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PubMed1993

Primary immunodeficiencies of food animals.

Although there are few, well-characterized PIDs of food animals, these diseases are important because they tend to be severe and with no cure. Most animals with PID do not receive the intensive and aggressive care required for survival: Veterinarians may be consulted only when the animals are in the terminal stages of illness; it is generally not economically practical for livestock producers or practitioners to pay for the exhaustive laboratory tests required to detect and characterize these anomalies. Another reason for the small numbers of characterized clinical cases of PID is that they are rare. It is possible, however, that intensive artificial insemination and embryo transfer could select for heterozygous carriers of these autosomal traits. As seen with bovine leukocyte adhesion deficiency, as the frequency of an allele increases in the population, the numbers of affected animals increase. Furthermore, other immunodeficient syndromes are likely to exist. Veterinarians therefore should be aware of these disorders and should seek laboratory assistance to arrive at a correct diagnosis. Because of the inheritable nature of PID, livestock producers need assistance from veterinarians to identify carriers and establish sound breeding and control programs. One positive outcome from studies of PID is that research scientists and veterinarians learn much about immune systems from these afflicted animals. In fact, these animals may become models for gene therapy or marrow reconstruction procedures.

Animals↗
PubMed2001

Normal and abnormal secretion by haemopoietic cells.

The secretory lysosomes found in haemopoietic cells provide a very efficient mechanism for delivering the effector proteins of many immune cells in response to antigen recognition. Although secretion shows some similarities to the secretion of specialized granules in other secretory cell types, some aspects of secretory lysosome release appear to be unique to melanocytes and cells of the haemopoietic lineage. Mast cells and platelets have provided excellent models for studying secretion, but recent advances in characterizing the immunological synapse allow a very fine dissection of the secretory process in T lymphocytes. These studies show that secretory lysosomes are secreted from the centre of the talin ring at the synapse. Proper secretion requires a series of Rab and cytoskeletal elements which play critical roles in the specialized secretion of lysosomes in haemopoietic cells.

Blood Cells↗
PubMed1977

Thymic activity in severe combined immunodeficiency diseases.

Thymic function was evaluated by quantitation of circulating thymic factor in patients with several forms of severe infantile immunodeficiency diseases. Direct quantitation of thymic factor in serum of patients with severe combined immunodeficiency revealed heterogeneity of this syndrome by this parameter, as was also shown by study of susceptibility of the marrow cells to differentiation in vitro. Thymic factor was not detectable in one patient with severe combined immunodeficiency, but was present in normal or near-normal concentrations in three others. Circulating levels of this hormonal activity were also not detectable in a patient with DiGeorge athymic syndrome. Following marrow or fetal liver transplantation, which corrected the severe combined immunodeficiency thymic factor levels either increased slightly or did not change appreciably. Fetal thymic transplantation, which together with fetal liver transplantation corrected the immunodeficiency in one patient with severe combined immunodeficiency, was associated with increase of thymic factor to normal levels. Fetal thymus transplantation alone, which was employed to correct the immunodeficiency of DiGeorge athymic syndrome, caused an increase in thymic factor activity to normal or near normal levels in this patient.

Bone Marrow Cells↗
PubMed2004

Guillain-Barre-Strohl syndrome unraveled as paraneoplastic syndrome of B-cell acute lymphoblastic leukemia in a patient with preceding common variable immunodeficiency syndrome with Evans syndrome.

A 26-year-old man with a history of common variable immunodeficiency and Evans syndrome (immunthrombocytopenia and immunhemolytic anemia) with sarcoid like lesions and lymph node enlargements in the previous history is described. The patient presented with symptoms resembling Guillain-Barre-Strohl as paraneoplastic syndrome just before the diagnosis of acute lymphoblastic leukemia of the L3 type. The patient was treated according to the B-ALL protocol of the German ALL study group and achieved a complete response after six cycles of chemotherapy together with a resolution of all neurologic symptoms.

Adult↗
PubMed2001

Rab27a is required for regulated secretion in cytotoxic T lymphocytes.

Rab27a activity is affected in several mouse models of human disease including Griscelli (ashen mice) and Hermansky-Pudlak (gunmetal mice) syndromes. A loss of function mutation occurs in the Rab27a gene in ashen (ash), whereas in gunmetal (gm) Rab27a dysfunction is secondary to a mutation in the alpha subunit of Rab geranylgeranyl transferase, an enzyme required for prenylation and activation of Rabs. We show here that Rab27a is normally expressed in cytotoxic T lymphocytes (CTLs), but absent in ashen homozygotes (ash/ash). Cytotoxicity and secretion assays show that ash/ash CTLs are unable to kill target cells or to secrete granzyme A and hexosaminidase. By immunofluorescence and electron microscopy, we show polarization but no membrane docking of ash/ash lytic granules at the immunological synapse. In gunmetal CTLs, we show underprenylation and redistribution of Rab27a to the cytosol, implying reduced activity. Gunmetal CTLs show a reduced ability to kill target cells but retain the ability to secrete hexosaminidase and granzyme A. However, only some of the granules polarize to the immunological synapse, and many remain dispersed around the periphery of the CTLs. These results demonstrate that Rab27a is required in a final secretory step and that other Rab proteins also affected in gunmetal are likely to be involved in polarization of the granules to the immunological synapse.

