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[Immunological monitoring of patients operated on for epidermoid bronchogenic carcinoma].

Sequential monitoring of non specific immunity has been carried out in 16 patients with squamous cell bronchogenic carcinoma undergoing radical surgery. At the time of diagnosis total lymphocyte counts, T-lymphocyte counts and the blastogenic response of lymphocytes to PHA were only slightly depressed; on the contrary C3c and C4 serum levels were significantly increased (P less than 0.01). Sequential determinations of the immunologic profile at monthly intervals up to the 12th postoperative month showed a transient increase of total lymphocyte and T-lymphocyte levels; the lymphocyte blastogenic response to PHA and also C3c and C4 serum levels progressively decreased. Delayed hypersensitivity response to cutaneous antigens, neutrophil chemotaxis, serum levels of IgG, IgA, IgM and B-lymphocyte counts were constantly found within normal range.

Adult↗

[Kidney involvement in "mixed connective tissue disease" (Sharp syndrome)].

The case in question of the Sharp-syndrome is a mixed collagenosis with symptoms of sclerodermia, Erythematodes visceralis, dermatomyositis and rheumatoid arthritis. Apart from the clinical symptomatology the immunological profile is decisive for the ascertainment of the diagnosis. With the help of 3 descriptions of cases is referred to a frequent participation of the kidneys, particularly histologically and immune-histologically. In contrast to the hitherto existing publications also in missing pathological findings of the urine a renal biopsy should be aspired to, since important therapeutic and prognostic considerations are dependent on this.

Adult↗

Proposals for the immunological classification of acute leukemias. European Group for the Immunological Characterization of Leukemias (EGIL).

Criteria for the immunological classification of acute leukemias are proposed by a recently established European group designated EGIL. The main aims of EGIL are to establish guidelines for the characterization of acute leukemias based on marker expression and provide a uniform basis for the diagnosis of the various types of these hemopoietic malignancies which should be helpful for future multinational clinical and laboratory investigations. Within the two major types (B and T cell lineage) of acute lymphoblastic leukemia (ALL), several groups are delineated according to the degree of cell differentiation. Within the acute myeloid leukemias (AML), only three subtypes as defined by the FAB classification: M0-AML, M6-AML and M7-AML, can be unequivocally defined by immunological markers; prospective studies are undertaken to see whether characteristic immunological profiles are associated with particular AML subtypes defined by specific cytogenetic abnormalities. Criteria for the definition of biphenotypic acute leukemia (BAL) are devised and a scoring system is outlined aimed to distinguish BAL from those acute leukemias with expression of a marker from another lineage. In addition, an uncommon subset of acute leukemias with no evidence of lymphoid or myeloid differentiation is recognized and the useful panel of markers to investigate and establish the cell nature of the acute leukemias is outlined. EGIL will focus in the future on testing the reproducibility of the proposed guidelines, particularly those for BAL, assessing their clinical value within a framework of multicentric trials and setting up uniform methodological criteria.

Acute Disease↗

The immunophenotype of hairy cell leukemia (HCL). Proposal for a scoring system to distinguish HCL from B-cell disorders with hairy or villous lymphocytes.

We have analysed the immunological profile of cells from 194 patients with a B-cell disorder associated with circulating hairy or villous lymphocytes. These included: 69 typical HCL, 25 HCL-variant and 100 splenic lymphoma with villous lymphocytes (SLVL). All cases were HLA-DR+, CD19+, CD2- and most expressed Smlg with light chain restriction. The kappa/lambda ratios were: HCL, 1.2; SLVL, 1.5; and HCL-variant, 0.55. The majority were FMC7+ (89-96% of cases) and membrane CD22+ (73-98% of cases) and often negative with CD5 and CD23, markers characteristic of chronic lymphocytic leukemia. CD24 was variably expressed ranging from 21% of cases in HCL-variant to 60% in HCL and 89% in SLVL; CD10 and CD38 were positive in one third of SLVL but usually negative in HCL and HCL-variant. Of the four markers considered typical of HCL, CD11c, CD25, HC2 and B-ly-7, CD25 and HC2 were consistently negative in HCL-variant and a minority of SLVL cases expressed CD25 or HC2+ or B-ly-7+; CD11c was positive in all three disorders (47 to 97% of cases). Based on the most common phenotype of typical HCL: CD11c+, CD25+, HC2+ and B-ly-7+, we propose a scoring system which considers the reactivity with each of these four markers and gives 1 point if positive and 0 points if negative. Scores range from 4 (typical of HCL) to 0 (atypical of HCL). 98% of HCL had high scores (3 or 4) whereas 88% of HCL-variant and 77% of SLVL scored 1 or 2 and no single case scored 3 or 4.(ABSTRACT TRUNCATED AT 250 WORDS)

