Platelet satellitism in autoimmune hemolytic anemia.

Platelet satellitism is a rare phenomenon observed in blood smears obtained from blood anticoagulated with EDTA with an estimated frequency at 0.008%. It is characterized by platelet rosetting around neutrophils and in rare cases around other blood cells, which may causes pseudothrombocytopenia. Our case involves a 26-year-old female patient with autoimmune hemolytic anemia (AIHA). Blood smear with EDTA as an anticoagulant had platelet satellitism whereas the phenomenon was not observed in tubes with different anticoagulants (citrate, heparin) and capillary blood. This phenomenon was still observed four weeks after the patient's discharge. Furthermore, we discuss the possible pathophysiological mechanisms of platelet satellitism proposed in the literature. To our knowledge, this is the first case of platelet satellitism reported in AIHA.

[Juvenile myelomonocytic leukemia: A three-case series]. / Leucémie myélomonocytaire juvénile : à propos de trois cas.

Juvenile myelomonocytic leukemia (JMML), previously known as juvenile chronic myeloid leukemia (JCML), is a rare, myelodysplastic-myeloproliferative disease typically presenting in early childhood. This disorder is difficult to distinguish from other myeloproliferative syndromes such as chronic myeloid leukemia (CML) because of the similarities in their clinical and bone marrow findings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinemia, and raised fetal hemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukemia (CMML) group, as seen in adult patients. We describe three cases of JMML, who had very similar clinical and laboratory findings.

[Thrombotic thrombocytopenic purpura in a newborn]. / Purpura thrombotique thrombocytopénique chez un nouveau-né.

We report the case of a newborn presenting with hemolytic anemia, thrombocytopenia, hyperbilirubinemia, and renal failure in the first hours of life. An early plasmatherapy was undertaken, with good outcome. The specific von Willebrand factor-cleaving protease activity (ADAMTS 13 for a disintegrin and metalloprotease with thrombospondin type 1 repeats) was found to be low. This is the specific biologic diagnostic element of congenital thrombotic thrombocytopenic purpura (TTP). This disease of constitutional thrombotic microangiopathy is rare. The prognosis, usually life-threatening, was completely transformed given the better understanding of the pathogenesis of the disease and therapeutic progress.

[Hematologic abnormalities in infantile visceral leishmaniasis]. / Anomalies hématologiques au cours de la leishmaniose viscérale infantile.

The clinical and biological manifestations of visceral leishmaniasis are often confusing, most particularly because it can mimic and lead to a variety of hematological disorders. The aim of this study was to investigate the hematologic abnormalities observed in infantile visceral leishmaniasis from January 2000 and December 2013. The study included 35 children with a mean age of 3.5 years. Clinical manifestations were dominated by splenomegaly, fever, and paleness, defining the classic triad in 16% of our patients. Anemia was present in all patients. Leukopenia was found in 51% of the cases. Thrombocytopenia was observed in 48% of our patients and 36% had pancytopenia. All cases were confirmed by the presence of Leishman bodies (amastigotes) in the bone marrow smears. Quantitative and qualitative megakaryocyte abnormalities were found. Similarly, dysgranulopoiesis was observed in 31% of the cases, eosinophilia was present in 6%, erythroid hypoplasia in 3%, and erythroid hyperplasia in 34%. Different features of dyserythropoiesis were revealed in 71% of the patients with images of hemophagocytosis in 6% and multiple dysplasias in 9%. The knowledge of these hematological abnormalities associated with infantile visceral leishmaniasis can assist us in searching for Leishman bodies in the bone marrow smears to provide a diagnosis more quickly without necessarily resorting to more sophisticated tests.

Antibacterial and antifungal activity of lawsone and novel naphthoquinone derivatives.

INTRODUCTION: Naphthoquinone derivatives are under investigation as potential therapeutic agents. The antibacterial and antifungal activity of lawsone and of some novel naphthoquinone derivatives was assessed in vitro. METHODS: The antimicrobial activity was determined using diffusion disk and the broth microdilution methods against seven bacteria and three Candida species, according to recommendations of the Clinical and Laboratory Standards Institute. RESULTS: Two compounds (P05 et P06) presented a good antibacterial effectiveness against two gram-positive bacteria. No antifungal potency was observed against the three Candida albicans strains used in the test. CONCLUSION: Our results prove that the introduction of substituents on ketone function position 4 decreased the antimicrobial properties of the synthetized compounds.

