Influenza infection may be complicated by various infectious or non-infectious diseases. Among them, hemophagocytic lympho-histiocytosis (HLH) is an uncommon hyperinflammatory syndrome caused by uncontrolled proliferation and activation of macrophages and lymphocytes, and it is often life threatening. A previously healthy male patient was suspected to have HLH after influenza B infection. The diagnosis was established based on clinical diagnostic criteria suggested in the HLH-2004 trial. Despite prompt antiviral therapy, the patient expired on day 19 of hospitalization. Influenza can thus be complicated by HLH. Due to the non-specific manifestations of HLH, clinical suspicion and early diagnosis are important.
Diagnosis ; Early Diagnosis ; Hospitalization ; Humans ; Influenza, Human* ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic* ; Macrophages ; Male

Influenza infection may be complicated by various infectious or non-infectious diseases. Among them, hemophagocytic lympho-histiocytosis (HLH) is an uncommon hyperinflammatory syndrome caused by uncontrolled proliferation and activation of macrophages and lymphocytes, and it is often life threatening. A previously healthy male patient was suspected to have HLH after influenza B infection. The diagnosis was established based on clinical diagnostic criteria suggested in the HLH-2004 trial. Despite prompt antiviral therapy, the patient expired on day 19 of hospitalization. Influenza can thus be complicated by HLH. Due to the non-specific manifestations of HLH, clinical suspicion and early diagnosis are important.
Diagnosis ; Early Diagnosis ; Hospitalization ; Humans ; Influenza, Human ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic ; Macrophages ; Male

Kikuchi's disease and hemophagocytic syndrome have the clinical similarity in initial presentations, but present different clinical course and prognosis. Kikuchi's disease usually have a self-limiting clinical course, but hemophagocytic syndrome can be fatal. It is important to make differential diagnoses and treat properly. We report a case of 18 year old boy who was admitted to the hospital with two weeks history of fever and progressive cervical lymph node swelling. Cervical lymph node biopsy showed the typical necrotizing lymphadenitis of Kikuchi's disease. There were signs of histiocytosis and hemophagocytic phenomenon in the bone marrow. The etiology, treatment, and prognosis of Kikuchi's disease with hemophagocytic syndrome are discussed.
Biopsy ; Bone Marrow ; Diagnosis, Differential ; Fever ; Histiocytic Necrotizing Lymphadenitis ; Histiocytosis ; Lymph Nodes ; Lymphadenitis ; Lymphohistiocytosis, Hemophagocytic ; Prognosis

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disease caused by dysregulated immune responses and overwhelming inflammation to infectious or other triggers of the immune system. HLH may be inherited (primary) or may be secondary to any severe infection, malignancy or rheumatologic disease. Clinical progress of HLH is usually rapid and fatal. Early recognition and assessment of potential causes of HLH is critical to improve survival. Urgent treatment is needed for immunosuppression and degradation of the activated antigen. Over the last two decades, research on genetics and pathophysiology of HLH has much improved treatment outcome of the patient. Here, we review recent advances in our understanding of etiology, pathophysiology, diagnosis and treatment of HLH.
Diagnosis ; Genetics ; Humans ; Immune System ; Immunosuppression ; Inflammation ; Lymphohistiocytosis, Hemophagocytic* ; Treatment Outcome

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 hemophagocytic syndrome is a disorder caused by systemic proliferation of benign histiocytes with avid phagocytosis of blood cells. Although this is an established disease entity, early clinical diagnosis is often difficult. The disease is known to be rare. The clinical and laboratory characteristics of 33 patients with the infection-associated hemophagocytic syndrome were reviewed which included previously reported 31 cases from the literature, and 2 recent cases presented in this study. The patients were 22 males and 11 females with a mean age of 20.8 years(range 1 to 69 years). Ten patients were associated with viral infection and seven with bacterial infection. The infection usually occurs in patients with preexisting immunological abnormalities, but in this study underlying illness was found in only 8 out of the 33 cases. The overall mortality rate was 63.6%, which is a higher percentage than in other countries. Since the clinical course can be fulminant, accurate diagnosis and effective treatment are needed.
Bacterial Infections ; Blood Cells ; Diagnosis ; Female ; Histiocytes ; Humans ; Lymphohistiocytosis, Hemophagocytic* ; Male ; Mortality ; Phagocytosis

