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Background: Ablation of the normal hepatic parenchyma surrounding the tumor (ablative margin [AM]) is necessary to prevent local tumor progression. Purpose: To assess the prognostic value of the ablative margin in patients with HCC ≤4 cm treated with US-guided laser ablation. Material and Methods: A cohort of 116 patients (53 women and 63 men, age range 42-82 years) with 132 HCC nodules ≤4 cm completely ablated by US-guided laser ablation was retrospectively analyzed. Rates of local tumor progression were compared using different ablative margin cut-offs (≥2.5, 5.0, 7.5, and 10.0 mm). Survival probability curves were obtained with the Kaplan-Meier method. Results: The mean period of follow-up was 42 months (range 3-114 months). Local tumor progression was identified in 24 out of 132 lesions (18%), with an average time to progression of 24 months (range 6-36 months). A significant difference in local tumor progression was observed only if the ablative margin was ≥7.5 mm (7% vs. 23%, P = 0.020). Survival curves of patients with or without an ablative margin ≥7.5 mm were not different (P = 0.665; mean survival time 43.8 ± 3.1 and 46.8 ± 6.1 for an AM < or ≥7.5 mm, respectively). Conclusion: An ablative margin ≥7.5 mm turned out to be useful in preventing local tumor progression but did not affect long-term survival in patients with HCC ≤4 cm treated with laser ablation. [ABSTRACT FROM AUTHOR]

Background: Hyperhaemolytic transfusion reaction (HHTR) has been well described in patients with sickle cell disease (SCD). It is characterised by a decrease in haemoglobin concentration to levels below those before transfusion and a fall in the absolute reticulocyte count. As red blood cells (RBC) alloantibodies are typically not detected in post-transfusion samples in acute forms of HHTR, we have previously proposed that both the transfused and autologous RBCs cells (HbSS/reticulocytes) are destroyed by activated macrophages. Case reports: We report a patient with SCD who presented with vaso-occlusive sickle cell crisis and developed a severe HHTR attributable to anti-Fy3. In addition to the usual supportive measures, the patient was treated with intravenous immunoglobulin (IVIG) and steroids. Serum ferritin levels were measured as an aspecific marker of macrophage activation. Results: Steroids and IVIG were effective in managing HHTR. Ferritin levels were high at the time of haemolysis, (>10000 µg L−1) whereas recovery and cessation of haemolysis correlated with a decrease in ferritin levels. Conclusion: Serum ferritin values >10 000 µg L−1 are considered pathognomic for conditions characterised by abnormal macrophage activation. In our case, serum ferritin levels correlate well with the disease activity and clinical response. This further supports our previous proposal that the activated macrophages play an important role in HHTR. Serum ferritin is a nonspecific marker of inflammation. A rapid specific bio-marker to measure the activity of macrophages in SCD in HHTR is desirable, and this area warrants further investigation. [ABSTRACT FROM AUTHOR]

Primary breast lymphoma (PBL) is a rare form of extranodal non-Hodgkin's lymphoma (NHL), whose own specific biological characteristics still need to be fully defined. No significant prognostic factor has been found and the optimal therapeutic strategy is uncertain. However, an intensified systemic therapy has been advocated to prevent relapse, even in patients who show a complete response to local treatment. We report two cases of primary diffuse large B-cell breast lymphoma, review the literature about this topic, and discuss treatment options. We conclude that differential therapeutic strategies based on the risk of relapse associated with the International Prognostic Index (IPI) are a reasonable way to approach PBL, and can avoid undue toxicity deriving from treatment. [ABSTRACT FROM AUTHOR]

The therapeutic activity and toxicity profile of gemtuzumab ozogamicin were assessed in 40 patients >60 years of age with acute myeloid leukemia (AML) who were not considered eligible for conventional chemotherapy because of advanced age or poor performance status. The drug was administered at the dose of 9 mg/m2 as a single 2-h i.v. infusion on days 1 and 15. Patients who achieved a complete remission (CR/CRp) were to receive a consolidation with two additional injections of the immunotoxin at the same dose. The overall CR/CRp rate was 17% (95% CI, 8-32%). The CR/CRp rate in patients 61-75 years old was 33% (6/18), and 5% (1/22) in patients older than 75 years. Induction death occurred in seven patients (17%), all aged above 75 years. Overall survival was significantly longer in patients aged 61-75 years than in older individuals (P=0.05), and in CD33+ cases than in CD33- cases (P=0.05). We conclude that the dose/schedule of gemtuzumab ozogamicin used in this trial is too toxic in the age group over 75 years. For these patients, additional studies with reduced doses of the immunotoxin are warranted. [ABSTRACT FROM AUTHOR]

