Which role for rituximab in hairy cell leukemia? Reflections on six cases.

Hairy cell leukemia (HCL) is a rare, chronic, B-cell, lymphoproliferative disorder. Treatment has been revolutionized by the advent of interferon (IFN)-alpha and purine analogs (PA). First-line therapy with PA yields complete response rates of 75-100%, with many long-lasting remissions. In the event of profound neutropenia and/or infectious complications, a short sequence of IFN-alpha may precede PA treatment. Because of the excellent results achieved with PA therapy, the potential role of rituximab (an anti-CD20 monoclonal antibody that is highly effective against most B-cell lymphomas) in HCL has yet to be elucidated. Six HCL cases treated with rituximab are reported herein with a view to elucidating the potential role of the drug in HCL. The indications essentially consist of relapsing or refractory disease, avoiding the cumulative toxicity of PA, consolidation therapy in order to eradicate minimal residual disease, and first-line therapy for patients with contraindications to PA and IFN-alpha.

[Acute meningoencephalitis revealing a Behçet's disease]. / Méningoencéphalite aiguë révélatrice d'une maladie de Behçet.

We report a 69-year-old man admitted in intensive care unit for aseptic meningoencephalitis. Initially, suspicion of an infectious etiology led to introduce an anti-infectious treatment. Behçet's disease was diagnosed during hospitalization incited to screen for noninfectious etiologies. A high dose steroid therapy was rapidly effective. The diagnosis of neuro-Behçet's disease was entertained.

Acute liver failure is frequent during heat stroke.

Acute liver failure (ALF) is relatively frequent during heat stroke (HS). This risk must be emphasized, because its incidence is higher than is usually thought. In a recent study by Weigand et al, two cases were reported in which liver failure was the leading symptom. We have confirmed their conclusion in a study of 25 cases of HS with ALF, compared with 25 other cases without ALF. Moreover, we observed that hypophosphatemia on admission could predict occurrence of ALF during HS. As for clinical and other biological parameters, phosphatemia should be monitored for at least 3 d in all cases of HS, even when it is thought to be mild.

[Bisalbuminemia: A case report]. / Les bisalbuminémies : à propos d'un cas.

INTRODUCTION: Bisalbuminemias consist in rare qualitative modifications of several aspects in the albumin pattern. Bisalbuminemias, most of which are not pathological, can be observed using capillary electrophoresis. CASE REPORTS: We report a case of hereditary bisalbuminemia diagnosed by chance while exploring chronic unexplained hypereosinophilia in a 42-year-old patient. The patient's normal lipid profile, the lack of an antibiotic treatment or pancreatic pathology, and the persistence of the bisalbuminemia after one month, suggested a diagnosis of genetic bisalbuminemia. In light of other such cases, we review the main causes of bisalbuminemia, both genetic and acquired. CONCLUSION: The diagnosis of genetic bisalbuminemia is made by first eliminating the usual acquired etiologies: analytical or drug interference, acute pancreatitis and binding of immunoglobulins. Then, after having checked the lipemic index, reviewed the patient's medical history, comorbidities, and treatments, repeating the electrophoresis will help identify the cause of the bisalbuminemia.

[Prostatic actinomycosis in a diabetic patient]. / Actinomycose prostatique chez un patient diabétique.

INTRODUCTION: Prostatic localization of actinomycosis is unusual. CASE REPORT: We report the case of a 59 years-old diabetic man, hospitalised for a prostatic actinomycosis spontaneously fistulised in the rectum. Two species of Actinomyces were found in blood culture and in pus of the fistula. DISCUSSION: Therapeutic management including surgical treatment of the fistula and a specific antibiotherapy led to cure the prostatic infection due to Actinomyces.

[Role of pharmacogenetics in chemotherapy of colorectal cancers]. / Implication de la pharmacogénétique dans les traitements des cancers colorectaux.

