A Case Series of Blastic Plasmacytoid Dendritic Cell Neoplasia.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN), an extremely rare and aggressive tumor, derives from plasmacytoid dendritic cell precursors and is characterized by CD4 and CD56 positivity accompanied by the expression of isolated myeloid, B- or T-cell lineage markers. Despite the recent introduction of specific targeted therapies, prognosis is still poor with a median overall survival of one year, and allogeneic bone marrow transplantation remains the only curative treatment in eligible patients. In this series, we described two cases of adult BPDCN treated with high dose cytarabine and methotrexate and autologous hematopoietic stem cell transplantation, or fludarabine, cytarabine, and idarubicin achieving the first a complete lasting remission, while the second only a transient improvement in skin lesions.

Immunoglobulin heavy and light chains and T-cell receptor beta and gamma chains PCR assessment on cytological samples. A study comparing FTA cards and cryopreserved lymph node fine-needle cytology.

OBJECTIVES: To evaluate and compare the DNA yield and quality extracted from lymph node fine needle cytology (FNC) samples stored on FTA cards to those cryopreserved, and to assess the immunoglobulin heavy and light chains (IGHK) and T-Cell receptor beta and gamma chains (TCRBG) PCR tests. METHODS: DNA extractions were performed on FNC of 80 non-Hodgkin lymphomas (NHL), four myelomas and 56 benign reactive hyperplasias (BRH) cryopreserved and stored on FTA cards. The JAK2 gene was amplified to assess the DNA integrity and the IGHK/TCRBG clonality status was tested. RESULTS: IGHK monoclonality was found in 99% of B-cell NHL and 100% of myeloma. TCRBG monoclonality was found in 100% of T-cell NHL. TCRBG polyclonality was detected in 97% of B-cell NHL, 100% of myeloma and 96% of BRH. IGHK/TCRBG PCR data were confirmed by histological and/or follow-up controls. No differences were found in the DNA quality between cryopreservation and FTA cards storage methods. CONCLUSIONS: IGHK/TCRBG PCR of the lymphoproliferative process on FTA cards is comparable to those cryopreserved. FTA cards can be used to store lymph node FNC for further molecular investigations.

In Vitro Apoptotic Effects of Farnesyltransferase blockade in Acute Myeloid Leukemia Cells.

Farnesyltransferase inhibitors (FTIs) are a class of oral anti-cancer drugs currently tested in phase I-II clinical trials for treatment of hematological malignancies. The in vitro effects of various FTIs (alpha-hydroxyfarnesylphosphonic acid, manumycin-A and SCH66336) were tested on CD34+ KG1a cell line and in primary acute myeloid leukemia (AML) cells from 64 patients. By cell viability and clonogeneic methylcellulose assays, FTIs showed a significant inhibitory activity in CD34+ KG1a and primary bone marrow (BM) leukemic cells from 56% of AML patients. FTIs also induced activation of caspase-3 and Fas-independent apoptosis, confirmed by the finding that inhibition of caspase-8 was not associated with the rescue of FTI-treated cells. We concluded that other cellular events induced by FTIs may trigger activation of caspase-3 and subsequent apoptosis, but the expression of proapoptotic molecules, as Bcl-2 and Bcl-XL, and antiapoptotic, as Bcl-X(s), were not modified by FTIs. By contrast, expression of inducible nitric oxide synthase (iNOS) was increased in FTI-treated AML cells. Our results suggest a very complex mechanism of action of FTIs that require more studies for a better clinical use of the drugs alone or in combination in the treatment of hematological malignancies.

A novel surgical approach to calcinosis cutis using a collagen-elastin matrix.

Calcinosis cutis is a cutaneous disorder characterised by abnormal deposition of calcium in the dermis. Treatment of this condition has variable success rates and includes medical, topical and surgical management. Here we describe a case of a woman with a painful calcinosis lesion on the buttocks, treated with excision and application of a bovine collagen-elastin dermal regeneration template, a thin, porous membrane consisting of a native bovine type I, II and V collagen-fibre template coated with elastin hydrolysate. The patient's wound healed without complication and without the use of a skin graft. She remains recurrence free at 10-month follow up, with satisfactory outcome.

Cutaneous lymphoadenoma: a rare clinicopathological entity.

Cutaneous Lymphadenoma (Benign Lymphoepithelial tumour of the skin) is a rare tumour, with distinctive clinical and histological features. To date, very few cases of this entity have been reported. We present a case of cutaneous lymphoadenoma in a 52-year-old man and a short review of the literature, summarizing the principal clinical and morphological characteristics of this rare tumour.

[Scrofuloderma. a clinical case]. / Scrofuloderma. Un caso clinico.

