Systemic lupus erythematosus presenting with neurological disorders.

Six patients are described who developed a wide variety of neurological manifestations heralding systemic lupus erythematosus (SLE), which included epileptic seizures, stroke, peripheral polyradiculoneuropathy similar to Guillain-Barré syndrome, transverse myelopathy and multifocal disorders with remitting course mimicking multiple sclerosis. The peculiarity of these cases was that the neurological disorders remained the only manifestations of SLE for many years and the nervous system appeared to be the main target even after the development of systemic SLE. In five patients the prognosis was favourable and corticosteroid treatment led to prolonged remission.

T-cell phenotypic profiles in the cerebrospinal fluid and peripheral blood of multiple sclerosis patients.

Thirty-nine patients with clinically definite multiple sclerosis (MS) entered the study. Of 28 subjects with a relapsing-remitting course, 19 were classified in acute relapse, 9 in remission; 11 patients had a progressive course without remissions. Furthermore, 6 subjects with inflammatory neurological disease (IND), and 10 with non-inflammatory and non-neoplastic neurological disease (NIND) were investigated. We simultaneously studied cerebrospinal fluid (CSF) and peripheral blood (PB) T-, B- and NK-cell subsets, as defined by following monoclonal antibodies: anti-CD3, -CD4, -CD8, -CD19, -CD16, -HLA-DR and -IL-2-R. We found a significant increase of CD4+ T-cells compared with controls in CSF, with respect to PB, of MS patients, particularly in acute relapse. An increase of HLA-DR+ cell percentages in the CSF than in the PB in all MS groups, especially in attacks of MS but also in remission, was also observed, with a positive correlation between CD4+ T-cell and DR+ cell percentages both in the CSF as well as in the PB of relapsing MS patients. These findings, together with the increase of IL-2-R+ cells in the PB, particularly in relapsing MS, give further support for the presence of a systemic T-cell activation in MS.

An Italian case of nasopharyngeal carcinoma correlated with Epstein-Barr virus specific antigens.

An Italian case of nasopharyngeal carcinoma was studied clinically; the immunologic study showed the correlation with the Epstein-Barr virus specific antigen.

[Malignant lymphoma present almost simultaneously in two brothers (author's transl)]. / Linfoma maligno in due fratelli.

A diagnosis of poorly differentiated, nodular, lymphocytic malignant lymphoma was made in two brothers. One had been operated 10 years earlier for an adenocarcinoma of the descending colon. In addition, another brother had died from a primary tumor of the liver. Although a genetic factor is not proved, the case is suggestive.

[Dysmyelopoietic syndrome 7 years after melphalan treatment of multiple myeloma. Clinical case]. / Sindrome dismielopoietica sette anni dopo trattamento di mieloma multiplo con melphalan. Caso clinico.

A case of myelodysplasia (refractory anaemia with excessive blasts) arising 7 years after a 3 year period of intermittent monthly treatment cycles with melphalan for multiple myelomas is reported. This is another example of preleukaemic syndrome possibly caused by melphalan. Among the possible pathogenetic mechanisms, the incidence of cyclic episodes of medullary hypoplasia are emphasised.

[Indirect immunoelectrophoresis in the study of urinary proteins]. / L'immunoelettroforesi indiretta nello studio delle proteine urinarie.

The indirect immunoelectrophoresis applied to the proteinurias is a simple and quick method, of usual execution, that makes use of not concentrated urines and discriminant between physiological and pathological proteinurias. The results obtained from a various casistic, collected in many years are presented. For the correlation which exist between morphological isototype and immunoelectropherogram, sometimes, it is possible to go up to the diagnosis of nephropathy also without applying to the renal biopsy.

[The significance of cryoglobulins and cryohemagglutinins. Immunological and clinical study of 39 cases]. / Sul significato delle crioglobuline e delle crioemoagglutinine. Studio immunologico e clinico di 39 casi.

In about 5 years of clinical and immunological research, 39 cases of cryoplasmopathies (5 with cryoglobulinaemia and 34 with cryohaemoagglutinaemia) were identified on the basis of immunological typing studies (immunoelectrophoresis, immunodiffusion, identification and isolation of cryoantibodies, cryocrit, titration, identificcation of antibody specificity). Discussion and physiopathological interpretation of possibly associated cases and diseases are conducted on the basis of personal immunological data and the most recent literature on matters of clinical immunohaematology.

[Prognostic factors and response to chemotherapy in breast cancer. bibliographic review]. / Fattori prognostici e risposta alla chemioterapia nel carcinoma della mammella. Revisione bibliografica.

