[Acquired hemophilia A associated with chronic myelomonocytic leukemia successfully treated by rituximab and azacitidine]. / Hémophilie A acquise associée à une leucémie myélomonocytaire chronique traitée efficacement par rituximab et azacitidine.

INTRODUCTION: Acquired hemophilia A is a factor VIII deficiency related to anti-factor VIII immunoglobulins. We are reporting the case of a patient with acquired hemophilia A related to chronic myelomonocytic leukemia. CASE REPORT: A 74-years old woman had a spontaneous calf hematoma with compression syndrome. Coagulation tests revealed an extended partial thromboplastin time and a factor VIII deficiency with circulating factor VIII antibodies. Chronic myelomonocytic leukemia was diagnosed in the same time. Initial management included hemostatic transfusion and steroids and was completed rituximab and azacitidine. Evolution was favorable. CONCLUSION: We report a case of acquired hemophilia A complicating a chronic myelomonocytic leukemia. Management with steroids, rituximab and azacitidine was successful. Useful of rituximab during acquired hemophilia is proposed.

Clinical characteristics and prognostic factors of plasmablastic lymphoma patients: analysis of 135 patients from the LYSA group.

Background: Plasmablastic lymphoma (PBL), initially described in 1997 in the oral cavity of HIV positive patients, is now recognized as a distinct aggressive and rare entity of diffuse large B-cells lymphoma by the World Health Organization (WHO) classification. Since the original description, others cases have been reported. However, these are largely derived from case reports or small series limiting any definitive conclusions on clinical characteristics and outcome. Patients and methods: The clinical, biological, pathological features and outcome of a cohort including 135 patients with PBL, from LYSA centers in France and Belgium, were reported and analyzed. Results: The median age was 58 years, with a male predominance. The cohort was divided into 56 HIV-positive patients, 17 post-transplant patients and 62 HIV-negative/non-transplanted patients. Within HIV-negative/non-transplanted, a relative immunosuppression was found in most cases (systemic inflammatory disease, history of cancer, increased age associated with weakened immune system). We have also described a new subtype, PBL arising in a chronic localized inflammatory site, without any sign of immunosuppression. At presentation, 19% of patients showed oral involvement. Immunophenotype showed CD138 positivity in 88% of cases and CD20 negativity in 90% of cases. Chemotherapy was administered to 80% of patients, with a complete response (CR) rate of 55%. The median overall survival (OS) was 32 months. In univariate analysis, HIV positive status showed better OS when compared with HIV negative status. In multivariate analysis, International Prognostic Index score, chemotherapy and CR were associated with survival benefit. Conclusion(s): This cohort, the largest reported to date, increases the spectrum of knowledge on PBL, rarely described. However, specific guidelines to clarify treatment are lacking, and may improve the poor prognosis of this rare disease.

[Association of lymphoma and granulomatosis: A case series]. / Association lymphome et granulomatose : à propos d'une série de cas.

PURPOSE: The sarcoidosis-lymphoma syndrome is a recognised entity. However, the presence of granulomas in patients with a haematological disease should not lead too easily to a diagnosis of sarcoidosis. The presence of granulomatous lesions during the follow-up of these patients raises diagnostic and therapeutic issues. METHODS: We included 25 patients followed by the department of haematology in a French hospital (Centre Léon-Bérard). These patients presented with granulomatous lesions. Patients with a history of sarcoidosis were excluded. We report the type of haematological disease, the time of onset of the granulomatous disease compared to that of lymphoma, associated symptoms, aetiology and outcome. Patients were divided into three groups according to the time of onset of the granulomatous lesions. RESULTS: Granulomatous lesions appeared before the haematological disease in 4 cases, was concomitant in 8 cases and appeared later in 13 remaining cases. The two main subtypes of lymphoma encountered were: diffuse large cell lymphoma (36%) and Hodgkin's lymphoma (28%). Granulomatous lesions were related to the progression of the hematological disease in 11 cases, to sarcoidosis in 4 cases, to infection in 3 cases, to drug allergy in one case, to inflammatory bowel disease in one case, to granuloma annulare in one case and was isolated in 4 cases (no identified etiology). In the group where granulomas appeared after the haematological disease, mean SUV was 11 for the haematological disease versus 6.4 for granulomas. CONCLUSION: Granulomatous diseases in lymphomas can be due to various aetiologies: infection, reaction to the haematological disease, or systemic sarcoidosis. It is an important challenge for clinicians, who can miss the diagnosis of lymphoma and or conclude to a treatment failure or a relapse. Computed tomography scan (CT-scan) or (18)F-deoxyglucose-positron emission tomography scan can help establish a diagnosis but do not replace biopsy.

Sarcoidosis and lymphoma: a comparative study.

AIM: To assess the clinical features and outcome of lymphoma when associated with sarcoidosis and to determine whether this association gives lymphoma a better prognosis. DESIGN: Multicentre retrospective cohort study. METHODS: Retrospective chart review. RESULTS: Twenty-one patients were included (9 males, 12 females). Median age at sarcoidosis diagnosis was 48 years (range: 24-68 years). In 14 cases, lymphoma occurred within a previously known sarcoidosis. Five patients received a concomitant diagnosis of sarcoidosis and lymphoma, whereas lymphoma preceded sarcoidosis in two patients. Three patients were diagnosed with Hodgkin's lymphoma and 18 patients with non-Hodgkin's lymphoma (diffuse large B-cell lymphoma (DLBCL) (n = 11), follicular lymphoma (n = 2), chronic lymphocytic leukemia/small lymphocytic lymphoma (n = 2), anaplastic large cell lymphoma ALK + (n = 1), angioimmunoblastic T-cell lymphoma (n = 1) and T-cell prolymphocytic leukemia (n = 1)). Thirteen patients were alive and in complete remission. Median age at the time of diagnosis of sarcoidosis was lower in patients with concomitant lymphoma compared with patients with sarcoidosis preceding lymphoma (34 years vs. 51 years, P = 0.01). Patients presenting with DLBCL associated with sarcoidosis were compared with DLBCL without sarcoidosis. No statistical difference was found in the risk of death or progression between the two groups (P = 0.685). CONCLUSIONS: We report here the largest series of lymphoma associated sarcoidosis patients. As opposed to previous studies, we observed a predominance of patients with DLBCL. Our study confirms the concept of the sarcoidosis-lymphoma syndrome. Large B-cell lymphoma does not have a better prognosis when associated with sarcoidosis.

[Recurrent aseptic meningitis secondary to nonsteroidal anti-inflammatory drugs in a patient with lupus]. / Méningite aseptique récidivante due aux anti-inflammatoires non stéroïdiens chez une patiente lupique.

We report a 39-year-old woman with systemic lupus who presented with recurrent aseptic meningitis secondary to treatment with nonsteroidal anti-inflammatory drugs (NSAIDs). She presented two episodes following ibuprofen administration that were characterized by aseptic meningitis with high protein level in cerebrospinal fluid, and increased serum acute phase reactants. No evidence of an infection or vasculitis was documented. Clinical manifestation resolved rapidly with ibuprofen discontinuation, and corticosteroids therapy was unnecessary. Aseptic meningitis related to NSAIDs reported in lupus patients should be considered because of their specific modality of care and their favourable outcome.