B-Lymphocytes↗

[Interferon gamma as an immunological strategy for the treatment of human leishmaniasis].

T lymphocytes from patients with visceral leishmaniasis treated in vitro with leishmania antigens are unable to proliferate and to produce gamma interferon. These patients have negative specific skin tests. Opposite results are obtained in patients with another clinical form of the disease named mucocutaneous leishmaniasis, in which both tests are positive. Nevertheless, patients with visceral leishmaniasis or mucocutaneous leishmaniasis, refractory to chemotherapy (antimonium complex), were cured when treated with antimonium in combination with gamma interferon, in spite of different immunological profiles. Different interpretative hypotheses of the reversion of chemoresistance induced by gamma interferon are discussed.

Adolescent↗

[Idiopathic CD4+ T-lymphocytopenia in 2 patients without indications for HIV infection].

We report on two patients with idiopathic CD4+ T cell depletion. A 26 year-old woman presented to us with acute respiratory failure requiring mechanical ventilation. Despite combined antibiotic therapy parenterally the opacities increased in the chest X-ray. An open lung biopsy was performed and led to the histological diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP). Respiratory function was improved impressively by high dose parenteral cortisone administration. This patient showed a general lymphocytopenia with severe CD4+ T cell depletion (60(37%)/mm3 blood). The CT4+ T cell concentration increased during a follow up period of 14 months, but did not reach normal values. The second patient was a 33 year-old homosexual. He was admitted with a mucocutaneous fungal infection which was successfully treated by antifungal drugs. This patient demonstrated a transient CD4+ T cell depletion (350(32%)/mm3 blood). In both patients HIV type 1 and 2 infections were excluded by antibody- and p 24-antigen testing, polymerase chain reaction and virus culture. CONCLUSION. Idiopathic CD4+ T lymphocytopenia differs from HIV infection in immunological profile, in the tendency to reversal of the CD4+ T cell depletion over time and in its better prognosis. It is unclear if this is a new syndrome and whether a transmissible agent, or possibly a genetically-determined reaction to noxious agents is responsible.

Adult↗

Up-regulation of T helper 2 and down-regulation of T helper 1 cytokines during murine retrovirus-induced immunodeficiency syndrome enhances susceptibility of a resistant mouse strain to Leishmania amazonensis.

Resistance to and recovery from leishmania infection is dependent on cell-mediated immunity. C57BL/6 mice are resistant to Leishmania amazonensis (La) infection but susceptible to LP-BM5 murine leukemia virus (MuLV) infection. MuLV infection leads to a state of immunodeficiency characterized by severe compromise of cell-mediated immunity. When infected with La alone, C57BL/6 mice developed a small transient lesion that evolved to spontaneous healing or a lesion with extremely slow growth. Lesions were predominantly comprised of a lympho-macrophagic infiltrate with few parasitized macrophages. When infected with La and, 4 weeks later, with MuLV (La-MuLV), the mice developed a large uncontrolled nonhealing lesion containing vacuolated and heavily parasitized macrophages. In contrast, mice infected with MuLV first and La 4 weeks later (MuLV-La) developed a small but persistent lesion, characterized histologically by a small number of heavily parasitized macrophages and few lymphocytes. Eight weeks after MuLV infection, both had similar immunological profiles with decreased lymphocyte proliferation, diminished production of interferon-gamma, and high production of interleukins 4 and 10. At the time of L. amazonensis infection, La-MuLV animals have a normal T cell function whereas in MuLV-La mice this function is already impaired; this may influence the recruitment of macrophages to the site of leishmania injection.

