ABSTRACT
El síndrome de activación macrofágica (SAM) presenta criterios clínicos y de laboratorio establecidos. Presentamos el caso de un adolescente varón con debut de Lupus eritematoso generalizado pediátrico grave, donde su manifestación principal fue un SAM y el receptor de interleucina 2 soluble en suero (IL-2rs) o CD25 soluble (CD25s) aumentado resultó clave en la confirmación diagnóstica, en el tratamiento y pronóstico de su enfermedad. Sin embargo, este receptor de citocinas no se mide habitualmente en la práctica clínica.
Macrophage activation syndrome (MAS) presents established clinical and laboratory criteria. We present the case of a male adolescent with the onset of severe pediatric systemic Lupus erythematosus, manifested mainly by MAS and how a laboratory marker, serum soluble interleukin-2 receptor (IL-2rs) or altered soluble CD25(CD25s), played a key role in treatment and prognosis of the disease. However, this cytokine receptor is rarely measured in clinical practice.
Subject(s)
Humans , Male , Child , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/therapy , Macrophage Activation Syndrome/diagnosis , Macrophage Activation Syndrome/therapy , Thorax/diagnostic imaging , Radiography, Thoracic/methods , Receptors, Interleukin-2 , Macrophage Activation Syndrome/pathology , Lupus Erythematosus, SystemicABSTRACT
Phlegmasia Caerulea Dolens is a rare complication of deep vein thrombosis. It presents with a sudden onset of pain, swelling, discoloration and arterial compromise of the affected limb. Theres usually history of prothrombotic events such as malignancy, femoral vein catheterism, antiphospholipid syndrome, recent surgery, pregnancy, etc. Left without treatment, it can evolvein to gangrene, septic shock and death. Diagnosis usually only requires clinical appreciation.Confirmation can be done with ultrasonographic studies with doppler. Treatment can be both medical and surgically based. Medical therapy can be done with heparin and elevation of the affected limb or the use of thrombolytic, whilst surgical therapy can be either venous thrombectomy or amputation. We present the case of a 57-year old smoker, diabetic, and with systemic lupus erythematosus history female patient, that goes to the emergency room with sudden left leg pain, with cyanosis and absence of distal pulses. Besides she presented with lower respiratory symptoms. Diagnosis was confirmed with ultrasound and CT pulmonary angiography was performed showing pulmonary embolism. Medical treatment was initiated with good response.