[Efficacy of autologous peripheral blood stem cell transplantation (auto-PBSCT) on the neuropathic manifestations in POEMS syndrome]. / Efficacité à moyen terme de l'autogreffe de cellules souches hématopoïétiques périphériques sur la polyneuropathie du syndrome POEMS: expérience chez 5 cas.

INTRODUCTION: POEMS syndrome (polyneuropathy, organomegaly, endocrynopathy, M-protein, and skin changes) is a rare multisystem disease associated with plasma cell dyscrasia. The efficacy of autologous peripheral blood stem cell transplantation (auto-PBSCT) reported in case series has been mainly based on hematologic criteria and clinical recovery of peripheral neuropathy dysfunctions but has not been specifically evaluated. This retrospective study aimed to analyze the efficacy of auto-PBSCT on disability and electrophysiological patterns in patients with POEMS syndrome. METHODS: Five patients presenting with POEMS syndrome received auto-PBSCT. Disability was evaluated before treatment and at 6 and 12 months using the Overall Neuropathy Limitation Scale (ONLS) and MRC sumscore of 28 muscles. Nerve conduction studies were performed before and one year after treatment, on median, ulnar, fibular and tibial nerves. RESULTS: Mean age was 60.6 years (49-70). Disease duration between first symptoms and auto-PBSCT was 15.4 months (2-33). Before auto-PBSCT, mean ONLS score was 4.2 (1-10) and mean MRC sumscore 115.8/140 (74-140). At M6, mean ONLS score decreased and mean MRC sumscore increased; both were improved in all patients at M12: mean ONLS score 3 (range 0-8) at M6 and 2.2 (range 0-7) at M12; mean MRC sumscore 118/140 (77-140) at M6 and 122.4/140 (80-140) at M12. Significant recovery in electrophysiological patterns was observed in all patients on ulnar and median nerves: before-after treatment differences were observed for motor conduction velocities (34.41 vs. 45.47 m/s; P<0.001), distal CMAP amplitudes (5.04 vs. 5.96 mV; P=0.004), and sensory conduction velocities (43.20 vs. 49.20 m/s; P=0.001). Distal CMAP amplitude remained low in fibular and tibial nerves (0.41 vs. 0.17 mV). CONCLUSIONS: Clinical and electrophysiological improvement is obvious in POEMS syndrome peripheral neuropathy within one year after treatment with auto-PBSCT, undoubtedly resulting from extensive remyelinisation and axonal regeneration. Further studies are required to examine long-term outcome in patients with POEMS syndrome given auto-PBSCT.

Heterogeneity of t(4;14) in multiple myeloma. Long-term follow-up of 100 cases treated with tandem transplantation in IFM99 trials.

One hundred de novo multiple myeloma patients with t(4;14) treated with double intensive therapy according to IFM99 protocols were retrospectively analyzed. The median overall survival (OS) and event-free survival (EFS) were 41.4 and 21 months, respectively, as compared to 65 and 37 for patients included in the IFM99 trials without t(4;14) (P<10(-7)). We identified a subgroup of patients presenting at diagnosis with both low beta(2)-microglobulin <4 mg/l and high hemoglobin (Hb) >/=10 g/l (46% of the cases) with a median OS of 54.6 months and a median EFS of 26 months, respectively, which benefits from high-dose therapy (HDT); conversely patients with one or both adverse prognostic factor (high beta(2)-microglobulin and/or low Hb) had a poor outcome. The achievement of either complete response or very good partial response after HDT was also a powerful independent prognostic factor for both OS and EFS.

[French recommendations on control measures to reduce the infectious risk in immunocompromised patients]. / Quelles mesures pour maîtriser le risque infectieux chez les patients immunodéprimés ? Recommandations formalisées d'experts.

The increase use of immunosuppressive treatments in patients with solid cancer and/or inflammatory diseases requires revisiting our practices for the prevention of infectious risk in the care setting. A review of the literature by a multidisciplinary working group at the beginning of 2014 wished to answer the following 4 questions to improve healthcare immunocompromised patients: (I) How can we define immunocompromised patients with high, intermediate and low infectious risk, (II) which air treatment should be recommended for this specific population? (III) What additional precautions should be recommended for immunocompromised patients at risk for infection? (IV) Which global environmental control should be recommended? Based on data from the literature and using the GRADE method, we propose 15 recommendations that could help to reduce the risk of infection in these exposed populations.

