1,721,226 research outputs found
Large granular lymphocytosis
No full textBACKGROUND AND OBJECTIVES:
An increased number of granular lymphocytes (GL) has been reported in various clinical conditions and is currently interpreted as a reactive process to an underlying antigen stimulation. In recent years, a disease characterized by a definite increase in granular lymphocytes has been identified and recognized as lymphoproliferative disease of GL (LDGL). The aim of this study is to review the clinical, biological and pathogenetic mechanisms leading to this disorder.
DESIGN AND METHODS:
Criteria for the diagnosis, immunologic and molecular evaluation, clinical features and new therapeutic approaches are reviewed.
RESULTS:
More than 500 patients have been adequately reported in the literature. Immunologic classification of this disease distinguishes a CD3+ form which is more common, and a CD3- variant; this latter accounting for nearly 15% of LDGL cases. CD3+ LDGL is symptomatic in approximately 50% of cases, neutropenia, infections and anemia being the most frequent findings. Clonality of the T-cell receptor is usually documented in these patients. Cytokines such as IL-2, IL-12 and IL-15 have been claimed to play a role in this disorder. Symptomatic patients may benefit from combination therapy with low dose methotrexate and steroids. CD3- LDGL are usually associated with viral infection of GL: in particular, Epstein Barr and human T lymphotropic virus I/II have been claimed to play a role. Neutropenia is usually less pronounced than in CD3+ LDGL patients. Clonality has rarely been demonstrated; however, when present, it correlates with an aggressive clinical course. Spontaneous regression of lymphocytosis has been reported in both CD3+ and CD3- patients.
INTERPRETATION AND CONCLUSIONS:
Lymphoproliferative disease of granular lymphocytes is a well recognized disorder which encompasses a large spectrum of conditions, ranging from mild asymptomatic lymphocytosis to aggressive, usually fatal, disorders. Diagnosis of this disease is related to the demonstration that a discrete subset of GL is chronically expanded. Therapy should be delayed in asymptomatic patients; however, when needed, the combination of methotrexate or cytoxan and steroids represents the best approach
State of the art in natural killer cell malignancies.
No full textThe recently updated World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues, published in 2008, has made great advances in revising the disorders previously included in the pool of natural killer (NK) cell tumors. Although NK cell neoplasms represent a relatively rare group of diseases, accounting for <5% of all lymphoid neoplasms, they include very distinctive conditions both clinically and pathologically. This family of diseases includes the most indolent clinical forms, such as the provisional new entry of chronic lymphoproliferative disorder of NK cells (CLPD-NK) in the WHO classification, as well as one of the most fatal diseases recognized in medical oncology, aggressive NK cell leukemia (ANKL), which is characterized by a prognosis of weeks, or even days. In addition, some disorders previously identified as blastic NK cell lymphoma within the NK cell system have been more properly defined and included in the blastic plasmacytoid dentritic cell neoplasms, although rare cases of bona fide immature NK lymphoid tumors (now classified as NK cell lymphoblastic leukemia/lymphoma) have been reported in the literature. This paper focuses on recent concepts and progress in morphology, pathogenesis, clinicopathological features, treatment approaches, and outcomes of NK cell malignancies
Neurophysiological and clinical responses to rituximab in patients with anti-MAG polyneuropathy.
No full textThe acquired immunodeficiency syndrome (AIDS): Insights into the immunopathogenesis of the pulmonary involvement.
No full textThe lung is involved in more than 50 per cent of patients with the acquired immune deficiency syndrome (AIDS), and pulmonary abnormalities can be easily investigated using the bronchoalveolar lavage (BAL). Besides providing information on the type of infecting microorganism, BAL has been used to characterize immunocompetent cells from the lower respiratory tract in these patients. This allows new insights into the immunopathogenetic mechanism involved in the persistence of opportunistic pulmonary infections and in the uncontrolled viral replication. This paper emphasizes the value of BAL evaluation as a simple and useful method for investigating the involvement of the distal respiratory tract in AIDS patients. A particular attention is payed on the immunological pulmonary abnormalities of this epidemic immunodeficiency syndrome
Steroid-responsive hyperammonemic encephalopathy as first manifestation of multiple myeloma
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HIV-1 and the lung. Infectivity, pathogenic mechanisms and cellular immune responses taking place in the lower respiratory tract.
No full textT-lymphocytes with gamma/delta T cell receptors in patients with AIDS and Pneumocystis carinii pneumonia.
No full textLenalidomide for bortezomib- resistant Multiple Myeloma
No full textWe read with great interest the paper by Ludwig and Zojer reporting a patient with bortezomib-resistant multiple myeloma who achieved renal recovery after lenalidomide therapy. A similar case was reported in 2009 by Layzer and Wolf in a patient with multiple myeloma and refractory polyneuropathy whose condition significantly improved after treatment with lenalidomide.The reduced neurotoxic effect of lenalidomide compared with thalidomide is well known, and results from a retrospective study reported that six of nine patients with bortezomib-induced neuropathy had symptomatic improvement after therapy with lenalidomide.
In line with these encouraging data, we would like to share our group’s preliminary experience with lenalidomide in patients with chemotherapy-induced peripheral neuropathy (CIPN)
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