Three scenarios of nasal obstruction revealing sarcoidosis.

The nasal location of sarcoidosis is rare. In this regard, we report three observations of sarcoidosis observed in three women with an average age of 53.6 years, revealed by annoying nasal manifestations. The presence of extra-nasal sites allowed us to suspect the diagnosis of sarcoidosis in all the patients.

Efficacy and Safety of Rituximab in the Management of Pediatric Systemic Lupus Erythematosus: A Systematic Review.

OBJECTIVES: To evaluate the efficacy and safety of rituximab for treating pediatric systemic lupus erythematosus (pSLE). STUDY DESIGN: We performed a systematic review to evaluate the efficacy and safety of rituximab in children with pSLE. Data from studies performed before July 2016 were collected from MEDLINE, the Cochrane Library, Scopus, and the International Rheumatic Disease Abstracts, with no language restrictions. Study eligibility criteria included clinical trials and observational studies with a minimal sample size of 5 patients, regarding treatment with rituximab in patients with refractory pSLE (aged <18 years at the time of diagnosis). Independent extraction of articles was performed by 2 investigators using predefined data fields. RESULTS: Twelve case series met the criteria for data extraction for the systematic review with a good quality assessment according to an 18-criteria checklist using a modified Delphi method. Among them, 3 studies were multicenter and 3 were prospective. The total number of patients was 272. Studies collected patients with active disease refractory to steroids and immunosuppressant drugs. Refractory lupus nephritis was the most common indication (33%). Acceptable evidence suggested improvements in renal, neuropsychiatric and haematological manifestations, disease activity, complement and anti-double stranded Desoxy-Nucleo-Adenosine, with a steroid-sparing effect. However, there was poor evidence suggesting efficacy on arthralgia, photosensitivity, and mucocutaneous manifestations of SLE in children. An overall acceptable safety profile with few major adverse events was shown. CONCLUSION: Rituximab exhibited a satisfactory profile regarding efficacy and safety indicating that this agent is a promising therapy for pSLE and should be further investigated.

[Characteristics of deep vein thrombosis in the elderly]. / Particularités de la thrombose veineuse profonde chez le sujet âgé.

PURPOSE OF THE STUDY: Deep vein thrombosis is a common condition in geriatric. The identification of risk factors for venous thrombosis in the elderly is important because it allows an appropriate prescription of preventive treatments. The purpose of this study is to identify the etiologic and therapeutic characteristics of deep vein thrombosis of the lower limbs in the elderly. PATIENTS AND METHODS: A retrospective study of 155 patients with lower limb deep vein thrombosis confirmed by venous Doppler. These patients were divided into two groups: group 1 (patients aged 65 years or more) and group 2 (patients whose age was below 65 years). A comparison between these two groups was performed. RESULTS: The average age of patients in group 1 was 74.89 years (± 6.43). The sex ratio in this group was 0.68. The thrombosis was more frequently proximal in patients of group 1. Patients of group 1 had significantly more risk factors than those of group 2 (p <0.05). Among risk factors studied, only the cancers were significantly more frequent in group 1 (p = 0.002). The frequency of hemorrhagic events in both groups was comparable but bleeding was more common in patients of group 1 with cancer. CONCLUSION: Our study confirms the multifactorial origin of deep vein thrombosis in the elderly and the incidence of malignant etiology. The results of this study also call for vigilance in the use of anticoagulants in the elderly especially in the presence of cancer because it is an additional risk factor for bleeding.

[Syndrome d'activation macrophagique: une série de 11 cas tunisiens]. / Hemophagocytic syndrome: a study of 11 patients in tunisian population.

