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Autoimmune lymphoproliferative syndrome (ALPS) represents a failure of apoptotic mechanisms to maintain lymphocyte homeostasis, permitting accumulation of lymphoid mass and persistence of autoreactive cells that often manifest in childhood with chronic nonmalignant lymphadenopathy, hepatosplenomegaly, and recurring multilineage cytopenias. Cytopenias in these patients can be the result of splen...
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This immune dysregulatory disorder is prominently associated with cytopenias and a unique combination of clinical signs and symptoms of extreme inflammation. Prompt initiation of immunochemotherapy is ...
CASE REPORTS We present two cases of blunt ocular trauma with clinical findings and complementary tests compatible with traumatic optic neuropathy (TON), and progression to sectorial optic atrophy. DISCUSSION TON have an essentially clinical diagnosis, and in the last few years has been treated with high doses of intravenous steroids, based on weak evidence of the benefit of the steroids in c...
Epinephrine autoinjectors are widely prescribed for the self-treatment of anaphylaxis. Accidental digital injection of epinephrine is an increasingly common occurrence and can be associated with profound ischemia of the affected digit. Several case reports of such injuries exist; however, it is unclear what the appropriate treatment of accidental digital epinephrine injection should be. We will...
Mantle cell lymphoma is included in the World Health Organization classification as distinct lymphoma subtype characterized by the t(11;14)(q13;q32) translocation, which results in overexpression of Cyclin D1. The clinical presentation often includes extranodal involvement, particularly of the bone marrow and gut. The prognosis of patients with mantle cell lymphoma (median overall survival, 3-5...
A 45-year-old man presented with progressively increasing breathlessness, a dry cough, drenching night sweats, and significant weight loss. Physical examination identified enlarged lymph nodes in both sides of the neck and both supraclavicular fossae. A chest radiograph showed widening of the mediastinum, which a computed tomographic (CT) scan confirmed as being the result of lymph nodes of max...
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