Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone. We performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared... |
We present a case of tuberculosis-associated hemophagocytic lymphohistiocytosis in a 14-year-old girl. The patient presented with weight loss, malaise, fatigue, prolonged fever, and generalized lymphadenopathy. Laboratory investigation revealed pancytopenia (white blood cells, 2,020 cells/µL; hemoglobin, 10.2 g/dL; platelets, 52,000 cells/µL), hypertriglyceridemia (229 mg/dL), and hyperferritinemia (1,420 ng/mL). Bone marrow biopsy showed a hypocellular bone marrow with a large numbers of... |
Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, splenomegaly, jaundice, and pathologic findings of hemophagocytosis in bone marrow or other tissues such as the lymph nodes and liver. Pleocytosis, or the presence of elevated protein levels in cerebrospinal fluid, could be helpful in diagnosing HLH. However, the pathologic diagnosis of the brain is not included in the diagnostic criteria for this... |
Griscelli syndrome type 2 (GS2) is a rare autosomal recessive disease caused by mutations in the |
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with central nervous system (CNS) involvement is usually fatal unless stem cell transplant (SCT) is offered. However, SCT with conventional intensity conditioning is associated with high transplant-related mortality. We describe our experience with unrelated SCTs after reduced-intensity conditioning (RIC) for patients with EBV-HLH with progressive CNS disease. This approach was associated with minimal toxicities and... |
Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from... |
Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and... |
The recent advances in the basic hematology and immunology have significantly enhanced the understanding of histiocytic disorders. The Histiocyte Society which was established in 1985 enabled the randomized trials for these diseases, and important knowledge regarding pathogenesis, clinical presentation, diagnosis, therapy and late consequences has been obtained. The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over last decades,... |
Hemophagocytic lymphohistiocytosis is a reactive disorder characterized by a generalized non-malignant histiocytic proliferation with prominent hemophagocytosis by stimulated histiocytes in the bone marrow and reticuloendothelial systems resulting in pancytopenia and liver dysfunction. Several diseases including infection, malignancy and autoimmune disease are known to be causative disorders. This case demonstrated histiocytic hemophagocytosis in the bone marrow, resulting in pancytopenia during treatment... |
Seconnday aneurysmal bone cyst is the disease that occurrs in the primary bone tumor and/or tumorus conditions Meticulous search is needed to make a correct diagnosis which dose not overlook the primary diseases. We have experinced an osteolytic lesion on the 7-year-old boy on his skull bone. Plain radiography of the skull revealed osteolytic lesion in the parieto-occipital bone. On... |
Congenital self-healing reticulohistiocytosis(CSHR) is a rare variant of cutaneous reticulohistiocytosis. We report a newborn who had about 25 reddish brown papulonodular lesions on whole body at birth. No extracutaneous involvement was found. All skin lesions involuted within 4 months spontaneously. Biopsy of a skin lesion showed marked aggregated histiocytic infiltration with some eosinophils. S-100 protein and lysozyme were positive. Electron... |
Familial erythrophagocytic lymphohistiocytosis(FEL) is an uncommon disorder characterized by multi-organ infiltration with phagocytic histiocytes and macrophages. It is a familial discorder presenting during infancy or young childhood with fever, hepartosplenomegaly, pancytopenia, bleeding diathesis, hypertriglyceridemia and neurologic manifestations. The course of the disease is extremely lethal and diagnosis of the disease during lifetime is very difficult. Exact diagnosis can only be... |
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare disease characterized by fever, hepatosplenomegaly, cytopenia and non-malignant lymphohistiocytic infiltration with hemophagocytosis in reticuloendothelial organs. We experienced three cases of FHL in identical male twins and their younger brother who presented with fever and severe hepatosplenomegaly. Cytompenia, elevated serum transaminase and low serum albumin levels, hypertriglyceridemia were common laboratory findings of them. One of... |
We made a clinical study on 10 cases of histiocytosis syndrome who had been admitted to the pediatric department of Soon Chun Hyang University Hospital from Jan 1982 to Dec. 1991. The results were obtained as follows 1) The sex incidence revealed male predominance with the ratio 4:1. 2) Among 10 cases, 4 cases were classified as eosinophilic granuloma, 1 case as Letterer-Siwe... |
We made a clinical study on 31 cases of histiocytosis X who had been admitted to the Dept, of Ped. of S.N.U.C.H. from Jan. 1981 to Dec. 1987. Among 31 cases, 28 patients received chemotherapy to include prednisolone, vinblastine, methotrex- ate and cyclophosphamide as induction therapy, and 6-mercaptopurine, methotrexate and cyclophos- phamide as maintenance therapy. The results obtained were as follows. 1) The sex incidence revealed male... |
A clinical study with particular emphasis on the result of chemotherapy has been made on. 34 cases of histiocytosis diagnosed at the Department of the Severance Hospital and Yonsei Cancer Center from January 1975 to December 1981. The results may be summarized as follows: 1) Among histiocytosis, HSC and LSD were each present in elevent cases, E-G in seven and HMR... |
A case of histiocytosis X (Hand-Schuller-Christian syndrome) in a 3 year old girl is presented. The patients has the triad of exophthalmos, diabetes insipidus and skeletal lesions, plus hepatomegaly, wide spread pulmonary infiltration and growth retardation. The patient was treated with chlorambucil, prednisone, radiation therapy and chlorpropamide therapy for diabetes insipidus with good response. Similar cases reported in literature are briefly... |
We experienced a cass of Letterer-Siwe disease in 2 month old infant. The patient was presented charateristic clinical findings such as hepatosplenomegaly, petechia, anemia, gene-ralized lymphadenopathy, and lung infiltration. The diagnosis was confirmed by lymphnode biopsy and bone marrow aspiration, which was compatible to Letterer-Siwe disease. A poor prognostic factor in Histiocytosis X is younger age (below 18 month), hepatospleno-megaly, anemia,... |