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Biomedical subjects

P Pugin

Publications and source records attributed to P Pugin.

At least 19 recordsLinked to original sources
PubMed1995

[Successful treatment of a case of cutaneous Langerhans cell granulomatosis with 2-chlorodeoxyadenosine and thalidomide].

We describe the case of a 65-year-old female patient with cutaneous Langerhans' cell granulomatosis without any signs of disease in other organs. She also had systemic lupus erythematosus that had been diagnosed several years before. The coexistence of these two diseases has not been described before as far as we know. The purine analogue 2-chlorodeoxyadenosine (Cladribin), which has been used successfully in the treatment of hairy cell leukaemia, induced complete remission in our patient after 1 week of treatment. After 2 months, however, the patient had a relapse; this was successfully treated with thalidomide. A new understanding of Langerhans' cells granulomatosis as a reactive but not cancerous disease has emerged as a result of recent investigations showing that tumour necrosis factor-alpha (TNF-alpha) plays an important part in the induction of Langerhans' cells from their immature precursors. Because thalidomide has been shown to inhibit TNF-alpha production, down-modulation of this cytokine seems to be a useful treatment strategy in Langerhans' cell granulomatosis. Some asspects of the diagnosis and therapy of this disease are briefly reviewed.

Aged↗
PubMed1993

[Primary Hodgkin disease of the lung].

We describe a patient with a rare primary pulmonary manifestation of Hodgkin's disease who presented with multiple bilateral nodular lesions on the chest x-ray without significant hilar involvement.

Adolescent↗
PubMed1991

[HTLV-I/II in Switzerland: apropos of 4 case reports].

HTLV-I/II (human T-cell lymphotropic virus type I and II) infection is endemic in South Japan, Subsaharan Africa, the Caribbean and in regions of South America and of the United States. The infection almost always remains asymptomatic but is also associated with two distinct diseases: tropical spastic paraparesis (TSP), and adult T-cell leukemia (ATL). Although very rare in Europe, HTLV-I/II infection may occur in patients originating from endemic areas. We report the cases of four patients seropositive for HTLV-I/II, all of them living in Switzerland. Three of them originate from Zaire; the fourth is a Swiss female married to a Taiwanese. One patient has TSP; one has pyramidal signs along with systemic vasculitis-associated neuropathy; the third is HIV-position with stage III B infection, without symptoms of TSP or ATL; the last is entirely asymptomatic.

Adult↗
PubMed1991

Interaction of heterozygous beta (0)-thalassemia and triplicated alpha globin loci in a Swiss-Spanish family.

We report a Swiss-Spanish family three members of which have the clinical picture of thalassemia intermedia. Restriction endonuclease mapping of the alpha-globin cluster and digestion with Mae I of the in vitro amplified 5' segment of the beta-globin gene shows a combination of triplicated alpha globin locus, anti-3.7 kb type, with heterozygous codon 39 C----T beta (0) thalassemic mutation. These, as well as 16 similar cases reported in the literature, permit the following conclusion: a single extra alpha-globin gene gives rise to a clinically significant degree of dyserythropoietic anemia only when it interacts with a severe beta(+) or beta(0) thalassemic mutation.

Adult↗
PubMed1991

[Hypothyroidism and acanthocytes: diagnostic significance of blood smear].

We report on 3 cases of hypothyroidism in which the diagnoses were suspected after having observed acanthocytes on the blood film. The diagnostic value of this erythrocyte change has been neglected, although the results of studies have shown a test specificity of 87-99%, other diseases related to acanthocytes being very rare. After an analysis of the studies and case reports dealing with acanthocytes we conclude that hypothyroidism must be excluded in all cases where acanthocytes are observed on the blood film.

Acanthocytes↗
PubMed1990

[Acute myeloid leukemia with poor prognosis: treatment with oral 4-demethoxydaunorubicin (idarubicin) and low-dose cytarabine via subcutaneous route].

26 patients with poor risk acute myelogenous leukemia (elderly, in relapse or resistant) were treated with a combination of oral idarubicin (30 mg/m2/d for 3 days) and low dose subcutaneous cytarabine (10 mg/m2 twice a day for 10 days). Of 26 patients, 14 achieved complete remission, 2 partial remission, and 5 died in aplasia (3 without evidence of response, 2 inevaluable); 5 further patients were non-responders. All responses but two occurred among patients treated for AML at presentation or in relapse. Side effects consisted mainly of severe hematologic and moderate gastrointestinal toxicities. The main interest of this regimen is adaptability to outpatient conditions and rapid cytoreduction in patients with hyperleukocytic presentation.

Administration, Oral↗
PubMed1990

Histiocytic necrotizing lymphadenitis or Kikuchi's disease. Anatomo-clinical study of 4 cases.

