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Antisynthetase syndrome belongs to the idiopathic myositis group which includes pulmonary interstitial disease, arthritis, Raynaud's phenomenon, and mechanic's hands , associated with the anti-Jo1 antibody. We report three cases of antisynthetase syndrome, and review the clinical characteristics, and prognosis factors dominated by interstitial pneumonia.

Kikuchi-Fujimoto disease, or necrotizing histiocyte lymphadenitis, is a rare anatomo-clinical entity of unknown cause. The patients present painful cervical lymph nodes, fever, and a flu-like syndrome, but the spontaneous course is usually favorable in six months. We observed two cases within six months in our unit. Both patients improved rapidly after diagnostic adenectomy.

Background: Risk factors for the thrombo-embolic disease are not yet completely validated in medical setting. Indeed, there is a lack of scientific data concerning their prevalence and their thrombogenic weight., Methods: We carried out a prospective case-control study in an internal medicine unit where general acute pathologies are recruited. We have included two patients without thrombosis for each patient admitted for a thrombo-embolic event. In all patients, at admission, clinical and biological risk factors for thromboembolism were systematically analysed in a physiopathological and chronological way., Results: 450 patients were included during the 27 months period of the study. Among these patients, 150 were hospitalised for a deep venous thrombosis of the leg or/and a pulmonary embolism. Height risk factors have demonstrated their high thrombogenic potential: paralysis of the legs (OR = 13; 95% CI: 1.4 to 5.1), post-thrombotic syndromes (OR = 13; CI: 1.7 to 4.3), extrinsic compressions (OR = 8; CI: 1.3 to 5.4), pregnancy (OR = 6; CI: 1.2 to 6), malignancy occurred one year before (OR = 4; CI: 1.1 to 6.7), personal history of deep vein thrombosis (OR = 3; CI: 1.6 to 4.5), varicosity (OR = 2.5; CI: 1.2 to 6.1) and venous insufficiency (OR = 1.7; CI: 1 to 7.4). Four parameters, generally considered as risk factors, didn't prove here their pathogenic power: the age over 60, obesity (BMI > 27), bedrest and the presence of an on-going malignancy., Conclusions: Only risk factors with high thrombogenic power (as paralysis or extrinsic compression for example) could be identified by this kind of study performed in polypathological patients. In this particular population, usually recognised risk factors as age, obesity and bedrest are not determinant parameters for the thomboembolic risk evaluation.

Purpose: Pathogeny of hypoglycemia associated with non-islet-cell tumors is unclear. We discuss the mechanisms of this syndrome., Current Knowledge and Key Points: We report three cases of spontaneous hypoglycemia revealing pleural fibroma. Endocrine tests before surgery showed low serum growth hormone and insulin-like growth factor I (IGF-I), reduced serum IGF-II levels in two patient. Insulin-like growth factor binding protein-3 (IGFBP-3) was low and electrophoretic profile of IGF-II was the 'big IGF-II' type. We discuss the mechanisms of hypoglycemia associated with non-islet-cell tumor. Impaired formation of the ternary complexes and its consequences seem the main pathogenic factor., Future Prospects and Projects: Analysis of IGF and IGFBP and in situ measurements of IGF mRNA could help in understanding this syndrome and allow therapeutic considerations in the management of hypoglycemia by corticosteroids and growth hormone.

Purpose: The thromboembolic risk is difficult to evaluate in medical inpatients because of the multiplicity of risk factors. The lack of scientific data leads to a certain empiricism in selection of patients who need a preventive treatment., Methods: All patients hospitalised in an internal medicine department were included during 34 months. The pathogenic mechanism of risk factors (venous stasis or hypercoagulability) was analysed as well as their progress (transient or permanent risk factors) and their pathogenic weight (major risk factor or not). Three levels of embolic risk (high, intermediate, low) were identified. A preventive treatment was initiated only for patients at high or intermediate risk. A clinical screening for thromboembolic events was performed daily. Therefore, we have excluded patients who needed a hypocoagulant treatment., Results: Nine hundred and forty-seven patients were included. Age (over 60) was the most frequent permanent risk factor (70%). Bed rest was the most frequent transient risk factor responsible for venous stasis. Fifty percent of patients had a transient and permanent risk factor and needed a preventive treatment according to our selection criteria. The incidence of thromboembolic events was 0.4% in the high-risk treated group and 0.2% in the low-risk non-treated group., Conclusions: Venous stasis factors are the more frequent risk factor in internal medicine, perhaps because of the increasing number of geriatric patients. The incidence of thromboembolic disease is low in high-risk inpatients when a preventive treatment is performed.

