Mise au point d'une thrombopénie

Thrombocytopenia is defined by a platelet level below 150 G/l. However, the limit of 100 G/l seems more appropriate to determine which thrombocytopenia will require further investigation. Initially, a thorough medical history should be performed as well as screening for any signs of bleeding. After having excluded the presence of platelet aggregates, it should be determined whether thrombocytopenia is isolated or associated with other abnormalities (cytopenias coagulation disorder, abnormal renal or liver tests). Causes of thrombocytopenia along with the biological tests to achieve diagnosis, will be detailed in this article. We will then determine the medical emergencies that will need to be addressed to a reference center: active bleeding biological signs of disseminated intravascular coagulation, acute renal failure, platelet count < 30 G/l (or < 50 G/l if the patient is on anticoagulation or antiplatelet therapy) significant and/or brutal onset pancytopenia. Outside these situations where vital prognosis is engaged, the patient should be rapidly addressed in case of platelet count between 30 and 50 G/l without any concomitant anticoagulation or antiplatelet therapy. Platelet levels between 50 and 100 G/l will require investigation, without any urgency, in outpatient haematology clinic.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

When the mask slips: A peripheral T-cell lymphoma disguised as lupus with myelofibrosis in a patient with May-Hegglin syndrome

We describe the case of a female patient with May-Hegglin syndrome who developed peripheral T-cell lymphoma not otherwise specified. The patient presents with systemic lupus erythematous phenotype and myelofibrosis secondary to T-cell lymphoma. Peripheral T-cell lymphoma not otherwise specified, represents 25 % of all peripheral T-cell lymphoma. Its diagnosis remains challenging due to the polymorphous clinical presentation and pathological heterogeneity. Myelofibrosis associated with malignant lymphomas is rare and peripheral T-cell lymphoma is even rarer. To our knowledge, this is the first case to describe an association between May-Hegglin syndrome and a peripheral T-cell lymphoma

Painful ophthalmoplegia in a patient with a history of marginal zone lymphoma

AbstractA 73-year-old man with a history of marginal zone lymphoma was admitted to the emergency room for diplopia and ipsilateral headache. The Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) demonstrated intense and symmetrical hypermetabolism of the cavernous sinuses, and hypermetabolic lesions diffusely in the lymph nodes and bones. The diagnosis of high-grade relapse of lymphomatous disease was made. In this context, the homogenous and symmetric lesion of the cavernous sinuses, without any other encephalic or meningeal lesions, raised the hypothesis of a paraneoplastic origin. A plausible paraneoplastic link between the neuro-ophthalmological lesion and the malignant disorder is IgG4-related disease, a condition that may be associated with lymphoma. As in our case, this diagnosis is often presumptive because histopathological confirmation is difficult to obtain.</jats:p

DIAGNOSIS AND TREATMENT OF PERIPHERAL T-CELL LYMPHOMAS: UPDATE RECOMMENDATIONS OF THE BELGIAN HEMATOLOGY SOCIETY (BHS)

Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of aggressive diseases associated with poor outcomes. Recent progress in understanding of the biology and pathogenesis based on molecular profiling and next-generation sequencing has led to the introduction of new provisional entities in the World Health Organization (WHO) classification system of 2017 and to the emergence of new drugs.1 Previous Belgian guidelines were published in 2013.2 This review will discuss the diagnosis, work-up and treatment of PTCL including these advances as well as the limitation of the availability of drugs according to the Belgian reimbursement rules