Scrofuloderma is a form of colliquative cutaneous tuberculosis resulting from the spread of an underlying focus, generally represented by lymphnodes; osteoarticular tubercular locations or epididimus locations can also lead to ulcerative cutaneous involvement. We report the case of a female patient aged 12 who had been in Italy for 15 days following her arrival from Marocco. In the left clavicular region she presented an ulcerated lesion with palpable lymphnodes on the corresponding side of the neck. Histopathological examination of one of the involved lymphnodes suggested a mycobacteriosis; differential diagnosis was carried out between MAIS (M. avium, M. intracellulare, M. scrofulaceum) group mycobacteria infection and tuberculosis. Bacteriological research in the lymphnode of alcohol-acid resistant bacilli gave a negative result, while cultural examination and protein chain reaction pointed to M. tuberculosis. The patient was subjected to combined treatment based on rifampycin, isoniazide, etambutolol and pyrazinamide. At the present time, tuberculosis is an important public health problem in developing countries. In western countries such as the United Kingdom, which have been open to immigration for a longer time, scrofuloderma is present with higher prevalence in immigrants from Asia and Africa; in these immigrant populations, this tubercular form involves a wide age segment between the ages of 10 and 50. In the autochthonous population the highest incidence is in subjects aged more than 50. In Italy too, extra-communitary immigration has also contributed, with a delay compared to other western countries, to bring tuberculosis back into the public eye after a period of decades during which it was wrongly considered to have disappeared.

The distribution and colocalisation of nicotinamide adenine dinucleotide phosphate reduced-diaphorase (NADPH-d)-/nitric oxide synthase (NOS)-containing neurons in the innervation of the duck ureter.

ensp;The distribution and colocalisation of nicotinamide adenine dinucleotide phosphate reduced-diaphorase (NADPH-d)-/nitric oxide synthase (NOS)-containing (nitrergic) neurons in the innervation of the duck ureter have been studied using histochemistry and immunohistochemistry. Quantitative analysis showed that nitrergic neurons made up 60% and 70% of the total intramural and adventitial neuronal populations, respectively. About 40% of intramural nitrergic neurons expressed VIP-immunoreactivity, and about 75% of nitrergic adventitial neurons expressed TH-immunoreactivity. The density of nitrergic adventitial neurons was significantly greater in the lower tract than in the upper and intermediate tracts. Nerve lesioning experiments showed that the majority of ureteral nitrergic innervation was extrinsic in origin; nitrergic adventitial neurons primarily projected caudocranially, whereas NOS-immunoreactive and NOS-/VIP-immunoreactive intramural neurons primarily projected craniocaudally. These findings suggest that, in birds, the nitrergic innervation plays a role in ureteral functions such as epithelial mucosecretion, muscular motility, and the closing and/or opening of the ureteral papilla.

Ultrastructural and freeze fracture cilia morphology of trachea epithelium in apparently healthy small ruminants.

Specimens of tracheal mucosa were obtained from ten adult apparently healthy small ruminants (five goats and five sheep) both by a fibre optic endoscope and in a slaughterhouse. Ultrastructurally, a total of 50,000 cilia were examined. Pathological cilia were found in all examined subjects. The prevalence of compound cilia showed a range of 0.3% to 3%. Intracytoplasmic and swollen cilia ranged from 0.2% to 0.5%. The microtubular pattern was examined in 4,000 cross-sectioned cilia and an abnormal pattern was found in 5-7%. Microtubular defects involved both peripheral and central doublets, being peripheral abnormalities the prevailing ones. A central plug of electron dense material was observed in 2-3% of the examined basal bodies. Rare basal bodies characterized by an abnormal spatial configuration were also shown. Freeze fracture studies revealed a ciliary necklace composed of 4 to 5 rows of intramembrane particles.

The sequential hypothesis of sleep function. III. The structure of postacquisition sleep in learning and nonlearning rats.

EEG methods were used to examine the structure of postacquisition sleep in learning (L) and nonlearning (NL) rats previously exposed to a session of two-way active avoidance training, and in control rats (C) left in their home cages. In agreement with literature data, the number and total amount of paradoxical sleep (PS) episodes were higher in L rats than in NL rats. In addition, significant differences between L and NL rats concerned the episodes of synchronized sleep followed by wakefulness or by PS (SS-W and SS-PS, respectively). The average duration and related parameters of SS-W episodes, and the average duration, number, amount and related parameters of SS-PS episodes increased in NL and L rats in comparison with C rats. Longer SS-W episodes occurred early in NL and L rats, but the effect lasted longer in NL rats. On the other hand, the increments concerning SS-PS episodes occurred earlier, were more pronounced and laster longer in L rats. The results support a role of SS in brain information processing, as envisaged by the sequential hypothesis on the role of sleep. They suggest, furthermore, that memory traces lacking adaptive value may be destabilized and cleared away during SS-W and SS-PS episodes, while the remaining memory traces may be retained and eventually stored again in more integrated form during SS-PS and PS episodes, respectively.

The sequential hypothesis of sleep function. IV. A correlative analysis of sleep variables in learning and nonlearning rats.

