ABSTRACT
BACKGROUND: Neurolymphomatosis (NL) is a rare clinical entity characterized by infiltration of malignant lymphocytes into the peripheral nervous system. We analyzed the clinicoradiological features, treatments, and outcomes in NL patients. METHODS: We identified six patients with NL seen at The University of Texas MD Anderson Cancer Center from 01/2010 to 10/2012. We extracted clinical presentations, imagings, CSF cytology, and electrodiagnostic studies from medical records. One patient had a nerve biopsy. We defined therapy response as clinical improvement of neurological deficits. FINDINGS: The mean age at onset was 57.1 years. Most were predominantly men with non-Hodgkin lymphoma. Positron emission tomography (PET) was positive in five patients. Nerve conduction demonstrated mononeuritis multiplex, plexopathy, demyelination, and axonal polyradiculoneuropathy, whereas electromyography was nonspecific. All patients received systemic chemotherapy, four intrathecal chemotherapy, and three intravenous immunoglobulin, plasma exchange or both. One patient who received intravenous immunoglobulin showed mild neurological improvement. Two patients responded, and the median overall survival was 15 months. CONCLUSIONS: NL is an increasingly recognized complication of NHL and leukemia. A high clinical suspicion is necessary for correct diagnosis. In the present series, patients with leukemia had mononeuritis multiplex, whereas those with lymphoma had plexopathy. Electrodiagnosis and PET scans were useful diagnostic tools. No factors correlated with poorer prognosis. International collaborative studies will help to better determine the risk factors of NL, response to treatment and outcomes.
Subject(s)
Marek Disease/diagnosis , Marek Disease/therapy , Treatment Outcome , Adult , Aged , Animals , Birds , Brachial Plexus/pathology , Female , Fluorodeoxyglucose F18 , Humans , Immunoglobulins, Intravenous/therapeutic use , Magnetic Resonance Imaging , Male , Marek Disease/complications , Marek Disease/diagnostic imaging , Middle Aged , Plasma Exchange , Positron-Emission TomographyABSTRACT
Marek's disease virus (MDV) encodes a basic leucine zipper oncoprotein, Meq, which structurally resembles jun/fos family of transcriptional activators. It has been clearly demonstrated that deletion of Meq results in loss of transformation and oncogenic capacity of MDV. The rMd5ΔMeq virus provided superior protection than CVI988/Rispens vaccine in 15×7 chickens when challenged with a very virulent plus (vv+) strain of MDV, 648A. The rMd5ΔMeq construct was also shown to be an effective vaccine in commercial chickens that were challenged under field conditions by exposure to seeder chicken inoculated with MDV strain 686, a vv+ and arguably the most pathogenic strain of MDV. Although deletion of Meq gene renders the virus non-oncogenic, it still induces lymphoid organ atrophy like that of the parental rMd5, in highly susceptible MDV maternal antibody negative (MAb-) chickens. We have generated 50 cell culture passages of attenuated rMd5ΔMeq viruses and found no significant lymphoid organ atrophy beginning at 40(th) passage onward when compared with the normal control chickens. The protective ability of these attenuated Meq null viruses against challenge with vv+ MDV strain 686 is similar to the original virus at 19(th) passage in maternal antibody negative chickens. The data indicate that attenuation of these Meq null viruses has no influence on their protective efficacy, but eliminated lymphoid organ atrophy and rendered them safe to use even in MAb- chickens, a characteristic that should facilitate commercialization and licensing by vaccine manufacturers.
