Humans ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; therapy

Hemophagocytic lymphohistiocytosis (HLH), or hemophagocytic syndrome (HPS), is characterized clinically by abrupt onset and progressive deterioration and even death. HLH is much more prevalent in children, and is potentially fatal if early diagnosis is not made and appropriate HLH-directed therapy not instituted. Increasing genetic defects and underlying diseases or causative factors have been identified to be closely implicated in the pathogenesis of HLH. In addition, great advances have been made in the past few years in terms of HLH diagnosis and clinical management. In the present review, the cause of disease, contemporary classification, epidemiology, genetic defects and molecular mechanisms, updated diagnostic criteria and novel treatment strategies for childhood HLH are summarized.
Child ; Humans ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; pathology ; therapy

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by pancytopenia and multiple organ infiltrations of lymphocytes and histiocytes with proliferation and hemohpagocytic activity. HLH is classified as primary (or familial) and secondary. Familial HLH is common in infants and young children, and is related to genetic defects. This article aims to review research advances on PRF1, UNC13D, STX11 and STXBP2, as well as the other 5 genes associated with familial HLH based on molecular genetics, and to summarize diagnosis and treatment methods for this disease.
Humans ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; etiology ; genetics ; therapy ; Molecular Biology

Child, Preschool ; Humans ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; genetics ; therapy ; Male

Humans ; Lymphohistiocytosis, Hemophagocytic/therapy* ; Receptors, Chimeric Antigen ; Immunotherapy, Adoptive

Brain ; Central Nervous System Diseases ; etiology ; therapy ; Child ; China ; Humans ; Lymphohistiocytosis, Hemophagocytic ; complications ; therapy

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease caused by uncontrolled proliferation of activated macrophage, and secreting high amounts of inflammatory cytokines which lead to multi-organ dysfunction syndrome. HLH patients often show different clinical characteristics during the disease was progressed, in which coagulopathy were the most common, including thrombocytopenia and hypofibrinogenemia, those are the major cause of death in patients, and the clinicians should increase awareness of the mechanisms, clinical characteristics, prognosis and treatment. In this review, the above problems are briefly summarized, to deepen understanding of the HLH related coagulation dysfunctions, and early identification and treatment to reduce mortality, so as to provide more opportunities for HLH patients to recieve subsequent treatment.
Afibrinogenemia ; Blood Coagulation Disorders/therapy* ; Humans ; Lymphohistiocytosis, Hemophagocytic/therapy* ; Prognosis ; Thrombocytopenia

The cutaneous manifestations of hemophagocytic lymphohistiocytosis (HLH) are variable and nonspecific. A 42-year-old man presented with multiple annular, erythematous patches on the trunk for 3 months. Two months later, he presented with bullae along with high fever. The laboratory examination showed pancytopenia, hypertriglyceridemia, and hypofibrinogenemia. The bone marrow biopsy specimen showed an active hemophagocytosis. On the basis of these findings, a diagnosis of HLH was concluded. After five cycles of chemotherapy, his skin lesion completely resolved. Taking the results together, we suggest that annular skin lesion can be added to the list of cutaneous manifestations of HLH.
Adult ; Biopsy ; Bone Marrow ; Diagnosis ; Drug Therapy ; Fever ; Humans ; Hypertriglyceridemia ; Lymphohistiocytosis, Hemophagocytic* ; Pancytopenia ; Skin*

The cutaneous manifestations of hemophagocytic lymphohistiocytosis (HLH) are variable and nonspecific. A 42-year-old man presented with multiple annular, erythematous patches on the trunk for 3 months. Two months later, he presented with bullae along with high fever. The laboratory examination showed pancytopenia, hypertriglyceridemia, and hypofibrinogenemia. The bone marrow biopsy specimen showed an active hemophagocytosis. On the basis of these findings, a diagnosis of HLH was concluded. After five cycles of chemotherapy, his skin lesion completely resolved. Taking the results together, we suggest that annular skin lesion can be added to the list of cutaneous manifestations of HLH.
Adult ; Biopsy ; Bone Marrow ; Diagnosis ; Drug Therapy ; Fever ; Humans ; Hypertriglyceridemia ; Lymphohistiocytosis, Hemophagocytic* ; Pancytopenia ; Skin*

Child ; Child, Preschool ; Female ; Humans ; Infant ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; drug therapy ; Male ; Practice Guidelines as Topic ; Prognosis