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HLH as defined by the HLH-04 criteria is a descriptive diagnosis. Its individual components are non-specific. [citation needed] The onset of HLH occurs before the age of one year in approximately 70 percent of cases. Familial HLH should be suspected if siblings are diagnosed with HLH or if symptoms recur when therapy has been stopped.
Diagnosis is confirmed histologically by tissue biopsy. Hematoxylin-eosin stain of biopsy slide will show features of Langerhans Cell e.g. distinct cell margin, pink granular cytoplasm. [34] Presence of Birbeck granules on electron microscopy and immuno-cytochemical features e. g. CD1 positivity are more specific. Initially routine blood tests ...
Any of these conditions that occurs in a family can indicate a diagnosis of Birt–Hogg–Dubé syndrome, though it is only confirmed by a genetic test for a mutation in the FLCN gene, which codes for the protein folliculin. Though its function is not fully understood, it appears to be a tumor suppressor gene that restricts cell growth and ...
Micrograph showing hemophagocytosis in the spleen. H&E stain.. Hemophagocytosis is a dangerous form of phagocytosis in which histiocytes engulf red blood cells, white blood cells, platelets, and their precursors [1] in bone marrow and other tissues.
X-linked lymphoproliferative disease; Other names: Duncan disease, Purtilo syndrome: Specialty: Hematology : Symptoms: Reduced resistance to the Epstein-Barr virus (EBV), leading to infectious mononucleosis, hemophagocytic lymphohistiocytosis (HLH), dysgammaglobulinemia, non-Hodgkin lymphoma, aplastic anemia, vasculitis, chronic gastritis, skin lesions
Erdheim–Chester disease (ECD) is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis).
According to the National Library of Medicine, the mortality rate for those diagnosed with HLH is 40%. Outzen was primarily a backup in his four seasons at Florida State but was thrust into a ...
In 1987, the Histiocyte Society published their classification of the histiocyte disorders together with criteria for diagnosis and clinical assessment of Langerhans cell histiocytosis. [10] Hand–Schüller–Christian disease is now considered a subtype of LCH, chronic multifocal Langerhans cell histiocytosis.