Human trichinellosis due to Trichinella britovi in southern France after consumption of frozen wild boar meat.

Six patients were infected with Trichinella britovi in southern France following consumption of frozen wild boar meat, which had been frozen at -35 degrees C for 7 days. Microscopic examination of a sample of frozen wild boar muscle revealed the presence of rare encapsulated Trichinella larvae, identified as T. britovi. People eating wild boar must follow individual prophylactic rules such as efficient cooking of meat (at least 65 degrees C at the core for 1 minute) as recommended by the International Commission on Trichinellosis, or freezing exceeding four weeks at -20 degrees C.

Effect of indinavir and higher CD4+ T-cell count on viral load response after 6 months of highly active antiretroviral therapy.

This retrospective, unmasked chart review was undertaken to determine which HIV-infected patients receiving protease inhibitors (PIs) for the first time were most likely to experience a decrease in plasma viral load (PVL) and which factors were associated with a PVL < 500 copies/mL below the detectable limits after 6 months. A total of 308 patients aged > 15 years with a PVL > 500 copies/mL received therapy that included a PI in addition to other antiretroviral therapies (128 patients, saquinavir hard-gel capsule 600 mg TID; 107 patients, indinavir 800 mg TID; and 73 patients, ritonavir 600 mg BID). The choice of drug was at individual clinicians' discretion. Patients were followed for a median of 10 (range, 6 to 21) months. Of the 128 patients who received saquinavir, 45% were switched to another PI (33%, indinavir; 12%, ritonavir). Seventy percent of the 73 patients initially given ritonavir were switched (45%, indinavir; 25%, saquinavir), as were 23% of the 107 patients initially given indinavir (15%, saquinavir; 8%, ritonavir). A total of 34.1% (n = 105) of patients achieved a PVL < 500 copies/mL; in 51.6%, PVL decreased > 0.5 log copies/mL. In this subgroup, both treatment-naive patients and those who were receiving a new combination of antiretroviral therapy when they started PI treatment had a more pronounced decline in PVL (P < 0.001). After adjustment by logistic regression analysis for age, sex, mode of transmission, and duration of highly active antiretroviral therapy (HAART), CD4+ cell count and initial type of PI received were independently associated with PVL < 500 copies/mL. In the present study, the treatment success rate was low (34.1%) compared with rates observed in randomized, controlled trials. A higher CD4+ cell count and use of indinavir at the initiation of HAART are associated with a better viral load response.

Knee arthritis revealing acute leukemia in a patient with rheumatoid arthritis.

We report one case of leukemic synovitis in a patient with a 40-year history of rheumatoid arthritis. The synovial fluid sample and synovial biopsy specimen showed myeloblastic cells. So, leukemic synovitis can be suspected in a patient with inflammatory rheumatism and adequate diagnostic procedures should be carried out.

Paraneoplastic Raynaud's phenomenon.

A testicular tumour could be diagnosed by the occurrence of a Raynaud's phenomenon complicated by severe digital arteritis. The arteritis rapidly regressed under prostacyclin therapy. Such vascular manifestations are frequent in testicular carcinoma, but they usually develop after chemotherapy. To our knowledge, this is the first case where they preceded the diagnosis and specific treatment of a tumour of the testis.

Erythema nodosum and Hodgkin's disease.

Erythema nodosum is an unusual manifestation of Hodgkin's lymphoma. One patient with long remission of Hodgkin's disease manifested persistent erythema nodosum beginning one and three months before relapse was clinically evident. So, the diagnosis of Hodgkin's disease should be considered in patients with unexplained recurrent erythema nodosum.

[Complete regression of cardiac non-Hodgkin's lymphoma after 23 months with chemotherapy]. / Disparition totale à 23 mois d'un volumineux lymphome non hodgkinien cardiaque par chimiothérapie.

The authors report the case of a non-Hodgkin malignant lymphoma (NHL) of the heart presenting with syncope. The diagnosis of a cardiac tumours was made by echocardiography. Myocardial biopsy enabled diagnosis of a highly malignant NHL in a patient with a history of low grade NHL. Chemotherapy with CNOP (Cyclophosphamide, Novantrone, Oncovin, Prednisone) induced total regression of the tumour. The patient is in total remission 23 months later. The authors emphasise the value of echocardiography in the diagnosis and follow-up of this pathology. The case is also noteworthy because of the unusual transformation of a low grade to a high grade cardiac NHL.