Background: In terms of preventive management of venous thromboembolism in medical inpatients, very large differences may be observed. Rationalization of behaviour requires the evaluation of simple and logical parameters, which takes into account both patient safety and economic considerations., Aim: The aim of this study was to evaluate a preventive scheme including the rationalization of the indications and the use of low molecular weight heparin., Experimental Design: Epidemiologic investigation., Setting and Patients: Patients hospitalized in five medical departments in the Hospital Center of Nantes, France., Intervention: The risk of venous thromboembolism was rated as high, intermediate and low. Patients with high or intermediate risk were eligible for prevention therapy (table I)., Measures: The main criterion was the occurrence during hospital stay of deep or superficial venous thrombosis of the lower limbs, pulmonary embolism, or unexplained sudden death. The screening was based on clinical features double-checked by venous doppler ultrasonography of the lower limbs and/or ventilation-perfusion lung scanning., Results: 24,497 patients were eligible (table II), 15% were considered at risk and treated with Nadroparin, 6% had the same risk profile but were not treated and 14. 7% had low risk and no prevention. No bleeding event was reported. The incidence of venous thromboembolism was 0.75%, 1.7% and 0.14% respectively (p <0.01) (table III). This efficacy does not appear to depend on body weight or the existence of multiple risk factors observed (table IV and V)., Conclusions: This analysis of risk factors separates two populations with rates of incidence dramatically and significantly different. The prevention of venous thromboembolism by fixed dose of low molecular weight heparin remains justified since it reduces the risk of venous thromboembolism by a factor of 2.5.

Introduction: The authors report a case of neurologic manifestations revealing Richter's syndrome in chronic lymphocytic leukemia., Exegesis: Cranial nerve palsies were the initial manifestation of the disease. Computed tomography and magnetic resonance imaging were normal. Cerebrospinal fluid analysis with alkaline phosphatase-antialkaline phosphatase revealed the existence of a large-cell lymphoma., Conclusions: Clinical aspects of this unusual localization of Richter's syndrome are reviewed.

Introduction: Willebrand's syndrome is rarely acquired. We report four cases associated with lymphoproliferative syndromes., Case Reports: We observed four patients with lymphoid hemopathies who developed acquired Willebrand's syndrome. Two patients had Waldenström's disease (kappa), one had a monoclonal gammapathy of undetermined signification (kappa immunoglobulin M) and the fourth had chronic lymphoid leukemia with mast cell infiltration of the skin. Anti-vWFRCo antibodies were evidenced in only 1 case. Chemotherapy, used in 3 cases, improved hemostasis in one patient. Intravenous immunoglobulins (1 patient) and desmopressin (2 patients) were ineffective. The pathogenic mechanisms and possible therapeutic approaches to acquired Willebrand's syndrome are discussed., Discussion: Acquired Willebrand's syndrome rarely occurs in association with lymphoproliferative disorders appears to be uncommon but the frequency is probably underestimated because appropriate tests are not always performed. The diagnostic search is important however since the hemostasis disorders due to acquired Willebrand's syndrome could be corrected if appropriate etiological treatment is given.

We report the case of a woman splenectomized to treat her hairy cell leukemia (at the moment in remission) 11 years before the detection of neutropenia. The neutropenia began just after the treatment of pulmonary embolism by acenocoumarol. The neutropenia disappeared quickly after substitution of acenocoumarol by fluindione. We discuss the attribution of the neutropenia to acenocoumarol and the part played by hairy cell leukemia.

