What is hlh in adults




















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Your comment will be reviewed and published at the journal's discretion. Please check for further notifications by email. Volume , Issue Previous Article Next Article. View Metrics. Nationwide survey of hemophagocytic lymphohistiocytosis in Japan. Int J Hematol ; Hepatic manifestations of hemophagocytic syndrome: a study of 30 cases.

Am J Gastroenterol ; Hemophagocytic syndrome in renal transplant recipients: report of 17 cases and review of literature. Transplantation ; Nephrotic syndrome associated with hemophagocytic syndrome.

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J Pediatr Hematol Oncol ; Flow cytometric findings in hemophagocytic lymphohistiocytosis. Am J Clin Pathol ; Hemophagocytic lymphohist-iocytosis in syntaxindeficient mice: T-cell exhaustion limits fatal disease. Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management.

Clin Exp Immunol ; Br J Haematol ; STX11 mutations and clinical phenotypes of familial hemophagocytic lymphohistiocytosis in North America.

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Henter JI. Biology and treatment of familial hemophagocytic lymphohistiocytosis: importance of perforin in lymphocyte-mediated cytotoxicity and triggering of apoptosis. Med Pediatr Oncol ; Munc is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis FHL3.

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Leuk Lymphoma ; Acquired hemophagocytic lymphohistiocytosis associated with multiple myeloma. Med Oncol ; Hemophagocytic lymphohistiocytosis in chronic lymphocytic leukemia. J Clin Oncol ;e Successful resolution of acute myelogenous leukemia-associated hemophagocytic lymphohistiocytosis with decitabine. Perforin gene mutations in adult-onset hemophagocytic lymphohistiocytosis.

Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistio-cytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party. Eur J Haematol ; Gene expression profiling of peripheral blood mononuclear cells from children with active hemophagocytic lymphohistiocytosis. Blood ;e Gene-expression signatures differ between different clinical forms of familial hemophagocytic lymphohistiocytosis.

HLH diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pathology of the liver in familial hemophagocytic lymphohistiocytosis.

Am J Surg Pathol ; Establishment of a reference interval for natural killer cell activity through flow cytometry and its clinical application in the diagnosis of hemophagocytic lymphohistiocytosis.

Int J Lab Hematol ; Soluble interleukin-2 receptor: a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis. Highly elevated ferritin levels and the diagnosis of hemophagocytic lympho-histiocytosis. Marked hyper-ferritinemia does not predict for HLH in the adult population. Hemophagocytic lymphohistiocytosis: a potentially underrecognized association with systemic in-flammatory response syndrome, severe sepsis, and septic shock in adults.

Chest ; Cloak and dagger: the case for adult onset still disease and hemophagocytic lymphohistiocytosis. Rheumatol Int ; The role of hemo-phagocytosis in bone marrow aspirates in the diagnosis of hemophagocytic lymphohistiocytosis. Imashuku S. Advances in the management of hemophagocytic lymphohistiocytosis. Treatment of hemophagocytic lymphohistiocytosis with HLH immuno-chemotherapy and bone marrow transplantation. Filipovich AH. Hemophagocytic lymphohistiocytosis HLH and related disorders.

Hemophagocytic lymphohistiocytosis. Report of children from the International Registry. Leukemia ; Successful correction of hemophagocytic lymphohistiocytosis with related or unrelated bone marrow transplantation. But in adults, many conditions can cause HLH. This is called acquired HLH. It can be caused by some infections, cancer, and autoimmune diseases. If you have acquired HLH, your body's immune system does not work normally.

Some white blood cells called histiocytes and lymphocytes attack your other blood cells. Abnormal blood cells then build up in your spleen and liver. This causes your spleen and liver to enlarge.

HLH is a life-threatening condition. It can cause death in weeks or months if not treated. It may also cause death in months despite treatment. HLH is a rare disease, and researchers are still learning what causes it. There are 2 types of HLH: familial and acquired.

Familial HLH is passed from parents to children. Acquired HLH in adults can be caused by:. Nervous system problems such as headaches, trouble walking, vision disturbances, and weakness. The symptoms of acquired HLH can look like other health conditions. See your healthcare provider for a diagnosis.

Familial-type HLH is usually fatal if not treated. Treatment for familial or persistent acquired HLH may include:. If drug treatments do not work, your healthcare providers may do a stem cell transplant.

In this procedure, healthy bone marrow cells from a donor replace your diseased bone marrow cells. Stem cell transplant can cure HLH in most cases. There is no way to prevent HLH, but as healthcare providers continue to learn more about it, treatment improves. Most children who are successfully treated go on to live normal lives. Healthcare providers do not regularly do genetic testing for HLH on newborns, because the disease is very rare.

Experts recommend genetic testing for these infants.



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