Hemophagocytic lymphohistiocytosis as severe adverse event of antineoplastic treatment in children.

نویسندگان

  • Herwig Lackner
  • Christian Urban
  • Petra Sovinz
  • Martin Benesch
  • Andrea Moser
  • Wolfgang Schwinger
چکیده

Hemophagocytic lymphohistiocytosis (HLH) during childhood cancer treatment is a rare adverse event posing major diagnostic and therapeutic challenges. Between 1995 and 2006, 6 children developed HLH while on conventional chemotherapy (n=4) or after allogeneic stem cell transplantation (n=2). Treatment of HLH included dexamethasone and etoposide, 2 children additionally received infliximab or daclizumab. Three children survived, whereas 3 children died 2, 5, and 47 days after diagnosis of HLH. HLH is a severe adverse event of childhood cancer therapy. Early diagnosis and immediate initiation of adequate treatment are mandatory to overcome this severe condition.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Successful Treatment of Scrub Typhus - Associated

Scrub typhus is caused by Orientia tsutsugamushi. Any delay in diagnosis can result in delayed treatment and severe complications, including secondary hemophagocytic lymphohistiocytosis, which is rare but potentially fatal. In this paper, the authors present 3 cases of secondary hemophagocytic lymphohistiocytosis associated with scrub typhus, successfully treated with chloramphenicol without ad...

متن کامل

Biology and Treatment of Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistocytosis (HLH) is a hyperinflammatory syndrome that occurs at all ages and is characterized by high levels of cytokines, secreted by activated T-lymphocytes and macrophages. All symptoms and laboratory changes can be explained by organ infiltration by these cells and hypercytokinemia. HLH occurs as an inherited form (genetic, primary HLH) with mutations primarily in the...

متن کامل

Etoposide in patients with rheuma-associated hemophagocytic lymphohistiocytosis / macrophage activation syndrome

Background/aim Rheuma-associated hemophagocytic lymphohistiocytosis (Rh-HLH), also called macrophage activation syndrome (MAS), is a severe complication of systemic inflammatory disorders. Rh-HLH has clinical and laboratory similarities to other forms of HLH, and is potentially life threatening. Treatment of Rh-HLH has not been standardized yet, but it commonly includes a variety of agents such...

متن کامل

Autoimmune Lymphoproliferative Syndrome Misdiagnosed as Hemophagocytic Lymphohistiocytosis A Case Report

Autoimmune lymphoproliferative Syndrome (ALPS) is a rare inherited disorder of apoptosis. It usually presents with chronic lymphadenopathy, splenomegaly, and symptomatic cytopenia in a child. Herein, we report a 14-year-old boy with symptoms misdiagnosed as hemophagocytic lymphohistiocytosis who was treated before ALPS was diagnosed for the patient. This case should alert pediatricians to consi...

متن کامل

Perforin Gene Analaysis in an Iranian Family with Familial Hemophagocytic Lymphohistiocytosis

Perforin gene (PRF1) mutations have been reported in 20-30% of patients with familial hemophagocytic lymphohistiocytosis (FHL), an immune disorder of infancy and early childhood. Cytotoxic T and natural killer (NK) cell activities are remarkably reduced or ab-sent in FHL patients. We report the first cases of familial hemophagocytic lymphohistiocy-tosis in an Iranian family with two siblings. E...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • Haematologica

دوره 93 2  شماره 

صفحات  -

تاریخ انتشار 2008