From a prospective study concerning 576 patients with Raynaud's phenomenon, the authors studied the results of Allen's clinical test and their etiologic significance. These results were compared to those obtained by the instrumental technique using the Doppler probe to locate and compress radial and ulnar arteries and digital plethysmography to measure the effects of this compression. This instrumental technique makes it possible to investigate hand vascularization when Allen's clinical test is non interpretable or impossible which corresponds to 30% patients. A pathological Allen's test, whether it be clinical or instrumental, rather favors a Raynaud's syndrome (p < 10(-4)). The absence of ulnar vascularization was the abnormality most often noted and was rather found in severe Raynaud's phenomena, the sclerodermatous particularly (p < 10(-4)).

The authors report 7 cases of bone marrow necrosis during hematological malignant diseases:3 leukemia, 4 lymphoid malignancies. The main clinical features were bone pain and fever. The bone marrow aspiration and/or biopsy allow diagnosis. The prognosis was in all cases very poor.

The authors report a case of amyloidosis AL associated with light lambda chain myeloma, mimicking giant cell temporal arteritis. The normality of inflammatory proteins, the primary resistance to corcicosteroids and the results of temporal artery biopsy (amyloid deposits without giant cell granuloma) excluded a diagnosis of giant cell arteritis associated with amyloidosis. The most probable mechanism was infiltration of branches of the external carotid artery. In patients with temporal arteritis, amyloidosis must be suspected and sought, particularly in case of monoclonal dysglobulinaemia or manifestations that are not typical of giant cell temporal arteritis. The prognosis of vascular amyloidosis seems to be more favourable than that of the classical visceral types. The primary amyloidosis of these patients must be treated with melphalan combined with prednisone and colchicine.

We evaluated the efficacy and toxicity of piperacillin-ofloxacin as an empiric treatment of fever in patients with neutropenia. 24 febrile episodes occurring in 21 patients (mean neutropenia: 204/mm3) were treated. The neutropenia was due to an hematologic malignancy in 6 cases and to chemotherapy in 15 cases. Fever was related to septicemia in 4 cases, urinary tract infection in 1 case, other infectious sites without microbiological documentation in 11 cases, and was of unknown origin in 8 cases. Empirical therapy was started within 24 hours of the occurrence of fever greater than 38.5 degrees C with the combination of intravenous piperacillin (12 g/day in 3 divided doses) and oral ofloxacin (400 mg/day in 2 doses). The overall response rate was 86% (19/22) of evaluable cases, with an immediate success rate (apyrexia within 48 hours) of 46%. Of the 3 failures, one was bacteriologically documented and was due to a multiply resistant strain of Staphylococcus haemolyticus (to which both piperacillin and ofloxacin were resistant). The therapy was clinically well tolerated in all except 3 patients, in whom intolerance to intravenous piperacillin was observed, leading to discontinuation of the drug in 2 cases. More extensive and comparative trials should better determine the place of this piperacillin-ofloxacin combination as first-line treatment of febrile episodes in patients with neutropenia.

Associated ultrasonic Doppler study (D) and plethysmography (P) were evaluated as compared to phlebography in diagnosis of venous thrombosis of the lower limbs (TVP). Probes (5 and 7.5 MHz respectively) were used in D for examination of proximal and calf veins. Plethysmography using a mercury ring gauge (Perivein, ETNA) was considered pathologic if filling volume was less than 1.5 vol% and/or emptying volume less than 40 ml/min/100 ml. One hundred and four patients hospitalized in an internal medicine department for suspected deep venous thrombosis were studied, including 97 for whom phlebography interpretation was unmistakable: 11 calf vein, 28 collector trunk and 42 total limb involvement TVP. In these localizations, D sensitivity was respectively 55, 72 and 100% (mean 85.5%) and that of P 63.5, 68 and 88% (mean 77.7%); association of D or P improved sensitivity: 82, 82 and 100% (mean 91.5%). Specificity was 86.6% for D, a poor 62.5% for P and 93.5% for associated D and P. The results are comparable to those in the literature but disappointing for diagnosis of isolated calf vein TVP. False-negatives in both D and P were due to strong collateral circulation or limited thrombus extension.