The recent literature on prognostic factors in breast cancer was examined with regard to: early or delayed diagnosis, age, menopause, tumour site and size, histological type and grading, involvement of lymph nodes, clinical stage, local recurrence, and distant metastasis. With respect to prognostic factors connected with the response to therapy, the following points were considered: menopause, involvement of lymph nodes, dimensions, clinical stage, free interval, performance and loss of weight, particular sites of metastasis, radiotherapy, oestrogen receptors, prior endocrine management, and certain body fluid parameters (haemoglobin, total lymphocytes, platelets, and white cells, albumin, LDH, SGOT, alkaline phosphatase, blood bilirubin and calcium). Radiotherapy appears to make patients less responsive to subsequent antiblastic treatment, whereas premenopausal status, good psychological and physical efficiency, and prior endocrine management seem to play a positive role in the response to chemotherapy.

[Prognosis of chronic lymphatic leukemia. Retrospective study of the survival period 47 patients]. / Sulla prognosi della leucemia linfatica cronica. Studio retrospettivo della durata di sopravvivenza di 47 pazienti.

Forty-seven consecutive patients with chronic lymphocytic leukemia (CLL) followed in the Clinica Medica and the Sezione di Ematologia of Ferrara from January 1966 to June 1979 were classified according to the staging system of Rai and to age, sex and the occurrence of enlarged spleen or liver or both. Analysis of actuarial survival curves revealed the following: 1) the method of staging proposed by Rai was confirmed to be a reliable predictor for prognosis of CLL; 2( neither age nor sex do appear to be prognostic factor; 3) the enlarged liver did not appear to make worse the prognosis of CLL, probably by hepatomegalies due to other aetiologies. Furthermore the bound of peripheral lymphocytosis of 15,000/mm3, also according to other authors, seems to be too high: numerous cases of undoubted CLL with smaller lymphocytosis ae left out of this and other statistics published on CLL.

[Hypocholesterolemia in multiple myeloma. Inverse relation to the component M and the clinical stage]. / Ipocolesterolemia nel mieloma multiplo. Relazione inversa con la componente M e con lo stadio clinico.

The following parameters were studied in 41 Durie & Salmon staged patients with multiple myeloma: M component, bone lesions, marrow plasma cell %, Hb, leukocytes/mm3, lymphocytes/mm3, platelets/mm3, blood calcium, serum albumin, blood creatinine, BUN, total blood cholesterol. Linear correlation was adopted for the relation between two variables, using Pearson's coefficient, and Student's t test for unpaired data for comparison between the means. A significant (slight to excellent) inverse correlation was noted between total blood cholesterol and the M component. There was also a significant difference between the 1st and 2nd stage of the disease in this respect (P less than 0.05), between the 1st and 3rd (P less than 0.01), and between the 1st and the 2nd and 3rd combined (P less than 0.01). Progress of the disease appears to result in a fall in blood cholesterol. This parameter may be of assistance in differentiating slow-progressing forms from the more aggressive forms requiring treatment. The reasons for this change are discussed. Since cholesterol is an essential part of the cell membranes, attention is given to theories postulating a disorder in intracellular membrane metabolism.

[Plasma cell leukemia. Bibliographic review and ultrastructural study (by transmission and scanning electron microscopy) in 2 cases]. / La leucemia plasmacellulare. Revisione bibliografica e studio ultrastrutturale (al M.E.T. ed al M.E.S.) in due casi.

After a brief examination of the recent literature on plasma cell leukemia, the Authors describe the clinical features and the ultrastructural findings of the peripheral blood plasma cells, examined with transmission (T.E.M.) and scanning (S.E.M.) electron microscopes, of two patients with acute plasma cell leukemia. Both of them had a previous history of myeloma. T.E.M. confirmed the diagnostic value of the asynchronous plasma cells, and S.E.M. showed the characteristic microvilli and blebs, previously observed.

[Non-secretory plasmacytoma. Bibliographic review and immunohistochemical study of a case]. / Plasmocitoma non secernente. Revisione bibliografica e studio immunoistochimico di un caso.

After a brief examination of the recent literature on non-secretory multiple myeloma, the Authors describe the immunohistologic study (peroxidase-antiperoxidase method) of a case of truly non producing plasmacytoma, interesting because of the presence of a small polyclonal plasma cell population within the neoplastic clone. Several possible explanations are considered.

[Hyper-IgD syndrome and other hereditary periodic fever syndromes]. / La sindrome da Iper-IgD e altre sindromi febbrili periodiche ereditarie.

