C0281 Heparin-induced thrombocytopenia (HIT) type 2 caused by preventive anticoagulation of the circuit of hemodialysis (HD): A case report

Abstract

and production of high amounts of pro-inflammatory cytokines. It's a rare and serious clinicobiological entity: a child on a million children is affected. The occurrence of a disseminated intra-vascular coagulation syndrome DIVC during a flare-up of HS testifies of the severity and the complexity of this syndrome. Methods: Observation: 23 years old woman in the family history of FHS at the origin of two deaths in the sibling is hospitalized for involvement of DIVC during a flare-up of HS. This patient is followed in pediatrics ‘center since the age of 09 years for FHS and report about 3 to 4 annual increases, activated usually by viral infections (EBV, CMV) justifying hospitalizations and antiviral therapeutics associated with a corticosteroid therapy. We observed a altered general health, a fever in 40 °C, a rash skin, a diffuse purpura, an acute diarrhea and a type 5 splenomegaly. The neurological examination brings to light amyotrophic of the left foot with a steppage gait in thewalking. The PA is estimated at 80/60 mmHg and the BMI at 17. There is nomeningeal irritation and the cardiopulmonary auscultation is normal except a regular tachycardia. Results: The biological check-up shows a deep pancytopenia, abnormal hepatic tests, hypofibrinemia, elevation of serum LDH, ferritinemia and triglyceride levels, an inflammatory syndrome, and signs of DIVC (increased D Dimers and decrease of prothrombine index..). The viral serology of HIV, hepatitis (HBS, HCV), CMV and EBV are negative. Hemoculturs and coproparasitology are also negative. A biopsy bone with immunohistochemical study oriented to spleeny lymphoma T. The symptomatic treatment undertaken in the emergency (antibiotic therapy, antiviral drugs, a corticosteroid therapy, and transfusions of globular sediments and of plasma) is followed by a favorable evolution. The patient is oriented hematology for specialized care. Comment: Conclusion: Recent advances, essentially due to genetic studies of FHS, have underlined the major role of T lymphocytes and TNFα in the pathogenesis of hemophagocytosis realized a real cytokine thunderstorm. The viral infections and the neoplastic process established the complex initiator of the involvement of the acute phase of the HS. In pediatric cases, prognosis has dramatically improved since allogenic bone marrow transplantation.