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The variation of natural killer (NK) cell activity and lymphocyte subsets 20 h after a single test dose of alpha-IFN, was studied in 17 thalassemic patients with chronic hepatitis C. All patients had suspended the alpha-IFN therapy at least 12 months before the study: 10 were considered responders and 7 nonresponders. Also NK cell cytotoxicity after in vitro incubation with alpha-IFN was studied. The administration of a single dose of alpha-IFN increased NK cell cytotoxic activity significantly in the group of responders and in non-responders; moreover the NK cell cytotoxic activity after alpha-IFN in vitro incubation increased both in responders and nonresponders, but to a lesser degree than in healthy controls. Absolute values of CD4+ and CD8+ lymphocytes decreased significantly only in responders. In conclusion, our data suggest that the variation of NK cytotoxic activity and lymphocyte subsets after a test dose of alpha-IFN can be considered a parameter related to IFN biological effects.

During the recent H1N1 pandemic, children with Sickle Cell Disease (SCD) experienced more hospitalizations and more complications than the general pediatric population. We performed a retrospective multicenter survey at 9 Pediatric Haematology-Oncology Units across Italy. H1N1 admission rate was 5.2%, with all admissions occurring before vaccine availability. Length Of Stay (LOS) was 6.06 days (7.85 for Acute Chest Syndrome), longer than in other countries. Vaccination coverage was not homogeneous, ranging from 0 to 99%; several family-related and health-system related barriers in accessing vaccinations were identified that should be ameliorated to improve coverage in this high risk group of children.

alpha-interferon (alpha-IFN) has been used to treat chronic non-A non-B hepatitis in thalassaemic patients with response rates from 45% to 83%. Unfortunately, treatment with alpha-IFN is associated with side-effects which have a negative effect on the quality of life of the patient. Therefore it would be useful if we could distinguish in advance those patients who would benefit from such therapy from those who would not. In the present study we found that the modification of lymphocyte subsets 20 h after the administration of the first dose of alpha-IFN revealed that relative numbers of T helper lymphocytes (CD4+) increased in three non-responding patients and decreased in five responding patients, whereas those of T suppressor lymphocytes (CD8+), and natural killer cells (CD57+, CD16+) decreased in non-responding patients and increased in responding patients. Therefore analysis of the lymphocyte subsets CD4, CD8, CD57 and CD16 before and 20 h after the administration of alpha-IFN can be used to predict the clinical response to treatment with alpha-IFN.

Children with acute lymphoblastic leukemia (ALL),the most common childhood malignancy, usually presentat diagnosis with signs of bone marrow failure. Pallor,fatigue, anemia, fever, neutropenia, and thrombocytope-nia are frequent signs. Skeletal manifestations on imagingstudies are found in 20–36% of cases [1,2]. These includemetaphyseal bands, periosteal new bone formations, lyticlesions, osteosclerosis, osteopenia, and mixed osteolysisand sclerosis [1–3]. Generalized osteopenia and vertebralcomplications have, however, reported in ALL [4]. Ourexperience with such a child is of interest.Shewas an 8-year-old female, whowas admitted to ourCenter because of anemia, leukopenia, and thrombocyto-penia. Three months earlier, she had experienced a sharpback pain and for this reason was admitted to a localhospital. Radiography and computed tomography of herspine showed collapse of L2, and diffuse severe osteo-porosis.Laboratorydatawerenormal,exceptforthebloodsedimentation rate (62 mm/hr). A working diagnosis ofjuvenilerheumatoidarthritiswasentertained,andaplastercast was applied for a month. When it was removed,the child continued to suffer from diffuse pain and haddifficulty in walking. For this reason, physiotherapy wasadministered for 2 months but her general condition wor-sened;diffusebonepainforcedhertostayinbed.Becauseof an altered blood cell count (hemoglobin 8.5 g%; whiteblood cell 3,000/mm

(1991). Six Rare Hemoglobin Variants Found in Sicily. Hemoglobin: Vol. 15, No. 5, pp. 431-437.

The population structure of two alleles with similar characteristics, HbS and beta-thalassemia, was studied in the same area in Sicily. It was observed that beta-thalassemia has uniform frequencies over the island, and that HbS shows a South-North cline. A singularity exists for HbS in the town of Butera, where the frequency of the S gene is six times higher than the island average. It was suggested that HbS entered Sicily from Greece (or directly from North Africa) when the beta-thalassemia allele was already established in the Island.

Recently, an association between Helicobacter pylori (HP) and iron deficiency anemia (IDA) was proposed. We describe 9 pediatric patients with a history of long-standing IDA and HP infection. After HP test results were confirmed to be positive, anti-HP therapy consisting of omeprazole, clarithromycin, and amoxicillin was administered for 2 weeks. The hematologic profile and iron status were assessed before and periodically after the end of the eradication regimen. The eradication of HP was associated with stable normalization of iron stores. HP infection may be involved in cases of IDA of unknown origin, and the eradication of HP is associated with the resolution of anemia.

Sicily, at the center of the Mediterranean, has been the meeting place of Eastern and Western civilizations, and in the Sicilian population the presence of many different alterations in the globin gene clusters can surely be considered testimony of past colonizations. From 1975 to 1994, 100,000 Sicilian subjects were screened by us to monitor the presence of hemoglobin (Hb) structural variants. In this paper we present the data gathered, emphasizing the high incidence (2.5%) of carriers of at least one abnormal Hb, and the great heterogeneity of globin molecular defects on the island. Twenty-six different mutations were identified: the most common occur in the beta-globin gene (beta(S), beta(C), deltabeta(Lepore), beta(G-San Jose), beta(O-Arab), but also quite frequent is the mutated allele alpha(J-Oxford). The chromosome haplotypes associated with some of them were characterized. Two uncommon Hbs, Copenhagen and D Punjab, and some 18 rare variants complete the wide spectrum of structural alterations of globin genes in Sicily. We think they are de novo mutations prevalently. It is not possible to exclude that the presence of a few of them is related to migratory phenomena, particularly from North Africa and East Asia. Numerous thalassemic alleles complete the picture of globin gene mutations in Silicy.