1,721,087 research outputs found
How I diagnose and treat splenic lymphomas.
Full text linkThe incidental finding of an isolated splenomegaly during clinical assessment of patients evaluated for unrelated causes has become increasingly frequent because of the widespread use of imaging. Therefore, the challenging approach to the differential diagnosis of spleen disorders has emerged as a rather common issue of clinical practice. A true diagnostic dilemma hides in distinguishing pathologic conditions primarily involving the spleen from those in which splenomegaly presents as an epiphenomenon of hepatic or systemic diseases. Among the causes of isolated splenomegaly, lymphoid malignancies account for a relevant, yet probably underestimated, number of cases. Splenic lymphomas constitute a wide and heterogeneous array of diseases, whose clinical behavior spans from indolent to highly aggressive. Such a clinical heterogeneity is paralleled by the high degree of biologic variation in the lymphoid populations from which they originate. Nevertheless, the presenting clinical, laboratory, and pathologic features of these diseases often display significant overlaps. In this manuscript, we present our approach to the diagnosis and treatment of these rare lymphomas, whose complexity has been so far determined by the lack of prospectively validated prognostic systems, treatment strategies, and response criteria
Splenic lymphangiomatosis showing rapid growth during lactation: a case report.
Full text linkSplenic lymphangiomatosis is a very rare condition that, from 1990 to date, has been described only nine times. In the present report, we describe the first case of splenic lymphangiomatosis with rapid growth during lactation in a 35-year-old woman. We also underline the difficultly in making an accurate preoperative diagnosis, despite more modern imaging techniques. Total splenectomy was considered to be the treatment needed, both to make a definitive diagnosis and to exclude the presence of malignancy
The risk of Second Primary Cancers (SPCS) in patients with Splenic Marginal Zone Lymphoma (SMZL)
No full textReply to Pich et al.: intrasinusoidal bone marrow infiltration and splenic marginal zone lymphoma: a quantitative study.
No full textA rapidly progressive motor neuron disease associated to a natural killer cells leukaemia
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system play a complex role, either protective or toxic, in ALS
pathogenesis [1–3]. In particular, compelling evidence indicate that
increased blood level of natural killer (NK) and NK-T cells may contribute
to the disease development and progression [2,3].
Here, we report on a patient with an aggressive Motor Neuron
Disease (MND) associated with NK/NK-T cells leukaemia.
1. Case report
A 79-year-old man presented with several months-history of a
progressive atrophy and weakness of the upper limbs, which quickly
spread to the lower limbs. Onset was subtle and apparently occurred in
the month of July (the specific date is not shown for privacy), when the
patient noticed a mild weakness in the proximal muscles of the right
arm. From July through August, the patient's global functioning was
however roughly normal. However, between September and early
October the patient experienced a rapid worsening, with a severe
weakness of the shoulder girdle, of the right arm and in both legs. He
was referred to our ALS Center for evaluation. The neurological examination
documented marked muscle atrophy of the shoulder girdle,
the right arm and both lower limbs, with bilateral foot drop. Deep
tendon reflexes were absent, with the exception of the left arm. Bulbar
functions were normal. Needle EMG showed widespread polyphasic
MUP and fibrillation potentials, with normal sensory and motor conduction
velocities and no conduction blocks. Sensory and motor nerves
amplitudes in the upper limbs were within the normal range. A brain
and cervical MRI was within normal range. Extensive biochemical and
immunological work-up, including CSF, blood cell count, a large antibody
battery which included antibodies to anti-Hu, anti-Ri, anti-Yo, and
anti-gangliosides were negative. Serum creatine phosphokinase level
was mildly increased. A diagnosis of a very rapidly progressing lower
motor neuron disease (MND) was made, with an ALSFRS-R score of 18/
48 and a ΔFS [4] of 10 (Fig. 1A).
Although the clinical features of the patient did not fit with a formal
diagnosis of ALS, a genetic testing for the major ALS related genes
(C9orf72, FUS, TARDBP, SOD1, and Angiogenin) was performed, which
gave negative results. In the middle of November 2016, a follow-up
blood test showed a marked increase of WBC (i.e., 76,050/μL), with a
high prevalence of lymphocytes.
He was then admitted to the Hematology Unit in our Hospital. Blood
cytofluorimetric analysis and a bone marrow biopsy showed a marked
infiltration by immature NK and NK-T cells (Fig. 1B). A new CSF analysis
was performed, which showed increased proteins and 360/μL WBC, of
which about 70% were NK/NK-T cells. A diagnosis of NK/NK-T cell leukaemia
was made. A systemic chemotherapy according to Hyper-CVAD
schema was started (i.e., High Dose Dexamethasone; Hyperfractionated
Cyclophosphamide; Oncovine and Doxorubicine), along with repeated
intrathecal injections with Methotrexate, Ara-C and Dexamethasone.
In the following weeks, the patient showed a transient improvement
in muscle strength and function; a ALSFRS-R evaluation, made in
December 2016, gave a score of 27/48, with a ΔFS dropping to 4.2
(Fig. 1A). Unfortunately, the general health status deteriorated over
time, and in late December, the patient was referred to a Hospice,
where a few weeks later he peacefully passed away.
2. Discussion
We have described an aggressive lower MND, which might be
paraneoplastic, associated with an NK-T leukaemia. A paraneoplastic
association between either lower MND and lymphoma or the upper
predominant MND and breast cancer has been reported [5–7].
The presence of a high number of NK/NK-T cells in the CSF indicates
a meningeal metastasis from the acute leukaemia. These cells
are most probably involved in the lower motor neuron degeneration in
our patient. The clinical diagnosis of MND in this case is supported by
several lines of evidence: a) the onset of atrophy and weakness occurred
in the shoulder girdle, involving first the right shoulder with a rapid
progression to the left shoulder, and then with a proximal-to-distal
spreading to the right arm and then to the lower limbs. This pattern of
spreading is consistent with a primarily MND involvement, rather than
a poly/multi-neuropathy; b) the EMG/ENG pattern was also consistent
with a lower motor neuron disease, as neither abnormal sensory/motor
amplitudes (recorded in the upper limbs) nor conduction velocities/
blocks (recorded in both the upper and lower limbs) could be
detected. It cannot, however, be excluded a contemporary involvement
of the motor neurons cell bodies and the proximal section of the axons (i.e.
the motor roots)
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Nuove prospettive nel Managment dei pazienti con osteonecrosi dei mascellari da bifosfonati
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Pseudotemporal ordering of spatial lymphoid tissue microenvironment profiles trails Unclassified DLBCL at the periphery of the follicle
Full text link: We have established a pseudotemporal ordering for the transcriptional signatures of distinct microregions within reactive lymphoid tissues, namely germinal center dark zones (DZ), germinal center light zones (LZ), and peri-follicular areas (Peri). By utilizing this pseudotime trajectory derived from the functional microenvironments of DZ, LZ, and Peri, we have ordered the transcriptomes of Diffuse Large B-cell Lymphoma cases. The apex of the resulting pseudotemporal trajectory, which is characterized by enrichment of molecular programs fronted by TNFR signaling and inhibitory immune checkpoint overexpression, intercepts a discrete peri-follicular biology. This observation is associated with DLBCL cases that are enriched in the Unclassified/type-3 COO category, raising questions about the potential extra-GC microenvironment imprint of this peculiar group of cases. This report offers a thought-provoking perspective on the relationship between transcriptional profiling of functional lymphoid tissue microenvironments and the evolving concept of the cell of origin in Diffuse Large B-cell Lymphomas
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