Characterization of CD34+ hematopoietic cells in systemic mastocytosis: Potential role in disease dissemination.

BACKGROUND: Recent studies show that most systemic mastocytosis (SM) patients, including indolent SM (ISM) with (ISMs+) and without skin lesions (ISMs-), carry the KIT D816V mutation in PB leukocytes. We investigated the potential association between the degree of involvement of BM hematopoiesis by the KIT D816V mutation and the distribution of different maturation-associated compartments of bone marrow (BM) and peripheral blood (PB) CD34+ hematopoietic precursors (HPC) in ISM and identified the specific PB cell compartments that carry this mutation. METHODS: The distribution of different maturation-associated subsets of BM and PB CD34+ HPC from 64 newly diagnosed (KIT-mutated) ISM patients and 14 healthy controls was analyzed by flow cytometry. In 18 patients, distinct FACS-purified PB cell compartments were also investigated for the KIT mutation. RESULTS: ISM patients showed higher percentages of both BM and PB MC-committed CD34+ HPC vs controls, particularly among ISM cases with MC-restricted KIT mutation (ISMMC ); this was associated with progressive blockade of maturation of CD34+ HPC to the neutrophil lineage from ISMMC to multilineage KIT-mutated cases (ISMML ). Regarding the frequency of KIT-mutated cases and cell populations in PB, variable patterns were observed, the percentage of KIT-mutated PB CD34+ HPC, eosinophils, neutrophils, monocytes and T cells increasing from ISMs-MC and ISMs+MC to ISMML patients. CONCLUSION: The presence of the KIT D816V mutation in PB of ISM patients is associated with (early) involvement of circulating CD34+ HPC and multiple myeloid cell subpopulations, KIT-mutated PB CD34+ HPC potentially contributing to early dissemination of the disease.

Increased IL6 plasma levels in indolent systemic mastocytosis patients are associated with high risk of disease progression.

Systemic mastocytosis (SM) is a heterogeneous disease with altered interleukin (IL)-6 and IL13 plasma levels. However, no study has simultaneously investigated the plasma levels of IL1ß, IL6, IL13, CCL23 and clusterin in SM at diagnosis and correlated them with disease outcome. Here we investigated IL1ß, IL6, IL13, CCL23 and clusterin plasma levels in 75 SM patients--66 indolent SM (ISM) and 9 aggressive SM--and analyzed their prognostic impact among ISM cases grouped according to the extent of hematopoietic involvement of the bone marrow cells by the KIT D816V mutation. Although increased IL1ß, IL6 and CCL23 levels were detected in SM patients versus healthy controls, only IL6 and CCL23 levels gradually increased with disease severity. Moreover, increased IL6 plasma levels were associated with ISM progression to more aggressive disease, in particular among ISM patients with multilineal KIT mutation (ISM-ML), these patients also showing a higher frequency of organomegalies, versus other ISM-ML patients. Of note, all ISM patients who progressed had increased IL6 plasma levels already at diagnosis. Our results indicate that SM patients display an altered plasma cytokine profile already at diagnosis, increased IL6 plasma levels emerging as an early marker for disease progression among ISM cases, in particular among high-risk ISM patients who carry multilineage KIT mutation.

Histeroscopia en hiperplasia y cáncer de endometrio / Hysteroscopy in hyperplasia and endometrial cancer

La histeroscopia permite observar directamente el interior del útero, La hiperplasia endometrial definida como una proliferación de glándulas, de forma y tamaño irregular, no presenta patrón histeroscópico determinado para cada tipo de hiperplasia. Cuando valoramos eficacia y correlación del diagnostico clínico con el histológico, la eficacia diagnóstica para hiperplasia oscila entre el 56 y el 83%, La normalidad de imagen histeroscópica no excluye la posibilidad de patología. La sensibilidad es del 98% con una especificidad del 99.9% en determinados grupos especialmente entrenados, entre los que nos encontramos, en el adenocarcinoma endometrial hacen a la histeroscopia herramienta imprescindible. La afectación cervical es diagnosticada con una sensibilidad que oscila entre el 64-100%, especificidad 73-98%. El VPN es de 100% y un VPP entre el 38.4 y 93.3%. La histeroscopia es el método de elección en el diagnostico y manejo de la patología maligna endometrial, favoreciendo el hallazgo temprano y el estadiaje del cáncer endometrial. Precisa entrenamiento y aprendizaje para la obtención de su mejor rendimiento diagnostico. Con una elevada sensibilidad, especificidad

The hysteroscopy allows to observe the interior of the uterus directly. The endometrial hyperplasia defined as a proliferation of glands, of form and irregular size, it doesn’t present hysteroscopic patron certain for each hyperplasia type. When we value effectiveness and correlation of the diagnose clinical with the histology, the effectiveness diagnostic for hyperplasia oscillates between the 56 and 83%. The normality of image hysteroscopic doesn’t exclude the pathology possibility. The sensibility is of 98% with a specificity of 99.9% in certain specially trained groups, in those that we find ourselves , the carcinoma endometrial makes to the hysteroscopy indispensable tool. The cervical affectation is diagnosed with a sensibility that oscillates between 64-100%, specificity 73-98%. The VPN is of 100% and a VPP between the 38.4 and 93.3%. The hysteroscopy is the election method in the diagnose and manage of the pathology wicked endometrial, favoring the early discovery and the stage of the endometrial cancer. It is necessary a training and a learning for the obtainig of their best yield diagnose. With a high sensibility, specificity

Segmentación y marketing sanitario / Sanitary service and marketing

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New benzyl alcohol glycosides from Pyrus bourgaeana.

Two new aromatic glycosides, benzyl beta-D-glucopyranosyl-(1-->2)-[beta-D-apiofuranosyl-(1-->6)]-beta- D- glucopyranoside and p-[beta-D-apiofuranosyl-(1-->6)]-beta-D- glucopyranosyloxy-benzylic alcohol, have been isolated from the aerial parts of Pyrus bourgaeana (Rosaceae). The structures were elucidated by spectral analyses (including 2D-NMR spectral measurements) and chemical methods. Arbutin and four other aromatic glycosides have also been isolated and identified.

[Acetylation polymorphism in lung cancer]. / Polimorfismo acetilador en el cáncer de pulmón.

The acetylation phenotype was determined, by means of sulfamethazine measurement, in 87 patients (83 male) with confirmed bronchogenic carcinoma and in 93 healthy control patients (41 male) of equal ages. 48 patients and 54 controls were classified as being "slow acetylators" (Ch2 n.s.) When the persons were individually analysed by phenotype, it was confirmed that the patients showed a significantly lower rate of acetylated sulfamethazine than the control group (p less than 0.02), owing to the poor acetylation of patients with small-cell lung cancer. This difference should be confirmed by more detailed pharmacokinetic studies before regarding it as a possible interference of paraneoplasic type. The polymorphism acetylator cannot be considered a genetic marker related to the risk of having lung cancer.

[Malignant epithelioid paravertebral schwannoma (author's transl)]. / Schwannoma epitelioide maligno paravertebral.

Tumors arising from the nerve sheaths are a controversial group of neoplasms because of disagreement regarding the clinical course and histopathologic characteristics. Malignant Schwannoma is a rare entity with aggressive biological behaviour (local infiltration and hematogenous metastases). The tumor arises from Schwann cells and may show diverse histological patterns. A malignant epithelioid paravertebral Schwannoma was diagnosed in a 48-year-old patient. Tumor metastases were demonstrated in the lungs, pericardium, liver, intestine, kidney, adrenals, bones and lymph nodes. The differential diagnosis and literature on this topic are commented on.