Hereditary periodic fever syndromes are a group of systemic disorders characterized by recurrent attacks of systemic inflammation (autoinflammation) without infectious or autoimmune cause. The hyper-IgD syndrome (HIDS) is a rare autosomal recessive inflammatory disorder characterized by recurrent fever, increased serum IgD (normal value < 100 U/ml) and generalized inflammation (lymphadenopathy, arthralgias/arthritis, abdominal complaints, skin rash, and headache). The attacks persist during the entire life although frequency and severity tend to diminish with age. HIDS is caused by specific mutations in the gene encoding mevalonate kinase, resulting in depressed enzymatic activity. At present the therapy for the syndrome is only supportive. Other than HIDS, other hereditary systemic inflammatory disorders have been described: the Familial Mediterranean Fever, the tumour necrosis factor receptor associated periodic syndrome (TRAPS), a disease related to the mutations of one of the TNF receptors, the Familial Cold Urticaria and the Muckle-Wells syndrome. The differential diagnosis with other causes of periodic fever is crucial for assessing appropriate management and treatment.

[Hyper-IgD syndrome (HIDS)]. / La sindrome iper-IgD (HIDS: hyper-IgD syndrome).

In 1984, Van der Meer first reported six patients with a long history of recurrent attacks of fever of unknown cause and a constantly elevated polyclonal IgD (> 100 U/mL); he suggested the acronym of "hyper-IgD syndrome" (HIDS). A recent literature review identified 60 cases (59 from Europe and 1 from Japan). The mean age was 27 years (range: 3-69 years). The family studies have shown a positive family history for periodic fever (40% out of the patients) but not for hyper-IgD. The median age at onset was 0.5 years (range from the first weeks of life to 53 years). The length of the febrile attacks, though variable, lasted from 3 to 7 days. The frequency of the attacks varied among the individual patients, but in general it was once a month or bimonthly. The fever was sustained in all 60 patients (from 38 degrees C to 41 degrees C), with a rapid rose, a plateau and a slow decline to normal values over 5 days. The associated clinical findings involve abdominal symptoms (pain, vomiting, diarrhea), recurrent peritonitis, lymphadenopathy, splenomegaly, articular manifestations (non-destructive recurrent arthritis) and skin lesions (vasculitis). The prognosis is benign. The aetiopathogenesis of HIDS is unknown. The role of IgD in the pathogenesis remains to be elucidated. The therapy is only supportive.

[Risk factors in breast cancer]. / Su alcuni fattori di rischio per il cancro della mammella.

PIP: Incidence of mortality caused by breast cancer goes from 25-30/100,000 in Canada, U.S. and Great Britain, to 3-4/100,000 in Japan. Incidence of morbidity is 74/100,000 in the U.S. 62.5/100,000 in Europe, and 85/100,000 in Sweden. This paper investigates the possible risk factors influencing breast cancer. Several authors have found a genetic predisposition to breast cancer; daughters of mothers with breast cancer have 27-30% more probability of getting the disease; a high percentage of cases with family antecedents, 35.8%, is diagnosed before menopause. According to some authors early menopause therapeutically induced has a protective effect against breast cancer; according to other authors estriol can modify the carcinogenic effects of other estrogens; thus, women with low levels of estriol have a higher risk of breast cancer. Level of plasmatic hormonal concentrations may also play a role; a diet poor in iodine may be an added risk. Other risk factors seem to be age under 13 at menarche, late menopause, low fertility especially after 35, absence of breastfeeding, height over 165 cm, weight above 70 kg, and a diet rich in animal fats. There is no proven association between oral contraceptive use and increased incidence of breast cancer.^ieng

[Pancreatic duct antibodies and subclinical insufficiency of the exocrine pancreas in Sjögren's syndrome]. / Gli anticorpi anti-duttuli e l'insufficienza subclinica del pancreas esocrino nella sindrome di Sjögren.

In previous studies we reported evidence of subclinical exocrine pancreatic insufficiency in primary or secondary Sjögren's syndrome (SSI, SSII) and rheumatoid arthritis (RA). In present study we evaluated the occurrence of pancreatic duct antibodies (PDA), and their relationship to exocrine pancreatic function in 36 women. Of these patients, 12 were classified as SSI, 12 as SSII, and 12 as RA. Exocrine pancreatic function was evaluated using the Secretin-Caerulein test (S.Cae test). The indirect immunofluorescent technique was used to evaluate patient sera for PDA. S.Cae test results, compared to controls, showed a statistically significant decrease in duodenal juice volumes, bicarbonates and enzymes in 58.3% of SSI, in 58.3% of SSI and in 30% of RA, according to our previous trials. PDA were found in two patients, the former with SSI and the latter with SSII, both asymptomatic with regard to pancreatic diseases but with impaired exocrine pancreatic function as evaluated by S.Cae test. We discuss the possible causes of these results.