Autoimmune haemolytic anaemia in pancreatic adenocarcinoma: a potential paraneoplastic presentation.

While autoimmune haemolytic anaemia (AIHA) is a well-known paraneoplastic syndrome in haematological malignancies, it has been described far less in solid tumours. We hereby report the case of a 61-year-old male patient presenting AIHA related to pancreatic cancer. Investigations excluded infectious, autoimmune and toxic causes of AIHA. CT of the abdomen highlighted the presence of hypodense, infiltrating mass of the tail of the pancreas measuring 70×37×36 mm, compatible with pancreas neoplasm. Histological examination of two associated nodular lesions of the liver showed metastasis of pancreatic adenocarcinoma. The patient was started on corticosteroid without improvement of haemoglobin. Palliative chemotherapy was initiated; this led to significant improvement in haemoglobin comforting our diagnosis. This case illustrates the rare association between AIHA and pancreatic cancer. However, such association cannot be considered before excluding other, more frequent, aetiologies.

Deep vein thrombosis of the left arm revealing an asymptomatic pulmonary tuberculosis.

Although not frequent, thromboembolic events occurring in patient presenting tuberculosis may be observed. Frequency of such thromboembolic events seems correlated with the severity of tuberculosis. We herein report a case of venous thrombosis revealing an asymptomatic tuberculosis. A man aged 32 years was admitted for deep and superficial vein thrombosis of the left arm. Chest CT angiography ruled out pulmonary embolism and showed a mediastinal and pulmonary heterogeneous necrotic mass at the apical segment of the left lung. Histological study of a CT scan-guided pulmonary biopsy showed signs of caseating granuloma compatible with tuberculosis. Diagnosis of venous thrombosis due to pulmonary tuberculosis with lymph nodes involvement was made. Antituberculosis treatment was initiated and required an adjustment of anticoagulant therapy. Our observation highlights the possibility of causative effect between tuberculosis and venous thrombosis.

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.

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.

Interstitial Cystitis: An uncommon revelation in primitive Sjögren syndrome.

Interstitial cystitis is an exceptional entity during primary Sjögren's syndrome. In this regard, we report the case of a 67-year-old patient in whom initially idiopathic interstitial cystitis revealed primary Sjögren's syndrome after 5 years of evolution in front of xerostomiaa, xerophtalmia and bilateral parotid hypertrophy with histological confirmation at the biopsy of accessory salivary glands.

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.

[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.

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.

[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.