[Acquired, non-amyloid related factor X deficiency: A first case associated with atypical chronic lymphocytic leukemia and literature review]. / Déficit non amyloïde acquis en facteur X : un premier cas satellite d'une leucémie lymphoïde chronique atypique et revue de la littérature.

INTRODUCTION: Acquired factor X deficiency is in most cases associated with AL amyloidosis. Acquired non-amyloid related factor X deficiency (DNAA-FX) has been exceptionally reported in the literature. CASE REPORT: We report the first case of acquired, non-amyloid related factor X deficiency associated with atypical chronic lymphoid leukemia in a 66-year-old patient with spontaneous hematomas. After therapeutic failure with polyclonal intravenous immunoglobulins, specific lymphoid malignancy treatment allowed symptoms and coagulation disorder resolution. CONCLUSION: DNAA-FX should be considered in case of bleeding events or coagulation disorders during low-grade hematological malignancies. Its occurrence can be considered as a treatment indication to prevent potentially fatal bleeding complications.

[Bing-Neel syndrome: Report of 4 cases and literature review]. / Syndrome de Bing et Neel : à propos de 4 cas et revue de la littérature.

INTRODUCTION: Neurological manifestations during Waldenstrom disease are common and are usually related to immune peripheral neuropathy or serum hyperviscosity syndrome. The infiltration of the central nervous system by the lymphoproliferative syndrome is known as the Bing-Neel syndrome. This extremely rare entity remains poorly described in the literature. CASE REPORTS: We report on 4 cases of patients for whom central neurological disorders led to the diagnosis of a Bing and Neel syndrome. These four cases illustrate different clinical presentations, diagnosis, therapeutic options, and outcome in this syndrome. Based on our literature review, we discuss about these differences. CONCLUSION: The polymorphic clinical manifestations of Bing and Neel syndrome can mimic many diagnoses. However, it may be necessary to consider this diagnosis. Cerebrospinal fluid analysis and MRI may allow rapid diagnosis or guide a biopsy. Prolonged remissions are possible with appropriate treatment.

[Can we prevent or cure infection-related cancers?]. / Peut-on prévenir ou guérir les cancers d'origine infectieuse?

Infections are an important cause of cancer in the world, representing approximately 16% of the neoplasia. Ten infectious agents have been classified as carcinogens of group I. Four of these pathogens (Helicobacter pylori, hepatitis B and C viruses, and some human papillomavirus) are responsible for 95% of cases of infection-related cancers. The carcinogenesis mechanisms are multiple, either direct via certain proteins from these microorganisms, or more often indirect through chronic inflammation. This allowed to consider prevention of certain cancers, for example with a prophylactic vaccine strategy. Advances were also made in the curative field. However, efforts remain to be done to discover new infectious causes of cancer and refine the understanding of the mechanisms of carcinogenesis, for a better targeting of anticancer therapeutics.

[Practice assessment in platelet transfusions]. / Évaluation des pratiques transfusionnelles plaquettaires.

PURPOSE OF THE STUDY: Platelet transfusion follows the national guidelines published in 2003 by the AFSSAPS, determining, for instance, indications, transfusion threshold and platelets dose. We wanted to assess how these guidelines are routinely used in our hospital, with a special focus on transfusion threshold and delivered dose. MATERIAL AND METHODS: We conducted a prospective study during 11 months on every platelet transfusion. Our establishment is a medium size structure, devoted to emergency and oncology, without bone marrow transplantation. During this period, 235 products were delivered to 105 patients. Half (52%) were delivered to oncological units, a third to emergency units and the remaining to medical and surgical units. RESULTS: The average dose was 4.3±0.8×10(11) platelets (2.0 to 7.6×10(11) platelets), corresponding to 0.45×10(11) platelets per 7kg. During prophylactic transfusions, the average platelet count was 9.4±5.5G/L ; during curative transfusions (43%), it was 39.0±47.8G/L and finally when platelets were infused during surgery (21%), the average platelet count was 57.8±61.4G/L. CONCLUSION: Globally, with regard to transfusion threshold, guidelines were followed in 71%, and 93% in oncological units. Transfusion efficacy, attested by post-transfusion platelet efficiency was above 20% in 59% of the cases. These data highlight a good respect of the transfusion thresholds in the usual platelets-consuming units, but raise the question of the dose, often under those proposed by the guidelines.

[Myelodysplastic syndrome with erythroblastopenia]. / Syndromes myélodysplasiques érythroblastopéniques.

Myelodysplastic syndrome with erythroid hypoplasia or erythroblastopenia has not yet been clearly defined, and in most patients it is mistaken for acquired pure red cell aplasia. Including one additional patient reported in this article, a literature review revealed only 50 cases over the last 20 years. These patients were predominantly elderly males, all required regular packed red cell transfusions, and they had a poor prognosis, mainly because of acute transformation. The mechanisms of erythroid aplasia remain unclear. However, recent data suggest the association of an intrinsic stem cell defect with immunological implication.

[Synchronous detection of T-cell clonality and FIP1L1-PDGFRA fusion gene in a hypereosinophilic syndrome]. / Détection simultanée d'une clonalité T et d'un transcrit FIP1L1-PDGFRA au cours d'un syndrome hyperéosinophilique.