A 63-year-old man, with a Waldenström's disease discovered by cryoglobulinemia (ischemic lesions of fingers) was quickly aggravating (hyperviscosity syndrome) under treatment by chlorambucil in a dosage of 8 mg daily. We obtained a dramatic remission with combination of cyclophosphamide, vincristine, adriamycin and prednisone surrounding a total body irradiation. A new trial of polychimiotherapy (with lower dosage) for severe Waldenström's disease is actually on study.

To evaluate two methods of obtaining lower respiratory tract secretions, fiberoptic bronchoscopy (FB) and transtracheal aspiration (TA) were performed in ten patients. FB used an open-end brush-in catheter. TA was performed as described by Pecora. TA gave significantly better sensitivity and specificity (3/5 and O false positive results respectively) than FB (2/5 and 5 false positive results). TA gives specimens which reflect the bacterial flora of the lung more reliably than those taken during FB.

A method is described for measuring digital venous compliance to cold by venous occlusion plethysmography (Perivein-Janssen apparatus). Determinations are made firstly to heat during immersion of the hand at 45 degrees C and then to cold during its immersion at 11 degrees C. In each case compliance is measured directly from a curve derived from successive venous occlusions of 1 mm Hg up to 120 mm Hg and by a standardized rate of recording. For pressures between 30 and 80 mm Hg a tracing is obtained that is assimilable to a linear slope from which compliance is calculated from the ratio delta P/delta V expressed as percentage variation of volume per cm Hg. The method was applied to 36 control subjects (19 men and 17 women) and 80 cases of Raynaud's phenomenon. Comparison of findings showed a significantly more marked fall in compliance to cold between: the female and male control subjects (p less than 10(-2)) the group of men with Raynaud's syndrome and male control subjects (p less than 10(-3)), the group with Raynaud's syndrome and Raynaud's disease (p less than 0.05). The findings suggest a possible pathogenic role of venospasm from cold in the determination of the syncopal phase of Raynaud's phenomenon and with respect to its predominance in members of the female sex.

A 48-year-old-woman had simultaneously a peri-arteritis nodosa (PAN) and a Waldenström disease. PAN's symptoms were quickly regressive, neuropathy excepted (with slowness) under treatment by cyclophosphamide and prednisone. Hemopathy is now stabilized. Such a pathological association is exceptional (2 other cases in literature). Is it accidental? No cryoglobulinaemia or circulating immune-complexes or HBS antigen were found. Immunofluorescence study of arteriolar lesions in muscle showed a polyclonal fixation in inflammatory granuloma and monoclonal (IgM lambda) on endothelial side. Passive deposit is not excluded, because research of specific activity of IgM lambda (against fibrin or endothelial cells) was negative. Waldenström's disease has probably facilitated apparition of necrotizing angeitis but mechanism is not proved.

Increased serum IgA in patients with direct toxic alcoholic peripheral neuropathy was studied prospectively in 33 patients with an obvious clinical polyneuropathy confirmed by electrophysiological investigations. The average concentration of IgA in this group (450 mg/100 ml) was significantly higher than in a group of 24 matched alcoholic control patients without peripheral neuropathy (306 mg/100 ml), p less than 0.0001. The authors discuss possible direct and indirect modifications of the humoral immune response due to the peripheral neuropathy.

The authors report a case of relapsing polychondritis, in a 41 year-old man, with renal complication. Immunofluorescence study of this segmental necrotizing glomerulonephritis showed an important fibrin fixation without immunoglobulins. Selective renal arteriography, preliminary to the biopsy, showed lobular arterial aneurysms. Because of other exceptional cases of literature, relation between periarteritis nodosa and relapsing polychondritis is discussed.