Animals↗

The clinicopathological spectrum of renal amyloidosis.

BACKGROUND: Renal amyloidosis is an uncommon cause of nephrotic syndrome. The clinical conditions in Chinese people remain obscure. This is a retrospective review of the clinicopathological spectrum of 12 cases diagnosed in Taichung Veterans General Hospital from October 1982 to November 1993. METHODS: Charts and renal pathological slides were reviewed retrospectively. A scoring system was set to evaluate the degree of amyloid deposition in the renal tissue. The clinical profile, immunological data, pathological picture and final outcomes are presented and discussed, with literature review. RESULTS: There were 12 cases of primary amyloidosis including 1 case of multiple myeloma. All were confirmed by renal biopsy. The cases were all male with mean age of onset as 53.3 +/- 11.3 (range: 32 to 65 years). The mean follow-up duration was 22.9 +/- 32.8 months. The initial average creatinine clearance was 66.7 +/- 42.7 ml/min; mean daily urine protein was 7.0 +/- 4.1 grams. Nephrotic syndrome was the main clinical manifestation, present in all 12 cases. Other presenting symptoms and signs included: malaise in 7 cases; hypertension and anorexia in 4 cases; limb numbness in 3 cases; low back pain, dizziness and microhematuria in 2 cases each; anemia, headache, stroke, restrictive cardiomyopathy, hepatomegaly, syncope, body weight loss, dysphagia and skin itching in one case individually. Amyloid cardiomyopathy was present in 4 of the 8 patients who received echocardiography. The mean serum albumin level was 1.9 +/- 0.7 mg/dl, globulin level 3.1 +/- 1.1 mg/dl. Urinary Bence-Jones protein examination was performed in 8 cases; none revealed positive response. The mean immunoglobulin (Ig) level for the patients included: IgG 1018 +/- 901 mg/dl, IgA 262.0 +/- 313.8 mg/dl, IgM 104.8 +/- 84.2 mg/dl. There were at least 4 cases with high levels of one Ig but depressed levels in the others. M-component was shown by immunoelectrophoresis (IEP) in 90% of the cases. IEP impression in 10 cases revealed 2 cases of IgA lambda, 2 cases IgA kappa, 3 cases IgG lambda and 1 case IgG kappa monogammopathy, 1 case free lambda myeloma and 1 case negative. The ratio of kappa to lambda chain was 3:6. Bone marrow biopsy performed in 8 cases found only 1 case with multiple myeloma, one with amyloidosis; the other 6 cases were unremarkable. Mesangium was the site of heaviest amyloid deposition, followed by tubular basement membrane, artery and interstitium. The median survival time for those whose total score was lower than 3 points was 97.5 months; 3 to 5 points, 14 months; 6 points or more, 18.5 months. The median survival time was 21.6 months and 3-year-survival rate was 32.7%. The 2 cases with long-term survival were of 111 months and 84 months. The possible reason included: 1) Organ-limited renal amyloidosis; 2) Light amyloid deposition; 3) Younger age; 4) Other undetected favorable factors. CONCLUSIONS: 1) Renal amyloidosis is not a frequent diagnosis of nephrotic syndrome in Taiwan, but it should be suspected in every patient over 50 years old with a recent onset of proteinuria. 2) Renal amyloidosis can be diagnosed only by renal biopsy. 3) Primary renal amyloidosis is a disease of poor prognosis.

Adult↗

The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders.

Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade disorder that regularly presents with peripheral blood involvement. We describe the immunophenotype of the circulating cells from 100 SLVL patients whose disease has been characterized on clinical, morphologic, and histologic grounds. Cells from all cases expressed B-cell antigens (CD19 and CD37) and/or HLA-Dr and showed light chain restriction (kappa/lambda: 1.5/1) with moderate to strong intensity of membrane Ig staining. Cells from most cases (> 80%) were CD24+, FMC7+, and expressed strongly membrane CD22. The monoclonal antibodies CD10, CD23, and CD38 were positive in one-third of the cases; CD11c in 47%; and CD25 in 25% of cases. A minority of cases (< 20%) were positive with HC2, B-ly-7, and CD5. However, none of the 19 CD5+ cases had the phenotype characteristic of chronic lymphocytic leukemia (CD5+, CD23+, FMC7-, weak surface Ig and membrane CD22). None of the 17 CD25+ cases had the immunophenotype typical of hairy cell leukemia (CD25+, CD11c+, HC2+, B-ly-7+). HC2 and B-ly-7 were the most useful reagents to distinguish SLVL from hairy cell leukemia. Our findings demonstrate that SLVL has a distinct immunologic profile and that monoclonal antibodies are important for the differential diagnosis between this disease and other B-lymphoproliferative disorders with which SLVL can be confused.

Adult↗

[Immunoglobulin E and acute myocardial infarct].

The authors examined repeatedly the immunoglobulin E (IgE) and immunoglobulin G (IgG) level in patients hospitalized on account of acute myocardial infarction (AMI). In a group of 33 patients aged 40-59 years (25 men, and 8 women) the authors investigated the case-history for hay fever, allergic rhinitis, eczema, allergy to grass, sensitizing household dust or domestic animals. The authors assessed also the presence of asthma and eczema in parents or relatives. Furthermore the authors investigated the relationship of different components of the immunological profile in relation to IgE and AMI. They found that in AMI the IgE serum level rises or is high on the 5th day after acute myocardial ischaemia. On the 10th and 21st day of hospitalization the IgE level declined and by the end of the third week it was close to baseline values of 300 U/ml or less. The IgG level displayed an almost indirect relation, i.e. during the first days it was close to the borderline of normal values, while it increased significantly on the 21st day of hospitalization. The rise of the IgG level is a response of the immune system to recovery of the necrotic myocardial mass. Consistent with some authors abroad, the authors found that patients with AMI with a high initial IgE serum level have a better prognosis quo ad vitam.

Adult↗

Misunderstanding in the classification of diabetes mellitus. What's in a name?

To assess whether physicians, residents, medical students, hospital diagnosis coders, and patients properly use the designations insulin-dependent diabetes mellitus (IDDM) and non-insulin-dependent diabetes mellitus (NIDDM) that were established by criteria of the National Diabetes Data Group, we reviewed clinic and hospital records and administered questionnaires. Although essentially all cases of true IDDM were identified as such and most cases of NIDDM not requiring insulin therapy were correctly identified by all groups, patients with NIDDM on insulin therapy were misidentified as having IDDM by 38% of residents in internal medicine clinics and 68% of primary care and surgical subspecialty residents. On a survey, of 22 patients with NIDDM on insulin therapy, 17 (77%) considered themselves to have IDDM. Thus, patients who have NIDDM by the established criteria who are on insulin therapy are commonly mislabeled as having IDDM. We present an approach for dealing with this problem by adapting nomenclature focusing on insulin deficiency and resistance. It would probably also be helpful to separately identify the subset of patients with "insulin-deficient diabetes" who are ketosis-prone. It is important to use immunologic profiling (islet cell antibody testing) and insulin sensitivity or deficiency testing (C-peptide levels).

Adult↗

Epidemioligical particularities of influenza outbreaks in a town of Romania during 1975.

Results are presented of the complex epidemiological influenza surveillance in a large town of Romania during 1975. The comprehensive and unitary methodology including systematic laboratory, clinical and epidemiological investigations supplied data on the influenza type A outbreak recorded during the first term of 1975. The factors of importance for epidemiological prognosis -- seroepidemiological indicators of the immunological profile of the population according to age groups, serodiagnostic and virological investigation clinically diagnosed influenza cases, complex epidemiological surveys in representative foci, study of the antigenic structure of the influenza strains isolated -- are outlined. The importance of a continuous, complex influenza surveillance for the differential orientation of prophylaxis and control steps is emphasized.

