[Occupational and environmental risk factors for essential thrombocythemia: a case-control study]. / Fattori di rischio occupazionali e ambientali per la Trombocitemia Essenziale: uno studio caso controllo.

BACKGROUND: Essential Thrombocythemia (ET) is a chronic myeloproliferative disorder characterized by a long median survival, even though serious complications such as acute hemorrhagic or thrombotic events are not infrequent. Very few information are available about risk factors for ET because of lack of epidemiological studies focused on this topic. METHODS: We conducted a case-control study in order to analyze the possible association between ET and some occupational and environmental risk factors. A total of 93 patients enrolled in two hospitals in Turin (Italy) and 280 subjects randomly selected from the general population as controls, were included in the analysis. RESULTS: We found an association between ET and selected occupations. OR estimates suggest a significant association between ET cooks/waiters (OR 4.96, CI 1.59-16.9) and clerks (OR 2.63, CI 1.53-4.51) and a non significant association between ET and hairdressers (OR 5.10, CI 0.82-31.4), nurses (OR 3.75, CI 0.80-19.4), farmers (OR 1.74, CI 0.84-3.56), electricians (OR 1.42, CI 0.48-3.78), and photographers (OR infinity). Data did not suggest any strong association between environmental risk factors and ET. CONCLUSIONS: Selected occupations seem to be associated to ET. The association of ET with occupations as hairdressers, farmers and electricians could be attributable to known risk factors for other hematolymphopoietic malignancies such as hair dye, pesticides and magnetic fields. The association with other occupations is more difficult to explain, but all the results we presented are consistent with data from previous studies on onco-hematological diseases.

Predictive value of the early response to chemotherapy in high-risk stages II and III Hodgkin's disease.

A series of 60 patients with "high risk" Stage II and III Hodgkin's disease (B symptoms, or large mediastinal mass, or E lung disease) were staged without laparotomy and treated with combined modality treatment: mechlorethamine, vincristine, procarbazine, and prednisone (6 MOPP) plus radiotherapy. Patients were restaged after the first three courses of MOPP and the status of response to therapy at that time was called early response to chemotherapy (ERC). The rate of nitrogen mustard and procarbazine delivery (MRD) during the first three cycles of chemotherapy also was assessed. At the completion of the therapy patients were restaged and the final response was assessed. Fifty-two (86.7%) patients entered complete remission (CR). Forty-eight percent of the complete responders achieved CR in the first three courses of MOPP. Eight-year survival and disease-free survival (DFS) rates of the patients achieving CR were 71% and 73%, respectively. Survival and DFS were significantly better for the patients who achieved CR in the first three cycles of chemotherapy than for patients who entered CR at a later stage of therapy: 8-year survival 90% versus 55% (P = 0.00); 8-year DFS 87% versus 59% (P = 0.01). The attainment of a complete ERC was adversely affected by lymphocyte depletion (LD) histologic type (P = 0.01) and MRD less than 65% (P = 0.04). However, when a multivariate regression analysis was used, ERC was the only significant prognostic variable for survival and DFS and its predictive value was confirmed even after correction by MRD. These data suggest that the rapidity of response to chemotherapy could be an important prognostic factor in high-risk Stage II and III Hodgkin's disease.

Peripheral erythroid precursor and prognosis in chronic granulocytic leukemia.

Seventy-four consecutive patients with nonblastic chronic granulocytic leukemia (CGL) were observed from diagnosis and retrospectively studied. The patients were segregated into three risk groups according to the staging system proposed by Sokal et al. A significant difference in survival was observed only between Stage I and III (P = 0.01). The prognostic role of other variables, different from those considered in the Sokal et al. equation, was then investigated. Multiple regression analysis of data was made, by forcing into the Cox's regression model the Sokal et al. equation, while allowing the remaining variables to move in and out of the model. Only the presence of peripheral nucleated erythrocytes improved the significance (chi-square improvement = 4.565; P value improvement = 0.033). The evaluation of peripheral erythroid precursors is proposed for further implementation of the staging systems in CGL.

Organization of alpha-globin genes in Hb Hasharon (alpha 47 asp replaced by his) carriers.

Restriction enzymes analysis of the DNA from two unrelated Italian families with Hb Hasharon, a variant Hb (alpha 47asp replaced by his) frequently occurring in the Polesine area in Italy, indicates that this variant is associated to an alpha globin gene deletion. The alpha Hash genotype most likely results from a mutation on an alpha thal2 genotype.

gamma chain composition in five Italian newborns heterozygous for Hb F Malta G gamma-117 His leads to Arg.

The percentage of Hb F Malta G gamma-177 His replaced by Arg and the gamma chain composition have been evaluated at birth and at different times after birth in five Italian newborns heterozygous for this variant. The percentage of Hb F Malta ranged at birth from 24% to 31% of the total Hb F, while the average G gamma/A gamma chain ratio was about 7/3, overlapping the values observed in normal newborns. T gamma chains were detected in three out of five newborns, with a percentage of about 10% of the total Hb F. After birth the Hb F Malta declined faster than the total Hb F; thus, the Hb F decrease during the first 45 d of life is mainly due to the switch-off of the G gamma locus containing the Hb F Malta gene.

[A case of melanoderma erroneously considered to be addisonian]. / Un caso di melanodermia erroneamente considerata addisoniana.

A case characterized by a dark pigmentation of the skin with an initial hypotension and a lung tuberculosis in the remote anamnesis is described. The skin pigment was formed by lipofuscin and emosiderin, but only the former was found in the liver biopsy. Anyway, the pigment was not melanine and the surrenalic function of the patient was completely normal; therefore, an Addisonism syndrome is excluded. The patient was treated for a very long time with DOCA: this caused hypertension, probably supported by the hypervolemia triggered by the DOCA depending retenction of sodium and water. A sure diagnosis of the case was not made, but an Addisonism syndrome was certainly excluded.

Biosynthetic studies and gamma-chain composition in the Greek type of hereditary persistence of fetal hemoglobin and in its association with beta-thalassemia.

Hematological data, biosynthetic studies and gamma-chain structure of three heterozygotes for HPFH Greek type and of two heterozygotes for both HPFH and beta-thalassemia are reported. In the HPFH heterozygotes, hematological data were normal and globin chain synthesis balanced, while subjects carrying both HPFH and beta-thalassemia presented a thalassemic picture and the same degree of alpha/non-alpha-chain imbalance as the beta-thalassemia carrier belonging to the same family. The gamma-chain composition studies showed only the presence of Agamma-chains in HPFH; in the association HPFH/beta-thalassemia also some Ggamma and Tgamma were found. The mechanisms determining the high production of Hb F in the association HPFH/beta-thalassemia are discussed.