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

Familial erythrophagocytic lymphohistiocytosis is a fatal early childhood disorder characterized by multiorgan lymphohistiocytic infiltration and active hemophagocytosis. Involvement of the central nervous system (CNS) is not uncommon and is characterized by rapidly progressive tissue damage affecting both the gray and white matter. We encountered a case of familial erythrophagocytic lymphohistiocytosis with CNS involvement. Initial T2-weighted MRI of the brain demonstrated high signal intensity in the right thalamus, though after chemotherapy, which led to the relief of neurologic symptoms, this disappeared. After four months, however, the patient's neurologic symptoms recurred, and follow-up T2-weighted MR images showed high signal intensity in the thalami, basal ganglia, and cerebral and cerebellar white matter. Brain MRI is a useful imaging modality for the evaluation of CNS involvement and monitoring the response to treatment.
Basal Ganglia ; Brain* ; Central Nervous System ; Drug Therapy ; Follow-Up Studies ; Lymphohistiocytosis, Hemophagocytic* ; Magnetic Resonance Imaging* ; Neurologic Manifestations ; Thalamus

The study was aimed to investigate the clinical characteristics, diagnosis and therapy of patients with lymphoma associated hemophagocytic syndrome (LAHS) so as to provide the clinical basis for improving its recognition and giving effective therapy. The clinical data of 14 patients with LAHS in Beijing Friendship Hospital, Capital Medical University during the period from June 2005 to May 2008 were collected, the informations including primary diseases, clinical manifestations, laboratory findings, therapy and outcome were analyzed retrospectively, the coincidence of each diagnostic index was compared before and after diagnosis. All 14 patients were given therapeutic regimens containing fludarabine, methylprednisolone and gammaglobulin (FDIg) after final diagnosis. The results indicated that 100% patients had abnormal changes on NK cell activity and sCD25 level in serum, but hemophagocytosis in less than 40% patients at early stage was found in bone marrow. Even after confirmed diagnosis of the disease, the percentage of patients with hemophagocytosis was not up to 50%. 9 out of the 14 patients had a good prognosis after treatment, and the other 5 patients died. It is concluded that the detection of NK cell activity and sCD25 level in serum may be valuable for the early diagnosis of LAHS, the hemophagocytosis is not necessary for the diagnosis of LAHS. Fludarabine combined with methylprednisolone and gamma globulin may provide a new strategy for LAHS therapy.
Humans ; Lymphohistiocytosis, Hemophagocytic ; complications ; diagnosis ; drug therapy ; Lymphoma ; complications ; Prognosis ; Retrospective Studies ; Treatment Outcome ; Vidarabine ; analogs & derivatives ; therapeutic use

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and potentially fatal syndrome that results from inappropriate activation of lymphocytes and macrophages. It is characterized by fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, and pathologic findings of hemo- phagocytosis in the bone marrow or other tissues. We report an adult HLH case admitted to hepatology department.
Humans ; Liver Diseases ; complications ; physiopathology ; Liver Function Tests ; Lymphohistiocytosis, Hemophagocytic ; drug therapy ; etiology ; Male ; Middle Aged ; Prednisone ; therapeutic use

Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening disorder due to hyperinflammation resulting in infiltration of different organs with extensive hemophagocytosis. Severe coagulopathy was one of the main reasons for death in HLH. Over secretion of plasminogen activator by activated macrophages leads to hyperfibrinolysis. We reported a 36-year-old woman who was diagnosed as HLH probably secondary to lymphoma. Massive bleeding from gut and retroperitoneal area were not able to be controlled by conventional hemostatic treatments. This patient received one dose recombinant activated factor VII (rFVIIa) 3.6 mg (70 μg/kg). Hemostatic effect was achieved in 0.5 hour and lasted 24 hours. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were quickly corrected to normal ranges. Fibrinogen level elevated from 0.5 g/L before using rFVIIa to 1.8 g/L 20 hours after. Although dexamethasone and etopside were administrated to treat HLH, this patient died from septic shock after persistent neutropenia. This suggests that rFVIIa may be effective in the management of intractable hemorrhage in patients with HLH.
Adult ; Disseminated Intravascular Coagulation ; complications ; Factor VIIa ; therapeutic use ; Female ; Humans ; Lymphohistiocytosis, Hemophagocytic ; drug therapy ; Recombinant Proteins ; therapeutic use

Child ; Child, Preschool ; Dexamethasone ; therapeutic use ; Female ; Humans ; Infant ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; drug therapy ; genetics ; Male ; Prednisolone ; therapeutic use ; Prognosis

The acquired immunodeficiency syndrome patients with compromised immunity are prone to hemophagocytic syndrome secondary to opportunistic infections.This paper reports a rare case of hemophagocytic syndrome secondary to human parvovirus B19 infection in an acquired immunodeficiency syndrome patient,and analyzes the clinical characteristics,aiming to improve the diagnosis and treatment of the disease and prevent missed diagnosis and misdiagnosis.
Humans ; Lymphohistiocytosis, Hemophagocytic/drug therapy* ; Erythema Infectiosum/complications* ; Acquired Immunodeficiency Syndrome/complications* ; Parvoviridae Infections/diagnosis* ; Parvovirus B19, Human

Adult ; Biomarkers ; blood ; Bone Marrow Transplantation ; Child ; Diagnosis, Differential ; Drug Therapy, Combination ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; drug therapy ; therapy ; Practice Guidelines as Topic