Improved in vivo two-photon imaging after blood replacement by perfluorocarbon.

Two-photon microscopy is a powerful method in biomedical research that allows functional and anatomical imaging at a subcellular resolution in vivo. The technique is seriously hampered by absorption and scattering of light by blood, which prevents imaging through large vessels. Here, we demonstrate in the rat cerebral cortex that blood replacement by perfluorocarbon emulsion, a compound also used in human critical care medicine, yields superior image quality, while preserving neuronal integrity. Shadows of large superficial vessels disappear completely and cells can be imaged underneath them. For the first time, it is possible to image complete populations of neurons and astrocytes in the upper layers of neocortex in vivo.

[Basedow's disease and celiac disease in an adolescent with Down syndrome]. / Maladie de basedow associée a une maladie coeliaque chez une adolescente trisomique 21.

INTRODUCTION: Down syndrome is a favourable land to the emergence of auto-immune disease. CASE RECORD: Graves' disease and celiac disease were diagnosed in a 16 years old adolescent with Down syndrome presenting chronic diarrhoea, important delayed development and signs of hyperthyroidism. DISCUSSION: Celiac disease and thyroid dysfunction would be screening in patient with Down syndrome.

[Asymptomatic saphenous vein graft aneurysm: a case report]. / Anévrisme asymptomatique d'un greffon veineux coronarien: a propos d'un cas.

The authors report the case of a 48-year-old man with a history of coronary bypass graft 15 years earlier. The follow-up by routine chest radiography showed a right paracardiac opacity, which MRI conformed to be an aneurysm of the saphenous vein graft.

Methylenetetrahydrofolate reductase C677T and A1298C polymorphism and changes in homocysteine concentrations in women with idiopathic recurrent pregnancy losses.

Because they have been described as strong risk factors for idiopathic recurrent pregnancy losses (RPLs), we assessed the association between the methylenetetrahydrofolate reductase (MTHFR) single-nucleotide polymorphisms (SNPs) C677T and A1298C and hyperhomocysteinemia in Tunisian women with idiopathic RPL. Study subjects comprised 200 patients with more than three consecutive RPLs, and 200 age-matched parous control women. C677T and A1298C SNPs were analyzed by PCR-RFLP analysis, and fasting serum homocysteine was measured with ELISA. The frequency of MTHFR 677T/T (30.0 vs 7.0%) and 1298C/C (13.5 vs 4.0%) genotypes was significantly higher in patients. While it was similar among patients and controls (P = 0.095), higher homocysteine was seen with the T/T (but not 1298A/C and 1298C/C) genotype among patients and controls compared with non-T/T carriers (P < 0.05), and in patients vs controls. Higher prevalence of MTHFR 677T/T was seen in late (P < 0.05) and early-late (P < 0.001) RPL, while higher prevalence of 1298C/C genotype was seen only in early-late RPL (P < 0.001), and the prevalence of double heterozygotes was statistically not significant between patients and controls (P = 0.10; odds ratio = 2.73). Logistic regression analysis showed that, after adjusting for all variables, homozygosity for MTHFR C677T was associated with late (P < 0.001), and combined early-late (P < 0.001), while homozygosity for A1298C was associated only with combined early-late (P = 0.026), as was secondary-level education, which was associated with early (P = 0.005), late (P = 0.026) and combined early-late (P = 0.004) abortions. Homozygosity for MTHFR C677T (late and early-late) and A1298C (early-late) are risk factor for RPLs, irrespectively of total homocysteine levels.

[Solitary eosinophilic granuloma of the orbit: a case report]. / Granulome éosinophile isolé de l'orbite. A propos d'un cas.

The authors report a case of eosinophilic granuloma involving the roof and the lateral wall of the left orbit in a 5-year-old boy. The clinical presentation and especially the imaging features (computed tomography and MRI) suggested a malignant tumor and the final diagnosis was obtained by fine needle aspiration biopsy with histopathologic examination. Despite its alarming radiologic appearance, there was spontaneous healing of the eosinophilic granuloma with restitution ad-integrum of the bone.