SCOPE: Clinical implications associated with polymorphisms in drug-metabolizing genes involved in the chemotherapy of colorectal cancers (5-flurorouracil, oxaliplatin and irinotecan) are reviewed. CURRENT SITUATION AND SALIENT POINTS: Treatments of colorectal cancers have been greatly improved last years but patients respond differently to identical medication. Genetic polymorphisms are one of the major causes of these individual responses to drugs associated with sometimes severe adverse effects. Pharmacogenetics is based on all polymorphisms that determine genetic human diversity associated with variable response to anticancer drugs. PERSPECTIVES: Morbidity and mortality related to toxicity or inefficacy of these drugs could be reduced by analyzing the pharmacogenetic profile of patients before treatment. Results should be integrated in protocols for monitoring and assessment the dosage of drugs.

[Early myocardial infarction associated with chemotherapy for testicular cancer]. / Infarctus du myocarde précoce après chimiothérapie pour tumeur testiculaire.

INTRODUCTION: In long-term survivors of testicular cancer, a greater risk of developing cardiovascular disease is reported. On the other hand, acute vascular event during chemotherapy is uncommon. CASE REPORT: We report on a case of acute myocardial infarction in a young man receiving chemotherapy (BEP) for testicular cancer. DISCUSSION: We suggest a causal association between chemotherapy and early myocardial infarction. The physiopathological mechanisms are discussed here.

[Acute abdominal pain in a 8-year-old girl with Epstein-Barr infection]. / Douleur abdominale aiguë au cours d'une primo-infection par le virus d'Epstein-Barr.

Abdominal pain is uncommon in patient with Epstein-Barr infection and is usually attributed to an enlargement of the liver or spleen. We report on an 8-year-old girl with a pseudoperitonitis due to a mesenteric lymphadenitis associated with Epstein-Barr infection. Outcome was favourable without surgery. We review the different causes of abdominal pain occurring during Epstein-Barr infection.

[Heterozygous prothrombin gene mutation G20210A and associated diseases]. / Mutation G20210A du gène de la prothrombine à l'état hétérozygote et pathologies associées.

PURPOSE: Prothrombin gene mutation G20210A (factor II) is, in frequency, the second genetic polymorphism involved in venous thrombosis. We report a retrospective studies on 38 patients issued from our medical department, all heterozygous for the factor II mutation and a literature review. METHODS: We have studied 38 patients, all heterozygous for the factor II mutation, selected through a population of 516 tested patients issued from our medical department from 1997 to 2002. The research was performed face with history of thrombotic or obstetrical events, angiopathy or familial screening. RESULTS: Twenty out of thirty-eight patients have at least one episode of venous thrombosis: superficial thromboses, deep thromboses and/or pulmonary embolism. One case of cerebral thrombophlebitis is observed. Venous thrombotic risk factors are associated in 12 cases (60%). Four out of thirty-eight patients have one episode of arterial thrombosis: cardiovascular, peripheral or cerebral. Arterial thrombotic risk factors are associated in all cases. Median age of the first venous thrombosis is earlier than the one of arterial thrombosis (39.11 versus 49.25 years). CONCLUSION: Our studies confirms the interest to search the prothrombin gene mutation when faced with a venous thrombotic event (deep vein thrombosis and/or pulmonary embolism) with or without acquired risk factors. Its involvement in thrombotic arterial disease is still a matter of debate. Data concerning its involvement in systemic diseases and angiopathies (thromboangeitis obliterans, Raynaud's phenomenon and migraine) are still needed. Mechanisms of thromboses could be an increase of prothrombin plasma level with high thrombin synthesis.

[Pleuropulmonary manifestations of necrotising vasculitis]. / Manifestations pleuropulmonaires des vascularites nécrosantes.

The pleuropulmonary manifestations of necrotising vasculitis are frequent and polymorphic. If the existence of extrapulmonary signs and the presence of neutrophil polynuclear anticytoplasmic antibodies are helpful for the diagnosis of a bout of vasculitis, the existence of pleuropulmonary symptoms can also make for discussion of infections or iatrogenic effects induced by immunosuppressive treatments.