Molecular profiling of different glioma specimens from an Ollier disease patient suggests a multifocal disease process in the setting of IDH mosaicism

Purpose of StudyATL is a peripheral T-cell neoplasm(PTCL) associated with human T-cell lymphotropic virus-1 (HTLV-1) infection. Currently there is no established therapy for relapsed/refractory disease. Initial in-vitro studies of HDAC inhibitors showed selective apoptosis of HTLV-1 infected T cell lines. In phase II trial for relapsed/refractory PTCL, 2 patients had EBV and 1 patient had HBV reactivation. It was unclear if this was from HDAC induced immunosuppression or direct promotion of viral replication or underlying disease process. All HDAC inhibitors trials in last 6 years excluded ATL patients although they are approved for this disease. We here describe our experience of 3 patients with relapsed/refractory ATL treated with HDAC inhibitor romidepsin.Methods UsedChart review of patients with relapsed/refractory ATL treated with romidepsin at King's County Hospital.Summary of ResultsCase 1: 43 year old male with acute ATL who progressed on EPOCH after 4 cycles. Romidepsin was started at 14 mg/m2 IV Day 1, 8, 15 Q28 days. He tolerated cycle 1 well but continued to have progressive disease. Patient died 40 days after initiation of therapy from infection.Case 2: 37 year old male with acute ATL who had disease progression on EPOCH×2 cycles. He was started on romidepsin 10 mg/m2 IV (dose reduced due to T. bili 3.5 gm/dl). After first dose, his platelets dropped to to 20 k/mm3 necessitating treatment delay and dose reduction to 6 mg/m2. He had temporary response as evidenced by reduction in WBC count from 103 kmm3 to 5 k/mm3 and improvement in liver function. He only received 1 cycle and died on Day 50 from disease progression.Case 3: 47 year old male with ATL lymphoma intially treated with CHOP×6 cycles and relapsed after 1 year with peripheral lymphocytosis to 57 k/mm3 and diffuse lymphadenopathy. He received ICE×2 cycles with progressive disease. He was started on romidepsin 14 mg/m2. He received 1 dose and had Grade IV anemia/thrombocytopenia. He developed urosepsis and expired on Day 20.ConclusionsIn our small experience of romidepsin in relapsed/refractory ATL, patients appear to have modest response rates and higher rate of cytopenias when compared to other PTCL subtypes in clinical trials. Given the concerns for viral reactivation and lack of data for use of romidepsin in ATL, it should be used cautiously.

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Ulcerogenic Tumors of the Pancreas

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100100676 ◽

1968 ◽

Vol 26 ◽

pp. 186-187

Author(s):

J. C. Garancis ◽

J. F. Kuzma ◽

S. D. Wilson ◽

E. H. Ellison

Keyword(s):

Endoplasmic Reticulum ◽

Tumor Cells ◽

Islet Cell ◽

Disease Process ◽

Central Core ◽

Islet Cell Tumors ◽

Opaque Zone ◽

Granular Form ◽

Zollinger Ellison Syndrome

It has been proposed that a gastrin-like hormone elaborated by non-beta islet tumors of the pancreas may be responsible for a fulminating ulcer diathesis. Subsequently, a potent gastric secretagogue was isolated from ulcerogenic tumors of the pancreas. This disease process is known now as “Zollinger-Ellison syndrome”.In our studies of two cases of Zollinger-Ellison syndrome, pancreatic lesions were identified as alpha islet cell tumors (Fig. 1). Tumor cells were fairly uniform. The sizes of the alpha granules were not significantly different, but their number and distribution varied greatly from one cell to another. Each granule consisted of a round, highly dense central core, separated from the limiting membrane by an opaque zone. The granular form of the endoplasmic reticulum was particularly prominent. Numerous mitochondria, round or elongated, were dispersed throughout the cytoplasm. Individual or clusters of lysosomes were observed in the majority of cells.

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Electron microscopy analysis of degenerating pancreatic ß cells in transgenic mice expressing the MHC Class I antigen Dd

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100160297 ◽

1990 ◽

Vol 48 (3) ◽

pp. 548-549

Author(s):

T. A. Stewart ◽

D. Liggitt ◽

S. Pitts ◽

L. Martin ◽

M. Siegel ◽

...