BACKGROUND: Hemophagocytic syndromes are mostly associated with underlying pathology, they can reveal: immunodeficiency, infections, hemopathies, cancers and auto-immune diseases. AIM: to investigate clinical, biological features, outcome characteristics and underlying pathology of Tunisian patients with hemophagocytic syndromes. METHODS: A retrospective study of patients with hemophagocytic syndromes admitted in an internal medicine department in Tunis over the period 2009-2012. RESULTS: There were 11 patients included, 4 men and and 7 women. Mean age was 47,3years. Purpura was observed in 3 cases, hepatosplenomegaly in 6 cases and peripheral lymph nodes in 3 cases. Hemorrhagic complication was noted in 2 cases. There were an increase of inflammatory indices in all the cases. Eight patients had pancytopenia and 3 had bicytopenia. Six patients developed intravascular dissiminated coagulation. High levels of triglyceridemia was noted in 5 cases and an hyperferritinaemia in all cases. Cytological examination of bone marrow confirmed hemophagocytosis in ten cases. Hemophagocytosis was associated to infectious diseases in 6 cases, there were 2 cases of leishmaniasis, septicemia to E.Coli and staphylococcus aureus and lymph nodes tuberculosis with HIV. We also noted a case of lymphoma, myelodysplasic syndrome, rheumatoid polyarthritis, adult onset still's disease. In one patient, the origin of hemophagocytosis remained indefinite. Visceral leishmaniose were treated by Glucantime®, adult Still's disease by corticosteroids and méthrotrexate®, lymphoma by chemotherapy. In lymph nodes tuberculosis, there were good evolution. Six patients died. CONCLUSION: Hemophagocytic syndrome is a rare and life-threatening disease. Pejorative prognosis requires an early therapy with etiological treatment.

[A leukemoid leucocytosis]. / Une hyperleucocytose « leucémoïde ¼

Leukemoid reaction is a rare paraneoplastic syndrome. It can occur in association with carcinomas, in particular of the lung, gastric and renal. However, its association with sarcoma is infrequent. Leukemoid reaction occuring in patients with uterine sarcoma have not been previously reported. We report the case of a « leukemoid ¼ leucocytosis revealing an uterine sarcoma with fatal evolution.

Evaluation of anticoagulant therapy management in a teaching hospital in Tunisia: information and education for vitamin k antagonist newly treated patients need to be improved.

OBJECTIVE: The objective of this study was to assess the level of Vitamin K antagonist (VKA) therapy knowledge among VKA newly treated patients. METHODS: Questionnaire-based interviews were administered in a university hospital to VKA newly treated patients covering both cognitive (i.e. indication, route of administration, treatment monitoring and side effects) and know-how (i.e. safety precautions, what affects anticoagulant therapy and the management of overdose signs) aspects of therapy knowledge. KEY FINDINGS: A total of 55 patients were included. The mean overall score of patients' knowledge was 5.78 (SD = 3.425) out of 20. The mean scores of cognitive and practical knowledge were 3.8 (2.26) and 1.98 (1.78) out of 10, respectively. Factors associated with an insufficient level of VKA therapy knowledge included low level of education, lack of access to therapy information and continuous access to this information. CONCLUSIONS: Knowledge about VKA therapy among newly treated patients is insufficient. Patient's medication knowledge should be improved to ensure better adherence.

Gelatinous Transformation of Bone Marrow in a Patient with Anorexia Nervosa: An Uncommon but Reversible Etiology.

BACKGROUND Anorexia nervosa is a relatively frequently encountered eating disorder. Beyond its psychiatric significance, it is associated with multiple hematological features such as cytopenia. It can be related to gelatinous transformation of the bone marrow. CASE REPORT We describe the case of a 20-year-old male admitted for rapid involuntarily weight loss. He presented with extreme bradycardia with a heart rate of 30 beats per minute with recent psychomotor impairment. Routine laboratory tests showed pancytopenia, and cytolysis on liver function tests. Cardiac investigations did not reveal abnormalities except for sinus bradycardia. Bone marrow aspirate revealed hypo-cellular marrow with infiltration by gelatinous substance. In addition, bone marrow biopsy showed fat cell atrophy along with loss of hematopoietic elements in areas of gelatinous transformation. During the patient's hospital stay, he had a restrictive oral diet. Thus, after consulting with a psychiatric specialist, a diagnosis of anorexia nervosa complicated with gelatinous transformation of bone marrow, bradycardia, and acute hepatitis was made. After psychiatric treatment and nutritional care, the patient regained weight, had a normal heart rate and normal liver function tests, and his full blood count and bone marrow examination were within normal range. CONCLUSIONS Gelatinous transformation of bone marrow can be the revealing feature of anorexia nervosa and some potentially reversible associations might prove life threatening if misdiagnosed. Furthermore, lack of suspicion can lead to unwarranted investigations increasing the cost incurred in healthcare delivery. Our patient had 3 systems involved: cardiac, hepatic, and hematological systems. Gelatinous transformation of bone marrow, although a rare condition, must be considered in such patients.