Female adult rats were trained for a two-way active avoidance task (4 h), and allowed free sleep (3 h). Control rats (C) were left in their home cages during the acquisition period. Dural electrodes and an intraventricular cannula, implanted one week in advance, were used for EEG recording during the period of sleep and for the injection of [3H]thymidine at the beginning of the training session, respectively. Rats were killed at the end of the sleep period, and the DNA-specific activity was determined in the main brain regions and in liver. Correlations among sleep, behavioral and biochemical variables were assessed using Spearman's nonparametric method. In learning rats (L), the number of avoidances was negatively correlated with SS-W variables, and positively correlated with SS-PS variables (episodes of synchronized sleep followed by wakefulness or paradoxical sleep, respectively) and with PS variables. An inverse pattern of correlations was shown by the number of escapes or freezings. No correlations occurred in rats unable to achieve the learning criterion (NL). In L rats, the specific activity of brain DNA was negatively correlated with SS-W variables and positively correlated with SS-PS variables, while essentially no correlation concerned PS variables. On the other hand, in NL rats, comparable correlations were positive with SS-W variables and negative with SS-PS and PS variables. Few and weak correlations occurred in C rats. The data support a role of SS in brain information processing, as postulated by the sequential hypothesis on the function of sleep. In addition, they suggest that the elimination of nonadaptive memory traces may require several SS-W episodes and a terminal SS-PS episode. During PS episodes, adaptive memory traces cleared of nonadaptive components may be copied in more suitable brain sites.

Active polysomes in the axoplasm of the squid giant axon.

Axons and axon terminals are widely believed to lack the capacity to synthesize proteins, relying instead on the delivery of proteins made in the perikaryon. In agreement with this view, axoplasmic proteins synthesized by the isolated giant axon of the squid are believed to derive entirely from periaxonal glial cells. However, squid axoplasm is known to contain the requisite components of an extra-mitochondrial protein synthetic system, including protein factors, tRNAs, rRNAs, and a heterogeneous family of mRNAs. Hence, the giant axon could, in principle, maintain an endogenous protein synthetic capacity. Here, we report that the squid giant axon also contains active polysomes and mRNA, which hybridizes to a riboprobe encoding murine neurofilament protein. Taken together, these findings provide direct evidence that proteins (including the putative neuron-specific neurofilament protein) are also synthesized de novo in the axonal compartment.

[Vascularization of the thymus in the duck]. / Sulla vascolarizzazione del timo nell'anatra.

The arrangement and the blood of Thymus lobes were shown by intra-vasal injection of Indian ink or Neoprene Latex. The origin and the distribution of the arteries were studied within thymic lobe and until the final branches represented by endolobulare capillaries. These branches represent the origin of the vein of the thymic lobe.

Innervation pattern of intrinsic laryngeal musculature of the rabbit.

The intrinsic laryngeal musculature of rabbit was studied using various neurohistological techniques. The vegetative and sensory nerve terminals are present in abundance, while the proprioceptors are seemingly absent. Furthermore, simple motor endplates, sometimes furnished with ultraexpansional fibers, complex endplates, or 'en grappe' are described. Their abundance within a musculature lacking in muscle-spindles is indicative of a possible vicarious function.

[Blood supply of the adrenal gland in the domestic duck]. / Sui vasi sanguigni della ghiandola adrenale nell'anatra domestica.

The Authors studied the arterial and venous vascularization of the duck adrenal gland. Thirty subjects of both sexes were used. Some were inoculated with plastic substance to obtain vasal casts. Others were perfused with Indian ink before the preparation of istological sections. The angioarchitecture of the adrenal glands presents some very interesting features with regard to both the arterious and the venous circulation. The former constitute two systems, superficial and deep, both participating in the formation of the sinusoidal network. The veins are represented by the adrenal vein and by a superficial vein. The latter has a larger diameter when in proximity of the organ smaller peripheral branches in connection with the flank region and with the penultimate intercostal space. The peculiar morphology of the superficial vein induces the Authors to retain that it is an afferent vein and that it may be considered as the adrenal portal vein.

Vascular feature of the hypophysis in Bubalus buffalus.

The hypophysis of Bubalus buffalus was examined morphologically and compared to that of the bovine. The morphology and behavior of the vessels supplying the hypophysis and their contribution to the hypophyseal portal system were described. We found characteristic vascular arches between the pars tuberalis and the infundibulum from which the portal vessels originate. These vessels may be divided into long and short vessels, have different origins and extension, and branch in different regions of the anterior lobe.

[The lymphatic component of Bubalus buffalus' pharynx]. / Sulla componente linfatica della faringe in Bubalus buffalus.

The lymphatic component in the pharynx of Buffalo presents some peculiar characteristics of species above all as regards the palatine tonsil. This is characterized by the presence of numerous lobes, by the fast pigmentation of the mucosa in the sinus and sometimes by the crypts and by the wealth of glandular adenomeres. The lingual tonsil, the tonsil of the soft palate, the pharyngeal tonsil and the tubal tonsil are represented by lymphatic elements arranged in the lamina propria of the mucosa and only sometimes they are organized as follicles.