Actin-Related Protein 2↗
PubMed1988

Role of imaging and interventional techniques in the diagnosis of respiratory disease in the immunocompromised host.

A pure "pattern-recognition" approach to lung diseases in immune compromised patients as seen on the chest film is of limited value since any infection can present with several different patterns, and processes such as embolism, aspiration, edema, and hemorrhage may give appearances similar to infection. However certain pulmonary infections do occur commonly in association with one type of immunosuppression but rarely with others. Knowledge of these associations assists in narrowing down the differential diagnosis and in deciding upon the most appropriate next confirmatory diagnostic step.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Radiology of immunologic diseases of the lung.

In the last two decades research in immunology has greatly expanded our knowledge of this important medical specialty. Many diseases of previously unknown etiology are now much better understood. This review emphasizes the basics of immunology in order to assist the reader in understanding the mechanisms that cause immunologic lung disease. The radiologic appearance of these diseases is discussed.

Acquired Immunodeficiency Syndrome↗
PubMed1982

Induction of E-rosette-promoting factor in human plasma by levamisole: an assessment in a patient with partial DiGeorge syndrome.

A male infant with partial DiGeorge syndrome responded to weekly administration of levamisole (2.5 mg/kg of body weight) with an increase of circulating E-rosette-forming T cells. Thymic hormone activity in plasma appeared to be elevated to a near-normal level of 11.6 ng thymopoietin equivalent/ml after levamisole administration. The in vitro incubation studies indicated that levamisole by itself had no E-rosette-promoting ability, but a dialysable and relatively heat-stable plasma factor induced by levamisole both in the patient and in healthy individuals had E-rosette-promoting activity for the patient's lymphocytes. Such a plasma factor, however, could not be induced in all four thymectomized myasthenic subjects examined, suggesting a thymus-dependent nature of the plasma factor. These results suggest that levamisole might mediate an increased secretion of humoral factor(s) with E-rosette-promoting activity, even from such a rudimentary thymus as in the partial DiGeorge syndrome.

DiGeorge Syndrome↗
PubMed1984

Immuno-reconstitution by thymic transplant in DiGeorge's syndrome.

Persistent hypocalcaemic tetany in a breast-fed neonate made us suspect DiGeorge's syndrome, particularly as the baby had an abnormal facies, and a ventricular septal defect. Immuno-reconstitution was successfully achieved by thymic transplant. Evidence of immuno-reconstitution on the basis of the histology of the post-transplant thymus has not been previously recorded.

DiGeorge Syndrome↗
PubMed2003

Immunodeficiency disorders.

Hematological complications occur frequently in patients with both primary and secondary immunodeficiency disorders. Anemia, thrombocytopenia or leukopenias may bring these individuals to the attention of hematologists. Conversely, evidence suggesting a lymphoproliferative disorder may be the cause for referral. This session will provide an update on the diagnosis and treatment of immunodeficiency diseases ranging from isolated defects in antibody production to the severe combined immunodeficiencies (SCID). Immunodeficiency diseases have traditionally been defined as defects in the development and function of T and B cells, the primary effector cells of specific cellular and humoral immunity. However, it has become increasingly evident that innate immune mechanisms contribute greatly to host defense, either through acting alone or by enhancing specific T and B cell responses. In Section I, Dr. Lewis Lanier reviews the burgeoning information on the extensive families of activating and inhibitory immunoreceptors that are expressed on NK cells, dendritic cells, T and B cells, and phagocytic cells. He provides an overview on the biological functions of these receptors in host defense. In Section II, Dr. Mary Ellen Conley defines the spectrum of antibody deficiency disorders, the most frequently occurring types of primary immunodeficiencies. She covers the different defects in B-cell development and function that lead to antibody deficiencies, and includes diagnosis and therapy of these disorders. In Section III, Dr. Jennifer Puck discusses the diagnosis and treatment of the different types of SCID. She describes the genetic basis for SCID, and the benefits, pitfalls, and complications of gene therapy and bone marrow transplantation in SCID patients.

Humans↗
PubMed1986

Polymyositis in an immunodeficiency disease in monkeys induced by a type D retrovirus.

Fifty percent of primates with acquired immunodeficiency caused by a well-characterized type D retrovirus (SAIDS D) developed clinical, laboratory, and histologic features of polymyositis. By use of specific antisera and immunochemical techniques, we found the virus in the lymphoid cells surrounding muscle fibers and invading the endomysia septa. SAIDS D virus was isolated from the involved muscles and infected myotubes of normal muscle in tissue culture. These results suggest that retroviruses, a group of viruses increasingly associated with human diseases, can cause polymyositis with immunodeficiency in nonhuman primates and could play a role in human polymyositis.

Acquired Immunodeficiency Syndrome↗