Adolescent↗

The first one hundred AIDS cases in Hong Kong.

OBJECTIVE: To study the clinical and epidemiological characteristics of the first 100 reported AIDS cases in Hong Kong. PATIENTS AND METHODS: The case records of the first 100 AIDS cases reported to the Department of Health were retrieved. An analysis was made on their demographic data, clinical and immunologic profile, treatment received as well as mortality. RESULTS: The AIDS patients were reported over a ten-year period, from February 1985 to March 1994. The majority of them were male, Chinese and between the age of 20 and 49. More than 80% acquired HIV via sexual contact. The most common primary AIDS- defining illness was Pneumocystis carinii pneumonia (PCP), which occurred in 46 patients. Eight episodes of Penicillium marneffei infection have been recorded. Fifty-nine patients have received antiretroviral therapy whereas PCP prophylaxis was given to 47 patients. The mean CD4 count at HIV and AIDS diagnosis was 394/microliters and 121 /microliters respectively. Sixty-seven patients were known to have died at the time of the analysis. PCP was the most common cause of death, accounting for over one-fifth of the cases. The median survival after AIDS diagnosis was less than 2 months for cases reported in 1985-1987 and over 15 months for those of 1990-1991. CONCLUSION: Pneumocystis carinii pneumonia was the most common initial AIDS-defining disease and cause of death in Hong Kong. Survival of the patients has gradually improved over the past few years.

Acquired Immunodeficiency Syndrome↗

Anticardiolipin and anti-beta 2-GPI are two distinct populations of autoantibodies.

This study has been undertaken to assess whether anticardiolipin and anti-beta 2-GPI are two distinct populations of (auto)antibodies, and to clarify whether the beta 2-GPI region critical for phospholipid binding is also crucial for anti-beta 2-GPI reactivity. Fourteen of the 62 anticardiolipin (aCL) ELISA positive sera (22.6%) were positive for anti-beta 2-GPI by immunoblotting, 42 (67.7%) for aCL using TLC immunostaining. IgG fractions from 5 sera gave the same anticardiolipin reactivity detected by TLC immunostaining in the corresponding sera. All anti-beta 2-GPI-positive sera were reactive with the phenylthiocarbamyl derivative of the protein, indicating that binding of phenylisothiocyanate with lysine residues does not modify the molecule antigenicity. In addition, incubation of IgG fractions with the phospholipid binding site did not modify reactivity with beta 2-GPI. These findings demonstrate that: a) "true" antiphospholipid antibodies are detectable in patients' sera; b) aCL and anti-beta 2-GPI have a different immunological profile; c) the beta 2-GPI phospholipid-binding site is not the region recognized by the antibodies.

Adult↗

Relapsing chronic low-dose corticosteroid-responsive multifocal motor neuropathy with conduction block.

Multifocal Motor Neuropathy (MMN) is a newly described disorder that might mimics the Motor Neuron Disease (MND). MMN is considered by some authors to be an entity distinct from Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP), nevertheless because a pure-motor variant of CIDP is well recognized and CIDP can present Partial Motor Conduction Block (PMCB), several authors believe that MMN can be a variant of CIDP. We describe a 27-year old male with a relapsing chronic low-dose corticosteroid-responsive MMN with conduction block. Our case stresses that MMN includes a wide range of motor neuropathies with different immunological profiles conditioning distinct treatment strategies.

Adrenal Cortex Hormones↗

[Parasitosis in an adult population with chronic gastrointestinal disorders].