We report a 49-year-old man suffering from chronic hypereosinophilia whose biological tests revealed a gene rearrangement between FIP1L1 and PDGFRA as well as a T-cell clonality. After 1 year of therapy with imatinib mesylate (100 mg daily), the patient was clinically asymptomatic, the fusion transcript was undetectable using RTQ-PCR and no lymphoproliferative disorders occurred. This unique combination raises the question of the physiopathology of such a grey zone hypereosinophilia and their management.

[Macrophage activation syndrome as the presenting feature of a Hodgkin's lymphoma in an elderly patient]. / Syndrome d'activation macrophagique révélant une maladie de Hodgkin chez un sujet âgé.

Macrophage activation syndrome (MAS) is a rare immunological disorder, rapidly life-threatening in the absence of specific treatment. Malignant lymphoma is common cause of MAS, but Hodgkin's lymphoma has been rarely associated with MAS. We report a 79-year-old man who presented a MAS as the presenting feature of Hodgkin's lymphoma, with a rapidly fatal outcome. We discuss the usefulness of sCD25 and interleukine 18 assays when diagnosis is difficult to obtain. We also review the literature data related to Hodgkin's lymphoma-associated MAS.

Safety and efficacy of rituximab in nonviral cryoglobulinemia vasculitis: data from the French Autoimmunity and Rituximab registry.

OBJECTIVE: Management of nonviral cryoglobulinemia vasculitis has yet to be defined. Rituximab has emerged as a novel and promising therapeutic alternative, but data are scarce. Our objective was to evaluate the safety and efficacy of rituximab in nonviral cryoglobulinemia vasculitis in off-trial real-life patients. METHODS: Prospective data from the French AutoImmunity and Rituximab (AIR) registry, which includes data on patients with autoimmune disorders treated with rituximab in off-label conditions, were analyzed. RESULTS: Twenty-three patients received treatment with rituximab for cryoglobulinemia vasculitis. Tolerance was marked by the occurrence of side effects in almost half of the patients, including severe infections in 6 (26%) of 23, with a rate of 14.1 per 100 patient-years. These infections occurred in a particular subset of patients ages>70 years, with essential type II mixed cryoglobulinemia and renal failure with a glomerular filtration rate of <60 ml/minute, and receiving high-dose corticosteroids. Three of these patients died. In contrast, clinical and immunologic efficacy was noted in all evaluable patients. Clinical relapses occurred in half of the patients after a median time of 13.5 months following rituximab administration, and were more frequent in patients refractory to previous immunosuppressive therapy than in previously untreated patients. CONCLUSION: Data from the AIR registry show a dramatic efficacy and a steroid-sparing effect of rituximab, but also show the occurrence of severe infections in elderly patients with renal failure and high-dose steroids. The role of rituximab in nonviral cryoglobulinemia vasculitis remains to be defined in well-designed randomized controlled trials.

[Recurrence of thromboembolic disease after splenectomy for hereditary xerocytosis]. / Maladie thromboembolique récidivante après splénectomie pour stomatocytose héréditaire.

CASE REPORT: The diagnosis of hereditary xerocytosis is made in a 57 year old woman splenectomized 30 years ago for a chronic hemolytic anemia. In following, she developed many thrombophlebitis of lower limbs and portal vein. DISCUSSION: The methods of diagnosis of this rare hereditary stomatocytosis are recalled, and the mechanisms of thrombotic tendency after splenectomy are discussed. This case underlines the fact that splenectomy is banned in the treatment of hereditary stomatocytosis, and that the serious consequences of iron overload, which is very frequent in this disease, must be prevented.

[Interest of careful blood film observation in haemolytic anaemia diagnosis]. / L'intérêt de l'examen attentif du frottis sanguin dans le diagnostic des anémies hémolytiques.

The discovery of anaemia raises the question of its aetiology. If many causes can easily be established, some remain without any accurate diagnosis. The mere issue is that their causes can be rare or mechanisms interlinked. A blood film examination by the biologist provides helpful elements to guide the diagnosis, it can be improved if it is orientated by the physician who prescribed it. Two cases of late discovery of haemolytic anaemia, in relation with red cell membrane disorders, are reported. They illustrate the interest of a good collaboration between the physician and the biologist that lead to diagnosis. The first case is about a band-3 protein defect, the second deals with an hereditary dehydrated stomatocytosis.

[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.

[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.

[Left atrial thrombus in multiple myeloma treated with thalidomide]. / Thrombus de l'oreillette gauche au cours d'un myélome multiple traité par thalidomide.

The occurrence of a left atrial thrombus without a haemodynamic predisposing factor (arrhythmia, mitral valvulopathy, severe left ventricular dysfunction) is a rare event. We report a case during the progression of refractory myeloma, four months after stopping treatment with thalidomide. The promoting haemodynamic factors for left atrial thrombosis in sinus rhythm, described in the literature, had been excluded. In our case the potential role of thalidomide is debatable, in the light of recent publications about venous and arterial thromboses observed with this treatment.