Subject(s)
Marek Disease Vaccines/immunology , Marek Disease/therapy , Oncogene Proteins, Viral/metabolism , Spleen/immunology , Thymus Gland/virology , Animals , Antibodies, Viral/immunology , Bursa of Fabricius/immunology , Bursa of Fabricius/pathology , Bursa of Fabricius/virology , Cells, Cultured , Chickens/immunology , Chickens/virology , Female , Gene Deletion , Gene Dosage , Genome, Viral , Immunohistochemistry , Male , Marek Disease/immunology , Marek Disease/virology , Marek Disease Vaccines/genetics , Oncogene Proteins, Viral/genetics , Oncogene Proteins, Viral/immunology , Organ Size , Recombinant Proteins/genetics , Recombinant Proteins/immunology , Recombinant Proteins/metabolism , Spleen/pathology , Spleen/virology , Thymus Gland/immunology , Thymus Gland/pathology , Virus Activation , Virus Cultivation/methodsABSTRACT
Gallinas ponedoras criadas de manera familiar, junto a otras especies, son tratadas homeopaticamente por presentarse enfermas en dos oportunidades distintas. Para la repertorizacion fue utilizado el Moderno Repertorio de Kent. Las palomas de un colombofilo afectadas de una enfermidad respitaroia cronica y tratadas con medicina tradicional sin exito, superaron el trastorno luego de recibir medicamentos homeopaticos
Subject(s)
Animals , Bird Diseases/therapy , Columbidae , Chickens , Marek Disease/therapy , Respiratory Tract Diseases/therapy , Avian Leukosis/therapyABSTRACT
Gallinas ponedoras criadas de manera familiar, junto a otras especies, son tratadas homeopaticamente por presentarse enfermas en dos oportunidades distintas. Para la repertorizacion fue utilizado el Moderno Repertorio de Kent. Las palomas de un colombofilo afectadas de una enfermidad respitaroia cronica y tratadas con medicina tradicional sin exito, superaron el trastorno luego de recibir medicamentos homeopaticos
Subject(s)
Animals , Bird Diseases/therapy , Chickens , Columbidae , Avian Leukosis/therapy , Marek Disease/therapy , Respiratory Tract Diseases/therapyABSTRACT
Gallinas ponedoras criadas de manera familiar, junto a otras especies, son tratadas homeopaticamente por presentarse enfermas en dos oportunidades distintas. Para la repertorizacion fue utilizado el Moderno Repertorio de Kent. Las palomas de un colombofilo afectadas de una enfermidad respitaroia cronica y tratadas con medicina tradicional sin exito, superaron el trastorno luego de recibir medicamentos homeopaticos
Subject(s)
Case Reports , Animals , Bird Diseases/therapy , Chickens , Columbidae , Avian Leukosis/therapy , Marek Disease/therapy , Respiratory Tract Diseases/therapyABSTRACT
Marek's disease virus (MDV)3 is a highly oncogenic herpesvirus that usually causes visceral lymphomas and lymphoid infiltration of the peripheral nerves in chickens. A relatively rare encephalitic condition, first found in farm flocks and referred to as transient paralysis (TP), is also caused by MDV(1). TP symptoms occur 9 to 11 days after MDV inoculation and range from mild ataxia to profound coma. Most birds recover by 24 to 72 hr after onset of symptoms, although severely affected birds may die within the same time period. Previous studies in this laboratory (2) showed that susceptibility to TP is a recessive trait controlled by major histocompatibility complex (MHC) genes (i.e., B complex genes of chickens). Inbred line G-B1 chickens (B13/B13) are resistant to TP, whereas chickens from related inbred lines G-B2 (B6/B6) and G-B3 (B15/B15) are highly susceptible. In this study chickens were immunosuppressed by neonatal cyclophosphamide (CY) treatment or surgical bursectomy (BX) to determine the possible role of antibodies in the pathogenesis of TP.
Subject(s)
Immunosuppressive Agents/administration & dosage , Marek Disease/immunology , Animals , Antibody Formation/drug effects , Bursa of Fabricius/surgery , Chickens , Cyclophosphamide , Herpesvirus 2, Gallid/immunology , Immune Tolerance , Marek Disease/therapyABSTRACT
A study of nonspecific stimulation of the avian immune system with Corynebacterium parvum and specific stimulation with Marek's tumor cell vaccines revealed that nonspecifically stimulated outbred White Leghorn-type cockerels had higher incidences of tumors than did controls. A study of tumor cell cytotoxicity of sera from Marek's disease virus exposed birds indicated that humoral factors may play some role in tumor resistance.