Virus associated hemophagocytic syndrome, class ll histiocytoses, characterized by high fever, severe constitutional symptoms, abnormal liver function and coagulation, perigheral blood pancytopenia and histiocytic hyperplasis with prominent hemophagocytosis in bone marrow and lymph nodes has been reported and associated with active viral infection. It is non-malignant and reversible. It must be differentiated from histiocytic medullary reticulosis because of the inappopriateness of immunosuppressive of cytotozic therapy which is the therapeutic method for HMR, but is contraindicated in the treatment of VAHS. This paper describes two patients whose clinicopathology was compatible with the diagnosis of virus associated hemophagocytic syndrome.
Bone Marrow ; Diagnosis ; Fever ; Histiocytosis ; Humans ; Liver ; Lymph Nodes ; Lymphohistiocytosis, Hemophagocytic ; Pancytopenia

Virus associated hemophagocytic syndrome, class ll histiocytoses, characterized by high fever, severe constitutional symptoms, abnormal liver function and coagulation, perigheral blood pancytopenia and histiocytic hyperplasis with prominent hemophagocytosis in bone marrow and lymph nodes has been reported and associated with active viral infection. It is non-malignant and reversible. It must be differentiated from histiocytic medullary reticulosis because of the inappopriateness of immunosuppressive of cytotozic therapy which is the therapeutic method for HMR, but is contraindicated in the treatment of VAHS. This paper describes two patients whose clinicopathology was compatible with the diagnosis of virus associated hemophagocytic syndrome.
Bone Marrow ; Diagnosis ; Fever ; Histiocytosis ; Humans ; Liver ; Lymph Nodes ; Lymphohistiocytosis, Hemophagocytic ; Pancytopenia

Hemophagocytic lymphohistiocytosis (HLH) has been described in patients with advanced stages of human immunodeficiency virus (HIV) infection, but rarely occurs during the seroconversion stage of acute HIV infection. We report a case of acute HIV syndrome that presented with virus-associated HLH. The patient recovered spontaneously without any immunomodulating therapy. This case suggests that acute HIV infection should be included in the differential diagnosis of HLH and indicates that HLH associated with acute HIV infection can have a favorable outcome.
Acquired Immunodeficiency Syndrome/complications/*diagnosis ; Adult ; Diagnosis, Differential ; HIV Infections/complications/*diagnosis ; Humans ; Korea ; Lymphohistiocytosis, Hemophagocytic/etiology/*pathology ; Male

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory clinical syndrome of uncontrolled immune response which results in hypercytokinemia due to underlying primary or secondary immune defect. A number of genetic defects in transport, processing and function of cytotoxic granules which result in defective granule exocytosis and cytotoxicity of cytotoxic T lymphocytes (CTL) and natural killer (NK) cells have been well identified at the cellular and molecular level. Important advances have been made during the last 20 years in the diagnosis and treatment of HLH. The Histiocyte Society has proposed diagnostic guideline using both clinical and laboratory findings in HLH-2004 protocol, and this has been modified partly in 2009. HLH used to be a fatal disease, but the survival of HLH patients has improved to more than 60% with the use of chemoimmunotherapy combined with hematopoietic cell transplantation (HCT) over the past 2 decades. However, HCT is still the only curative option of treatment for primary HLH and refractory/relapsed HLH after proper chemoimmunotherapy. The outcome of HCT for HLH patients was also improved steadily during last decades, but HCT for HLH still carries significant mortality and morbidity. Moreover, there remain ongoing controversies in various aspects of HCT including indication of HCT, donor selection, timing of HCT, conditioning regimen, and mixed chimerism after HCT. This review summarized the important practical issues which were proven by previous studies on HCT for HLH, and tried to delineate the controversies among them.
Cell Transplantation* ; Chimerism ; Diagnosis ; Donor Selection ; Exocytosis ; Hematopoietic Stem Cell Transplantation ; Histiocytes ; Humans ; Lymphohistiocytosis, Hemophagocytic* ; Mortality ; T-Lymphocytes, Cytotoxic ; Transplants*