Keyword(s):

Type I Diabetes ◽

Disease Process ◽

Class Ii ◽

Class I ◽

Type I ◽

Aberrant Expression ◽

Mhc Molecules ◽

Endogenous Insulin ◽

Class Ii Mhc ◽

Ifn Γ

Insulin-dependant (Type I) diabetes mellitus (IDDM) is a metabolic disorder resulting from the lack of endogenous insulin secretion. The disease is thought to result from the autoimmune mediated destruction of the insulin producing ß cells within the islets of Langerhans. The disease process is probably triggered by environmental agents, e.g. virus or chemical toxins on a background of genetic susceptibility associated with particular alleles within the major histocompatiblity complex (MHC). The relation between IDDM and the MHC locus has been reinforced by the demonstration of both class I and class II MHC proteins on the surface of ß cells from newly diagnosed patients as well as mounting evidence that IDDM has an autoimmune pathogenesis. In 1984, a series of observations were used to advance a hypothesis, in which it was suggested that aberrant expression of class II MHC molecules, perhaps induced by gamma-interferon (IFN γ) could present self antigens and initiate an autoimmune disease. We have tested some aspects of this model and demonstrated that expression of IFN γ by pancreatic ß cells can initiate an inflammatory destruction of both the islets and pancreas and does lead to IDDM.

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Auditory Dysfunction in Alzheimer's Disease

Perspectives on Gerontology ◽

10.1044/gero15.1.4 ◽

2010 ◽

Vol 15 (1) ◽

pp. 4-11 ◽

Cited By ~ 1

Author(s):

Sridhar Krishnamurti

Keyword(s):

Alzheimer’S Disease ◽

Older Adults ◽

Alzheimer's Disease ◽

Health Care ◽

Care Setting ◽

Neurodegenerative Disorder ◽

Disease Process ◽

Clinical Protocols ◽

Speech Language Pathologist ◽

Auditory Dysfunction

Alzheimer's disease is neurodegenerative disorder which affects a growing number of older adults every year. With an understanding of auditory dysfunction in Alzheimer's disease, the speech-language pathologist working in the health care setting can provide better service to these individuals. The pathophysiology of the disease process in Alzheimer's disease increases the likelihood of specific types of auditory deficits as opposed to others. This article will discuss the auditory deficits in Alzheimer's disease, their implications, and the value of clinical protocols for individuals with this disease.

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Drug-Induced Movement Disorders

Perspectives on Neurophysiology and Neurogenic Speech and Language Disorders ◽

10.1044/nnsld25.2.70 ◽

2015 ◽

Vol 25 (2) ◽

pp. 70-77

Author(s):

Amy Lustig ◽

Cesar Ruiz

Keyword(s):

Movement Disorders ◽

Medical Management ◽

Disease Process ◽

Underlying Disease ◽

Extrapyramidal Symptoms ◽

Drug Induced ◽

General Overview ◽

Management Approaches ◽

Broad Understanding ◽

Underlying Disease Process

The purpose of this article is to present a general overview of the features of drug-induced movement disorders (DIMDs) comprised by Parkinsonism and extrapyramidal symptoms. Speech-language pathologists (SLPs) who work with patients presenting with these issues must have a broad understanding of the underlying disease process. This article will provide a brief introduction to the neuropathophysiology of DIMDs, a discussion of the associated symptomatology, the pharmacology implicated in causing DIMDs, and the medical management approaches currently in use.

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Molecular profiling informs ovarian cancer therapy

PsycEXTRA Dataset ◽

10.1037/e458562008-001 ◽

2006 ◽

Author(s):

Heather Maisey

Keyword(s):

Ovarian Cancer ◽

Cancer Therapy ◽

Molecular Profiling

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Systemic intervention with families in disease process in addiction

PsycEXTRA Dataset ◽

10.1037/e635102013-133 ◽

2013 ◽

Author(s):

Nadja Vilela dos Santos

Keyword(s):

Disease Process ◽

Systemic Intervention

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Evaluating the Validity of a Bayesian Program for Predicting Stage in Hodgkin’s Disease

Methods of Information in Medicine ◽

10.1055/s-0038-1635305 ◽

1981 ◽

Vol 20 (03) ◽

pp. 174-178 ◽

Cited By ~ 2

Author(s):

A. I. Barnett ◽

J. Cynthia ◽

F. Jane ◽

Nancy Gutensohn ◽

B. Davies

Keyword(s):

New England ◽

Hodgkin's Disease ◽

Bayesian Model ◽

Medical Center ◽

Hodgkin’S Disease ◽

Test Procedure ◽

Disease Patient ◽

Probabilistic Information ◽

Probabilistic Forecasts ◽

England Medical

A Bayesian model that provides probabilistic information about the spread of malignancy in a Hodgkin’s disease patient has been developed at the Tufts New England Medical Center. In assessing the model’s reliability, it seemed important to use it to make predictions about patients other than those relevant to its construction. The accuracy of these predictions could then be tested statistically. This paper describes such a test, based on 243 Hodgkin’s disease patients of known pathologic stage. The results obtained were supportive of the model, and the test procedure might interest those wishing to determine whether the imperfections that attend any attempt to make probabilistic forecasts have gravely damaged their accuracy.

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