We worked with 185 middle-class patients above 18 years of age, both sexes, who presented diarrhea and/or chronic gastrointestinal disorders. The faeces were collected serially in formol 10% and processed in the following way: direct microscopy, with and without wet staining, concentration by Ritchie's method, 1% safranine technique for a specific investigation of Cryptosporidium sp., and faecal sieving macroparasites. Twenty eight point six of the studied patients showed at least one enteroparasite in their faeces, 48 harboured one parasite and 5 harboured two parasites. The following parasites were found and their corresponding percentages in the entire studied population are given below: Blastocystis hominis 15.7%, Giardia lamblia 7.5%, Cryptosporidium sp. 1.6%, Entamoeba coli 3.3%, Chilomastix mesnilii 1.1%, Ancylostoma duodenale-Necator americanus 0.5%, Ascaris lumbricoides 0.5%, Enterobious vermicularis 0.5% y Endolimax nana 0.5%. The most frequently found enteroparasites in the positive patients were B. hominis and G. lamblia. Cryptosporidium sp. was diagnosed in only three patients. The source of infection could be presumed in all of them. The symptomatology coincided with that described for this coccid in the bibliography. In spite of the fact that they were HIV seronegative patients the diarrhea was not self-limiting, but the immunologic profile of their relatives remained unknown and no other cause of immunosuppression could be detected with justified chronicity. The treatment with spiramycin was effective. Giardiasis was found in 17 patients, and the source of infection could not be inferred in any of them. They all had chronic diarrhea and their most frequent symptoms were abdominal pain, metallic taste, flatulency and nausea. Most of these patients were harboured one parasite, and only 2 of them simultaneously presented another faecal parasite associated to G. lamblia. Treatment with metronidazole was successful in all of them. Twenty nine patients were found to have B. hominis. The source of infection could not be inferred, this amoeboid was present as the only parasite in 25 patients. Predominant symptoms were flatulence, abdominal distention and colis. All patients suffered from chronic diarrhea, alternating, in some cases, with constipation. Good therapeutic results were obtained with metronidazole. Considering that one third of the patients examined presented faecal parasites associated to chronic disorders, it is important to insist on the detection of parasites to chronic disorders, it is important to insist on the detection of parasites using appropriate diagnostic techniques since the application of specific therapy made their eradication possible as well as relieving the patients' symptomatology.

Adolescent↗

[The incidence of the antiphospholipid syndrome in the clinical and biological manifestations of systemic lupus erythematosus].

In the present investigation a study was made on the incidence of antiphospholipid syndrome (APS) on clinical and biological manifestations in a series or 32 patients (28 females and 4 males with a mean age of 25 years) diagnosed of SLE (ARA criteria) and APS (Harris criteria) compared with a group of 25 patients (19 females and 6 males with a mean age of 38 years) diagnosed of SLE without APS. This entails a selection from 124 patients diagnosed of SLE, and an incidence of 25.8% and 9.7% for ACA and LA, respectively. After a clinical protocol was filled, a complete immunological profile was obtained, with lymphocyte subsets, IL-2 receptor, coagulation study, isotype determination for anticardiolipin antibody (ACA), lupus anticoagulant (LA), serology for syphilis and imaging diagnostic techniques. Comparative results, with an statistic assessment, are shown in tables. It is concluded that SLE + APS population can be considered as definite for a peculiar SLE subtype.

Adult↗

Aetiopathogenesis and treatment of idiopathic retroperitoneal fibrosis.

Idiopathic retroperitoneal fibrosis (IRF) is a rare urological disease, for which many pathogenic theories have been proposed. The authors report a series of 13 cases of IRF in order to evaluate the clinical, diagnostic, laboratory, therapeutic and prognostic aspects. They also report a rare case (the ninth case reported in the literature) of multifocal fibrosclerosis. A possible genetic predisposition was studied by testing for the presence of immunophenotype HLA-B27; this test was positive in 44% of cases. A study of the immunological profile and lymphocyte populations revealed the typical features of chronic immune disease. Experience with medical and surgical treatment is reported, comparing various procedures: ureterolysis followed by application of a vascularized omental flap over the ureter (without subsequent corticosteroid therapy) gave the best results, with complete resolution of the symptoms and long-term successful alleviation of ureteric obstruction in 100% of patients, with a mean follow-up of 58 months.

Adult↗