[Cutaneous hemangioma associated with eosinophilia]. / Angiome cutané associé à une éosinophilie.

A patient with a cutaneous angioma developed an intravascular coagulation syndrome with a eosinophilic leukocytosis and multiple arterial and venous thromboses affecting the vital prognosis. The possible role of the eosinophilia in the mechanism of complications is discussed. Local modifications in the angioma are suggestive signs preceding the onset of general thrombotic complications, their severity contrasting with the simplicity of the secondary surgical excision of the angioma.

[Drug-induced benign intracranial hypertension. Apropos of a case with amphotericin B. Review of the literature]. / Hypertension intracrânienne bénigne médicamenteuse. A propos d'un cas rapporté à l'amphotéricine B. Revue de la littérature.

Benign intracranial hypertension (BICH) is a rare adverse event. We report the case of a 31-year-old female drug addict who had been seropositive for HIV since 1987. She had stage IV C1 AIDS, and was receiving intravenous amphotericin B for generalized cryptococcosis with no neuromeningeal involvement. She developed BICH that regressed when the antifungal drug was withdrawn and treatment for cerebral edema was started. BICH is a clinical entity involving intracranial hypertension with no focal neurological signs or detectable intracranial lesion. The manifestations include headache, transitory or permanent visual disturbances (diplopia, loss of visual acuity) and the perception of intracranial noise. The cerebrospinal fluid is under increased pressure but the composition is normal. The eye fundus examination shows papillary edema, and the neuroradiological workup is normal. BICH can only be diagnosed once an expansive intracranial process, neuromeningeal infection, and non-communicative hydrocephalus have been ruled out. In the majority of cases, no etiology is found. Such cases of idiopathic BICH usually occur in overweight young women, although drugs can be implicated. Amphotericin B has not previously been held responsible for BICH. On the basis of this observation, we present a review of the literature.

[Thrombopenia due to pefloxacin (Peflacine): dose-dependent toxicity?]. / Thrombopénies dues à la péfloxacine (Péflacine): une toxicité dose-dépendante?

Pefloxacin (Peflacine) can give rise to thrombocytopenia, although the responsibility of the drug can be difficult to demonstrate in infectious patients and those receiving other drugs simultaneously. We have collated 18 cases in which the responsibility of pefloxacin was suspected. In 13 cases, the patients were also taking other drugs which may have been contributory (heparin, Bactrim, Augmentin, ranitidine,...). The remaining five cases were of particular interest as pefloxacin was the only drug administered. The mean age of the patients was 75 years, and the mean bodyweight 56 kg (range, 47-65 kg). The pefloxacin dosage was 800 to 1,600 mg/day i.v. or p.o., i.e. 13-18 mg/kg/day. Thrombocytopenia occurred from five to 19 days after beginning treatment and resolved between 7 and 12 days after drug withdrawal. A number of factors argue in favor of dose dependency: 1 patient had high plasma concentrations (peak and residual); thrombocytopenia occurred in one patient when the previous, well-tolerated dosage of 800 mg/day (for 15 days) was increased to 1,600 mg/day; thrombocytopenia resolved in one patient when the dosage was reduced to 400 mg/day (1 tablet) and continued for a further 10 days. This toxic reaction may be avoided by reducing the drug dosage to 400 mg/day in elderly patients with a low bodyweight. Differential blood counts appear to be warranted for patients at risk.

[Visceral leishmaniasis caused by a dermotropic strain of Leishmania infantum in an AIDS patient]. / Leishmaniose viscérale causée par une souche dermotrope de Leishmania infantum chez un sidéen.

A case of fatal visceral leishmaniasis due to a dermotropic strain of Leishmania infantum (Zymodeme MON-24) is related in an AIDS patient in spite of a specific treatment.

[Jaundice caused by rifampicin: 3 cases]. / Ictère dû à la rifampicine: trois observations.

Three cases are reported of cholestatic hepatitis occurring during treatment with rifampicin for staphylococcal septicemia (2) and tuberculosis (1). There was no previous history of hepatic affection. The administration of rifampicin caused cholestasis alone. No immunoallergic phenomenon has been shown.