Sjögren's syndrome (SS) is a systemic autoimmune disease that mainly affects exocrine glands. A diagnosis of SS in its early stages has a potential clinical relevance, but it is difficult and cannot be made solely on clinical grounds. Several sets of diagnostic criteria have been proposed, but none has met with a general consensus. Minor salivary gland has been judged to be the “gold standard” for the diagnosis of SS. However, it is a painful procedure and has a small but significant proportion of both false positive and false negative results. The aim of our study was to develop a simple mathematical score that uses clinical and laboratory variables for diagnosing SS, thereby reducing the need of minor salivary gland. The following variables were included in the model: ANA, SS-A/SS-B, Schirmer's Test/BUT, C3/C4, serum gammaglobulin levels. One hundred consecutive individuals reporting clinical syndromes consistent with a sicca syndrome were included in the study. The application of our multifactorial mathematical model has shown a high predictive value for SS vs controls or vs patients with other autoimmune disorders (Sensitivity 93%, Specificity 100%), with an estimated minor salivary gland reduction of 77%. We conclude that our mathematical model can be considered a useful non-invasive approach for diagnosing Sjogren's Syndrome and recommend its validation on a larger scale. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which is related to transient red cell aplasia (TRCA) in paediatric patients.

FAB proposals for the diagnosis of AML-M0 represent the formal recognition of a distinct entity which has been described over the past few years by several authors and called minimally differentiated acute myeloid leukemia. By definition, AML-M0 includes acute leukemias which do not fit morphological and cytochemical criteria for the diagnosis of AML, and for which myeloid lineage assignment can be made by immunological assay showing positivity for MPO, CD13, and CD33 and negativity for lymphoid markers. Involvement of an early myeloid progenitor in the leukemic process is a possible theory hypothesized to explain the existence of such a form. Validity of this assumption has been based on the observation that AML-M0 frequently bears "stem cell" markers such as CD34, HLA-DR, Tdt, CD7, and promiscuous IgH/TCR gene rearrangements, which are thought to occur in uncommitted cells. Finally, AML-M0 very frequently carries cytogenetic abnormalities common to MDS or secondary AML, such as -5/5q- or -7/7q- deletions and or complex karyotype. In our experience, AML-M0 is also very often associated with the MDR phenotype, which in turn has been found strictly linked to "stem cell" features, especially in MDS. These biological aspects, altogether, translate into a very unfavorable prognosis, confirming even from a clinical point of view that AML-M0 is a distinct entity. In conclusion, "stem cell" markers, MDR phenotype, complex chromosome lesions, frequent occurrence in elderly patients, and intrinsic chemoresistance characterize AML-M0 and indicate the need for tailored treatments, possibly involving the use of MDR modulators and/or differentiating agents. [ABSTRACT FROM AUTHOR]

Diagnostic accuracy in acute leukemia (AL) can be improved if traditional morphology and cytochemistry are supplemented with immunophenotypic and genotypic analyses. This multiparameter approach is of crucial importance for the management of patients, as it enables the identification of leukemic syndromes with distinct biological features and response to treatment. Immunophenotyping using monoclonal antibodies has been universally accepted as a useful adjunct to morphological criteria. This technique is particularly valuable in diagnosing and subclassifying acute lymphoblastic leukemia and is also essential in certain types of acute myeloid leukemia (AML), such as AML with minimal differentiation or acute megakaryoblastic leukemia. Cytogenetic findings can be quite helpful in establishing the correct diagnosis and can add information of prognostic significance. A number of specific chromosomal abnormalities have been recognized that are very closely, and sometimes uniquely, associated with morphologically and clinically distinct subsets of leukemia. An even more basic understanding of normal and malignant hematopoietic cells has begun to evolve as molecular biology begins to unravel gene misprogramming by Southern and Northern blot analysis, the polymerase chain reaction, and fluorescence in situ hybridization. With the extensive use of these techniques it has become apparent that a proportion of leukemias exhibit the biologically relevant molecular defect in the absence of a karyotypic equivalent. On the other hand, apparently uniform chromosomal abnormalities such as the t(1;19) (q23;p13), t(9;22) (q33;q11), t(8;14) (q24;q32), or t(15;17) (q21;q21) may differ at the molecular level. Data collected from these modern technologies have introduced a greater complexity, which needs to be taken into consideration to improve both the diagnostic precision and the reproducibility of current classifications. [ABSTRACT FROM AUTHOR]

Focuses on a procedure for red blood cell (RBC) depletion and different cryoprotective solutions with or without rate-controlled freezing in an attempt to develop an inexpensive and simple method for processing and cryopreserving umbilical cord blood (UCB) hemopoietic cells. Cooling rate and concentration of the cryoprotectant; Analysis of RBC depletion by double gravity sedimentation and hyroxyethyl starch; Implications for UCB banks.

Thirteen refractory/resistant AML patients no suitable for additional aggressive chemotherapy, were treated with a combination including all-trans retinoic acid (45 mg/m2 sine die) and low doses of Ara-C (20 mg/m2 subcutaneously, twice in a day, days 1-10, every 28 days). Ten patients were evaluable; 8 of them achieved a complete remission, two patients with an important tumor burden, failed to achieve a response. One complete remission patient relapsed after 7 months but is still receiving the same therapy and is now in partial remission. We believe this combination effective as inducer of complete remission in those AML patients which cannot tolerate additional heavy treatments. The role of tumor burden in affecting response to therapy remains to be still evaluated. [ABSTRACT FROM AUTHOR]