Chediak–Higashi Syndrome Presented With Hemophagocytic Lymphohistiocytosis
نویسندگان
چکیده
منابع مشابه
A Case of Chediak-Higashi Syndrome Presented with Hemophagocytic Lymphohistiocytosis
Chediak Higashi syndrome, is a rare autosomal recessive disorder characterised by oculocutaneus albinism, recurrent respiratory system infections and other pyogenic infections. Hemophagocytic lymphohistiocytosis can develop in any time of the life in patients with Chediak Higashi syndrome. A 14-month-old girl patient was diagnosed as hemophagocytic lymphohistiocytosis with the laboratory findin...
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A 12-month-old female infant was referred to our hospital with prolonged fever and status epilepticus. Her weight and height were below the 5th percentile for age. Physical examination revealed marked hypotonia, fever, pallor, partial albinism with silvery gray hair, and hepatosplenomegaly (Figure 1A). Laboratory investigations showed anemia, thrombocytopenia, hypofibrinogenemia, hyperferritine...
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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...
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Background Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease in which an exaggerated but ineffective immune response leads to severe hyper inflammation. It commonly affects infants from birth to 18 months of age, but cases in older children and adults have also been reported. Key players in HLH are activated lymphocytes and histiocytes, they infiltrate all organs and secret...
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Hemophagocytic lymphohistiocytosis (HLH) is the result of excessive cytokine release, leading to over-response by immune cells, such as macrophages and T lymphocytes. Here, we report a lethal case of HLH with a complete clinical course. The patient was a 45-year-old man with fever and chills since two months ago plus splenomegaly, hepatomegaly, and pancytopenia. The Anti-HBc IgM was positive, b...
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ژورنال
عنوان ژورنال: Indian Journal of Medical and Paediatric Oncology
سال: 2018
ISSN: 0971-5851,0975-2129
DOI: 10.4103/ijmpo.ijmpo_54_16