Histiocytic necrotizing lymphadentis (HNL) is an uncommon clinical and histologic entity, essentially diagnosed in Japan since 1972. The clinical picture is usually characterized by cervical lymphadenopathy and fever, females being more often affected. Leukopenia and elevated erythrocyte sedimentation rate are frequent. The etiology is still unknown, but a viral origin is most likely. The clinical course is always favorable without treatment, except in one case. The histological picture, with necrotic foci surrounded by histiocytes, immunoblasts, small T lymphocytes and plasmacytoid monocytes (so-called plasmacytoid T cells), is characteristic. Nevertheless, HNL may be mistaken for malignant lymphoma both clinically and histologically. We report 4 cases of HNL. One of these presented severe leukothrombopenia; the serum of this patient significantly suppressed the maturation of granulocytic precursor cells in the bone marrow.

Adolescent↗
PubMed1989

[Light chain myeloma or the pitfalls of myeloma diagnosis].

The diagnosis of multiple myeloma is often suggested by disturbances found in routine laboratory tests such as sedimentation rate, electrophoresis of serum proteins and search for proteinuria. In light chain myeloma these tests are nonspecific and therefore misleading. We present 8 cases of light chain myeloma and discuss the diagnosis of multiple myeloma with its associated pitfalls.

Aged↗
PubMed1988

X-linked lymphoproliferative syndrome. Identification of a large family in Switzerland.

Observation of a patient with acquired hypogammaglobulinemia associated with a mononucleosis syndrome led to the identification of one of the largest families affected by the X-linked lymphoproliferative (XLP) syndrome in the world. It is the first such family identified in Switzerland and the largest in Europe. At least nine male subjects over two generations presented phenotypic expressions consistent with the XLP syndrome. Study of the pedigree extending over seven generations suggests that the mutation occurred in the proband's great-grandmother. In the next generation, a second mutation of the X chromosome in one branch of the family resulted in expression of hemophilia A in the children. This remarkably large family, comprising six living obligate female carriers, displays a wide spectrum of the XLP syndrome and offers valuable information for future genetic linkage studies and for genetic counseling.

Adult↗
PubMed1983

Bacteriological study of amniotic fluid during labor.

Amniotic fluid from 207 women in labor was analysed at the time of artificial rupture of membranes or by amniocentesis. The following organisms were identified in concentrations of more than 1 000/ml: Staphylococcus aureus (1), Propionibacterium (1), E. coli (1), group B Streptococci (3), Lactobacilli (16). The 6 patient-carriers of pathogens became infected as did 4 of their babies. Leukocyte counts and LDH levels performed on amniotic fluid did not correlate with the appearance of symptoms of infection. Quantitative bacteriology of amniotic fluid seems to be of value in identifying patients at high risk of developing endometritis and/or neonatal sepsis.

Amniotic Fluid↗
PubMed1979

[Selective aplasia of neutrophils: auto-immune origin].

Autoimmunity is a recognized factor in pure red cell aplasia and in some cases of aplastic anemia, but not in agranulocytosis. The case reported here demonstrated that pure neutrophilic aplasia of autoimmune origin may exist in man. A 75-year-old male is described who presented with complete agranulocytosis and absence of neutrophilic precursors in the bone marrow without thrombocytopenia or anemia. After 6 weeks, remission was induced by immunosuppressive treatment and 22 months later the patient is doing well without therapy. The absence of known cases of agranulocytosis, a good response to immunosuppressive therapy, and the demonstrated fact that the patient's peripheral blood mononuclear cells inhibit the growth of granulocytic colonies of normal human marrow in vitro allow the conclusion that this patient had pure neutropenic aplasia, probably due to autoimmune phenomena.

Aged↗
PubMed1979

[Sideroblastic anemia: clinical and hematological study on 57 patients].

Sideroblastic anemias (SA) are a heterogeneous group of hematologic disorders marked by a defect of heme synthesis, disturbance of iron metabolism and the presence of ringed sideroblasts. 57 cases of SA, including 23 primary and 34 secondary forms, are discussed. In the group of secondary SA 13 patients were alcoholics, 3 had received chloramphenicol, 2 had lead poisoning, 2 had received busulfan and 2 had immune hemolytic anemia. In some cases serum ferritin was found to be excessively high. The caryotype of one patient presented deletion of the long arm on chromosome 20. None of the patients with primary SA responded to vitamin treatment, and 3 developed a myeloproliferative syndrome.

Alcoholism↗
PubMed1979

[Kala-azar; clinical and physiopathological study a propos of a new case studied in Switzerland].

Kala-azar, a parasitic disease caused by Leishmania donovani, is usually found in tropical areas but may occasionally occur in other regions such as the Mediterranean. A case of kala-azar in a women who had been on a holiday in Greece is reported. The parasite was demonstrated in lymph node biopsies and bone marrow smears and treatment with stibogluconate was begun. High levels of circulating immune complexes were demonstrable before and during the demonstration of parasites; the immune complexes were partially characterized. The diminution of complexes in serum paralleled the regression of clinical symptoms, suggesting a relationship between the clinical course and levels of circulating immune complexes. The disease may be a serum sickness-like syndrome induced by the parasite.

Adult↗