[Spinal cord compression of sarcoidosis origin: a case]. / Compression médullaire d'origine sarcoïdosique: une observation.

This observation reports the case of a rapid paraparesia occurring in a young man. Imaging disclosed a spina tumor. Diagnosis of sarcoidosis leads us to suspect a specific spinal granuloma involvement and to start corticosteroid pulses. Clinical and radiological symptoms improved dramatically.

[Rhodococcus equi infection in AIDS: a case with pulmonary abscess. Review of the literature]. / L'infection à Rhodococcus equi au cours du sida: un cas avec abcès pulmonaire. Revue de la littérature.

Rhodococcus equi is an easily missed opportunistic infection in patient infected by the human immunodeficiency virus. We report one case with lung abscess in a patient with AIDS with literature review.

[Primary hyperparathyroidism, a differential diagnosis of motor neuron diseases]. / Hyperparathyroïdie primitive, un diagnostic différentiel des maladies du motoneurone.

INTRODUCTION: Motor neuron diseases are always lethal. Other curable causes of neurologic disorders have to be sought. We report an example. EXEGESIS: A 72-year-old man presented a distal weakness and atrophy of the upper extremities. Electromyography showed thenar and hypothenar denervation, without fasciculation. Hypercalcemia led to the discovery of a primary hyperparathyroidism. Five months after parathyroid surgery, there was no worsening. CONCLUSION: Von Recklinghausen and Vical were the first to describe neuromuscular involvement in primary hyperparathyroidism. Faced with symptoms mimicking motor neuron diseases, calcium and phosphorus levels have to be measured because hyperparathyroidism can be cured and neurologic disorders disappear after surgery.

[Necrobiotic xanthogranuloma , a cutaneous disorder associated with monoclonal gammopathy]. / La xanthogranulomatose nécrobiotique, une histiocytose non langerhansienne à expression cutanée associée à une gammapathie monoclonale.

INTRODUCTION: Necrobiotic xanthogranuloma is a rare cutaneous disorder usually associated with monoclonal gammapathy. We describe two new cases. EXEGESIS: A 70-year-old patient was affected by a monoclonal gammopathy. She presented with a diplopia related to a retro-orbital tumor. The biopsy showed inflammatory lesions. Five years later, inflammatory xanthomatous skin lesions appeared. Biopsy specimens gave the diagnosis of necrobiotic xanthogranuloma. A 70-year-old woman was referred for inflammatory cutaneous lesions. Clinical, biological investigations and skin biopsies led to the diagnosis of cutaneous sarcoidosis associated with monoclonal gammopathy. Four years later, she developed a nephrotic syndrome. New skin biopsy specimens showed a necrobiotic xanthogranuloma. CONCLUSION: Necrobiotic xanthogranuloma is a systemic disease. It is a rare non-Langerhans cell histiocytosis characterized by frequent cutaneous and ophthalmologic lesions and associated with monoclonal gammopathy. To our knowledge, retro-orbital involvement has never been reported in necrobiotic xanthogranuloma. Treatment is difficult.

[Systemic complications of beta-blocking eyedrops. Apropos of 6 cases]. / Accidents systémiques des bêta-bloquants en collyres. A propos de six observations.

Six cases of systemic reactions to topical treatment with beta-blocking eyedrops are reported, bradycardia and faintness due to an overdosage of ophthalmic timolol; decompensated heart failure one month after the prescription of carteolol eyedrops: bronchospasm after two weeks of treatment with metipranolol eyedrops; crippling Raynaud's phenomenon of otherwise unknown origin, which had begun with timolol eyedrops, continued with carteolol eyedrops and regressed after discontinuation of ophthalmic beta-blockers; aggravation of an anaphylactoid shock in a patient treated with ophthalmic timolol, and myocardial infarction possibly due to the abrupt withdrawal of timolol eyedrops. It cannot be overstressed that the rules governing the prescription of oral beta-blockers also apply to ophthalmic preparations of these drugs: respect of contra-indications, strict adherence to the dosage recommended, gradual drug withdrawal and regular supervision. Only controlled studies and long-term follow-up will be able to demonstrate differences in safety between the five beta-blockers commercialized as eyedrops in this country.