[B cell prolymphocytic leukemia with poor prognosis]

B cell prolymphocytic leukemia is a rare disease with poor prognosis compared with chronic lymphocytic leukemia. Patient aged 75 years old, diabetic and hypertensive, with a massive splenomegaly associated with leukocytosis exceeding 100000/mm3 predominantly prolymphocytic, invading the bone marrow. Immunophenotyping showed CD19 +, CD20 +, CD22 +, CD5 part, CD23+ CD79b + FMC7 stronger and stronger, Smg + in vafour for a type B-cell prolymphocytic leukemia. The evolution was rapidly unfavorable despite an first line treatment of eight cycles of COP [cyclophosphamide, vincristine and prednisone]. Partial remission was short, followed by the reappearance of the splanomegaly, new increase in the of Prolymphocyte tate with thrombocytopenia and profound anemia. The patient had received two courses of Fludarabine. Dath occurred after eighteen months of follow up due to a state of septic shock and tumor lysis syndrome. This malignancy accounts for 2% of all chronic lymphocytic leukemia with 80% phenotype B. It frequently affects patients of 70 years old with a clear male predominance. The prognosis is more severe than that of chronic lymphocytic leukemia. Anemia <11g/dl and lymphocytosis > 100000/mm3 are the factors of poor prognosis. The median survival was 65 months reported by Shvidel et al. in a series of 35 observations. Promising results are reported with new treatments. The antibady anti-CD20 has been used successfully for the second type of B-cell prolymphocytic leukemia after relapse. The allograft or autograft are indicated, but it is mostly used in patients, who have contraindications to a heavy treatment

[Human immunodeficiency virus [HIV] associated arthritis]

Infection with human immunodeficiency virus [HIV] may be associated with different types of arthritis. The oligoarthritis related to human immunodeficiency virus is less frequent, rarely revealing the retrovirus. We report a case illustrating this association with a review of the literature. Patients aged 38 years, followed for myelodysplastic syndrome who developed a subacute oligoarthritis of the knees characterized by functional impairment. The radiograph of both knees were normal. The joint aspiration revealed sterile exudative fluid with lymphocytic predominance. Rheumatoid factor, antinuclear antibodies and HLA B27 were negative. A significant lymphopenia was found, justifying the implementation of serology for human immunodeficiency virus type 1, which returned positive. The CD4 count was a 128/mm3, the viral load of 4,000,000 copies/ ml. the patient was classified as stage C disease and has been received AZT+ 3 TC + Efavirenz, ketoprofen and paracetamol, with disappearance of oligoarthritis after 4 weeks. Aseptic arthritis can occur at any stage of HIV infection, most frequently described in an advanced stage of disease. They are found in 7.8% of cases and are rarely revealing as is the case with our patient, characterized by functional impairment and bone response to anti-inflammatory drugs. The serological testing must be systematic in an etiologic oligoarthritis

[Immune thrombocytopenic purpura]

It is characterized by a low platelet count < 150000/mm[3], which is the result of both increased platelet destruction and insufficient platelet production. In this paper, we will focus on current aspects of geoepidemiology, pathophysiology, diagnosis and management of primary immune thrombocytopenia in adult. The development of autoantibodies against platelet glycoproteins remains central in the pathophysiology of immune thrombocytopenic purpura, several abnormalities involving the cellular mechanisms of immune modulation have been identified. The goal treatment is to raise the platelet count to high enough levels to prevent bleeding. Corticosteroids, intravenous immune globulin, and splenectomy remain mainstays of treatment; however, newer therapies including rituximab and the thrombopoietin receptor agonists are remodeling conventional treatment algorithms. Effective treatments are aimed at different steps in the pathophysiologic process including the reduction of autoantibody production, interference with FcR uptake and signaling, suppression of B and T cells, and increase in TPO activity. The development of biologic therapies, particularly anti-CD20 and the emergence of new drug to increase platelet production rather than modulating the immune response, however, may radically change the management of immune thrombocytopenic purpura and make the information rarest of splenectomy

Mycophenolate mofetil in the treatment of systemic discoid lupus erythematosus

The mycophenolate mofetile has been succesfully used for the treatement of several autoimmune skin diseases in a case of a 27 year old fenale patient, who was diagnosed as acute disseminater SLE in may 2002, according to the ARA document revised in 1987. Two years later, she had an intrauterine fetal death of 32 weeks, causing a severe, psychological impact. She complained of a generalised discoid lupus C face, thorax and both upper and lower limbs]. Due to the resistant oral and local hydroxychloroquine and local dapsone treatment, she had cortisonic diabetes, aseptic necrosis of the head of the femur, and macular toxicity caused by antimalarial therapy. The skin lesions was associated with proliferative segmental and focal lupus nephritis. Induction treatment with 29 per day of mycophenolate mofetil associated with corticaltherapy by prenidsone 1 mg/ kg/ id lead to the dissaperance of the skin lessions and porteinurea

[Peripheral neuropathies with monoclonal gammopathy of undetermined significance]

The association of peripheral neuropathy and the monoclonal gammopathy of undetermined significance CMGUSD has been frequently reported in 8 to 36% of cases. While the neuropathy associated with IgM-MGUS is well characterized and is often associated with a reactivity of the monoclonal protein with neural antigens, the relationship between the neuropathy and IgG and IgA MGUS is less clear. We report two cases of sensitive-motor chronic poly neuropathies associated with IgG MGUS in the first, and IgM MGUS in the second. Their clinical symptoms are foot numbness, paresthesias, imbalance, and gait ataxia, progress about months. Treatment associate plasma exchange, corticosteroids in combination with immunosuppressant. neuropathies with IgM MGUS tend to be most refractory than IgG MGUS