[Hepatic hydatidosis and portal hypertension in children. Is it the Budd-Chiari syndrome?]. / Hydatidose hépatique et hypertension portale chez l'enfant. S'agit-il d'un syndrome de Budd-Chiari?

A case of portal hypertension in a five-year-old with multiple hydatid cysts in the liver is reported. Compression of the portal vein was the likely mechanism. Abdominal ultrasonography disclosed four hydatid cysts of which the largest was located in segments VII and VIII and caused compression of the supra-hepatic veins and inferior vena cava. Doppler ultrasonography showed continuous venous flow without triphasic modulation in the middle suprahepatic vein and inferior vena cava. Abdominal computed tomography demonstrated compression of the inferior vena cava and failed to visualize the suprahepatic veins. Upon surgery, evidence of hepatic venous statis was found. The child died 24 hours post-surgery. Budd-Chiari syndrome should be looked for routinely in patients with hydatid disease of the liver.

Phase I trial of a 5-day infusion of L-leucovorin plus daily bolus 5-fluorouracil in patients with advanced gastrointestinal malignancies.

The combination of leucovorin [(6d,l)-5-formyl-tetrahydrofolate] and 5-fluorouracil (5-FU) has increased efficacy compared to 5-FU alone as treatment of advanced colorectal cancer. Leucovorin is metabolized to methylene tetrahydrofolate, which potentiates the antitumor actions of 5-FU by forming a ternary complex of thymidylate synthase, fluorodeoxyuridine and methylene tetrahydrofolate. Only l-leucovorin is metabolized to methylene tetrahydrofolate and forms this ternary complex. However, d-leucovorin may not be inert. d-Leucovorin may impair cellular uptake and metabolism of l-leucovorin, thereby inhibiting the actions of l-leucovorin. Because of this possible limitation to the effectiveness of racemic leucovorin, we have begun to explore the effects of the pure, biologically active isomer, l-leucovorin. In this phase I trial, patients with advanced gastrointestinal malignancies were treated with a 5-day continuous infusion of l-leucovorin and daily intravenous boluses of 5-FU at 370 mg/m2. The dose of l-leucovorin was escalated in groups of three patients at four doses, 200 mg/m2 per day, 400 mg/m2 per day, 700 mg/m2 per day and 1000 mg/m2 per day. Treatment was repeated every 28 days. Seventeen patients with advanced gastrointestinal cancers entered the trial. Sixteen patients were evaluable for toxicity. Toxicity was similar to that expected for leucovorin plus 5-FU. The most common severe toxicities (and the number of patents affected) were: diarrhea (2), mucositis (2), nausea/vomiting (1), and abdominal/rectal pain (2). The maximum tolerated dose of l-leucovorin was 700 mg/m2 per day. Twelve patients were evaluable for response. One complete, one partial and one minor response were observed. All responses occurred among the nine patients with colorectal carcinomas. The combination of l-leucovorin and 5-FU is well tolerated by patients and appears active for treatment of advanced colorectal carcinomas. Additional clinical trials are necessary to determine if l-leucovorin is more effective than d,l-leucovorin for modulating the effectiveness of 5-FU.

The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability.

To determine whether the vasoocclusive severity of homozygous sickle cell (SS) disease is influenced by cellular dehydration, we correlated the incidence of painful crisis with steady-state measurements of red cell hydration. Sixteen children with SS disease were followed for 3.3 to 8 years (mean, 6.8 years), and a single crisis rate was calculated for each patient. At the time of well visits, cellular hydration was assessed by measuring cell deformability, the percentage of red cells with a density greater than or equal to 1.1056 g/mL, and the percentage of irreversibly sickled cells (ISC). The incidence of painful crisis showed a strong positive correlation with Omax, a deformability measurement reflecting cellular hydration (r = .84, P less than .002), and with hemoglobin concentration (r = .59, P = .04). That is, higher crisis rates were observed in patients with less dehydrated, more deformable red cells and also in patients with higher hemoglobin concentrations. Furthermore, cell deformability and hemoglobin concentration were independent predictors of the incidence of painful crisis, which is consistent with separate effects of these two red cells parameters on vasoocclusive severity.