severe sepsis/septic shock and to assess the association of these levels with relevant clinical outcomes. METHODS: The study cohort included 102 mechanically ventilated patients with severe sepsis or septic shock. Demographics, comorbidities, clinical data and severity scores were recorded. Plasma homocysteine, vitamin B12, folate, creatinine, and protein C levels were measured in all study subjects upon enrollment, and genotyping for the C677T and A1298C polymorphisisms of the methylenetetrahydrofolate reductase (MTHFR) gene and for factor V Leiden (FVL) mutations was performed as well. The primary outcomes were mortality at 28 and 90 days; secondary outcomes included the number of days without renal or cardiovascular failure and the ventilator-free days during the study period. RESULTS: Homocysteine levels were not significantly associated with any primary or secondary outcomes in the multivariable analysis. In addition, a synergistic effect of homocysteine with protein C levels was not detected. CONCLUSION: Our data suggest that plasma homocysteine levels may not inform the prognosis of mechanically ventilated patients with severe sepsis/septic shock.

Erythrocyte ferritin in patients with chronic renal failure and heterozygous beta-thalassemia.

AIM: The aim of this research is to study the variance of erythrocyte ferritin (EF) in patients with chronic renal failure (CRF) and heterozygous beta-thalassemia (beta-TA), as well as the use of EF as a more reliable index for assessing the body iron status. METHODS: We studied 63 subjects with CRF, 40 subjects with heterozygous beta-TA, 53 subjects with CRF and heterozygous beta-TA and 24 normal subjects. In 11 patients with CRF and heterozygous beta-TA, sternal bone marrow aspiration was performed to evaluate iron stores in the bone marrow. EF was determined in the hemolysate of washed erythrocytes by a radioimmunoassay. RESULTS: EF showed the strongest correlation with bone marrow iron (p < 0.001) in comparison with the remaining hematological parameters that were examined. Patients with CRF without heterozygous beta-TA showed an increase in serum ferritin (SF), even in cases of iron deficiency. In the group of heterozygous beta-TA without renal failure, 22.5% of patients showed an increased EF content up to 150 ag/cell and a tendency for iron overload. Patients with CRF and heterozygous beta-TA showed a high value of EF, up to 200 ag/cell, and iron overload in 22.6%, almost the same proportion as in the previous group. It was also observed that a high value of SF does not indicate iron overload for these patients. In the group of hemodialysis, patients without heterozygous beta-TA who were under erythropoietin (EPO) treatment presented iron deficiency. Many patients with CRF and heterozygous beta-TA who were taking EPO presented iron overload, while very few of them presented iron deficiency. CONCLUSION: These findings suggest that EF is a reliable index for assessing the iron status in patients with CRF and heterozygous beta-TA.

A study of beta-thromboglobulin and platelet factor-4 plasma levels in steady state sickle cell patients.

To evaluate the platelet function in sickle cell syndromes we measured the beta-thromboglobulin (beta-TG) and platelet factor 4 (PF-4) plasma values of 45 patients suffering from homozygous sickle cell anaemia (10) and sickle cell beta-thalassaemia (35) in steady state. The results were compared to those of 32 normal controls. Both the beta-TG and PF-4 levels were found to be significantly higher in patients than in controls but the beta-TG:PF-4 ratio was significantly lower in the patients group. This finding and the absence of any statistical correlation between platelet number and beta-TG or PF-4 indicate that platelets seem to be somehow activated in sickle cell syndromes, both in homozygotes and sickle cell/beta-thalassaemia heterozygotes. This platelet activation seems to exist even in steady state sickle cell disease patients, regardless of the functional status of the spleen.

Study of platelet function in patients with sickle cell anemia during steady state and vaso-occlusive crisis.

Although there are many reports providing evidence for platelet hyperactivity during acute infarctive crisis in sickle cell disease, little attention has been paid to the study of platelet release reaction in steady state. Plasma levels of the alpha-granule constituents beta-thromboglobulin and platelet factor 4 were measured in patients with sickle cell anemia during steady state and vaso-occlusive crisis in order to determine in vivo platelet activity. Significantly higher plasma levels of both proteins were found in steady state and in crisis when compared with those of normal controls. Platelet hyperactivity was also found to be more vigorous in painful crisis, suggesting a possible therapeutic role for drugs that inhibit platelet function.

Evidence for non-adaptive immune response in HIV infection.

Increased levels of soluble forms of adhesion molecules such as intercellular adhesion molecule 1 (ICAM-1) and E-selectin have been found in the sera of HIV-infected patients and have been associated with disease progression. The aim of the present study was to investigate whether this phenomenon reflects activation of the non-adaptive immune response in HIV infection. Fifty-one patients with HIV infection (42 men, nine women) were classified into two subgroups: those with HIV infection but without evidence of AIDS indicator conditions (HIV infected non-AIDS cases, n = 27) and those with AIDS (AIDS cases, n = 24). The activation of non-adaptive immune response was evaluated as the production of reactive oxygen species that cause lipid peroxidation, which was assessed by measuring thiobarbituric reactive substances (TBARS) using the thiobarbituric acid assay (TBA). Plasma levels of von Willebrand factor (vWF), measured by rocket immunoelectrophoresis, were used to show activation of endothelial cells even in the absence of any other causative agent, in these patients. TBARS levels in non-AIDS cases were significantly higher than in control subjects (n = 17) or AIDS cases (P < 0.001). The mean vWF levels were higher in AIDS cases than in non-AIDS cases or normal subjects (P < 0.05). TBARS levels remained significantly higher in non-AIDS cases after adjusting for age, CD4 T-cell and neutrophil counts, antiretroviral therapy and vWF plasma levels. The above findings indicate that in HIV infection, the virus per se is responsible for the increased oxidative stress that in turn activates various transduction pathways, may be leading to endothelial cell activation and shedding of adhesion molecules from the cell surface.