[Cutaneous lupus profundus]. / Le lupus cutané profond.

The objective of this studies is to review clinical and laboratory features of lupus panniculitis. The authors report 3 cases of lupus profundus from a group of 70 lupus erythematosus. In both cases the lupus panniculitis presented as subcutaneous infiltrated and indurated nodules. The diagnosis was confirmed on clinical, histological and therapeutic data. The evolution is slow and is characterised by regression of the inflammatory lesions with treatment by antimalarial drugs. The lupus panniculitis has generally a favorable prognosis.

Deep vein thrombosis and tuberculosis: a causative link?

Pulmonary tuberculosis is very devastating in developing countries and its thrombogenic potential is a disturbing new entity. We report an 18-year-old woman who presented with a first episode of deep vein thrombosis. Pulmonary, hepatic and splenic tuberculosis was diagnosed while looking for secondary causes. The patient was treated with rifampicin, isoniazid, pyrazinamide and ethambutol along with low-molecular-weight heparin and antivitamin K. Tuberculosis has several mechanisms that induce a hypercoagulable state and can lead to thromboembolic complications.

Liver involvement in tuberous sclerosis.

Tuberous sclerosis complex (TSC) is a rare inherited disease that affects many organs and tissues, which explains the diversity of its clinical manifestations. This disease is often diagnosed at an early age when cutaneous angiofibromas, epilepsy and mental retardation are associated. The hepatic involvement in this phakomatosis is an uncommon location. In this context, we report the case of a tuberous sclerosis revealed in adulthood by abdominal pain and distension related to renal and hepatic angiomyolipomas associated with typical skin lesions.

Orbital metastasis as the inaugural presentation of occult rectal cancer.

Orbital metastasis is uncommon and occurs in 2-3% of patients with cancer. It is rarely the initial manifestation of a systemic malignancy. It usually indicates extensive haematogenous dissemination of a primary cancer and is associated with poor prognosis. Breast, lungs and prostate cancers are the most common primary cancers leading to orbital metastasis. However, orbital tumour revealing a rectal adenocarcinoma is exceptional. We describe a case of orbital tumour in a 67-year-old man with no history of systemic cancer while presenting with ophthalmic symptoms. Investigations revealed rectal adenocarcinoma as the primary malignant tumour.

Haemophagocytic syndrome with disseminated intravascular coagulation associated with tuberculosis.

Haemophagocytic syndrome (HPS) is a clinical entity that combines non-specific clinical and biological features. The diagnosis is usually confirmed by a bone marrow examination. HPS may be primary or secondary to a malignancy or to an infectious or autoimmune disease. Early aggressive survey of the aetiology and optimal treatment of the underlying disease improve the outcome of life-threatening HPS. Infection-associated HPS occurs predominantly in immunocompromised patients and is usually fatal. Leading trigger agents are viruses, especially the Epstein-Barr virus  and cytomegalovirus. Mycobacterial infections associated with HPS are rare but should be considered in those patients where there is associated fever of unknown origin. We present a case of disseminated tuberculosis-associated HPS.

Sternal tuberculosis in an immunocompetent adult.

Skeletal tuberculosis accounts for 1-3% of patients with mycobacterial infection. Any bone can be a site for tuberculosis, but sternum involvement is quite rare. We report the case of a 37-year-old woman admitted because of chest pain and increased swelling over the anterior chest. She was immunocompetent and had no systemic features. She was diagnosed with tuberculosis of the sternum without active pulmonary disease. Conservative management with oral multidrug antituberculous therapy completely cured the patient.