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Original Article
Cardiology
Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide
Jung Eun Choi, Yujin Kwak, Jung Won Huh, Eun-Sun Yoo, Kyung-Ha Ryu, Sejung Sohn, Young Mi Hong
Clin Exp Pediatr. 2018;61(5):167-173.   Published online May 28, 2018
Purpose

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.

Methods

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...

Case Report
Infection
Tuberculosis-associated hemophagocytic lymphohistiocytosis in adolescent diagnosed by polymerase chain reaction
Ju-Hee Seo, Jun Ah Lee, Dong Ho Kim, Joongbum Cho, Jung Sub Lim
Clin Exp Pediatr. 2016;59(1):43-46.   Published online January 22, 2016

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 diagnosed by brain biopsy
Hee Young Ju, Che Ry Hong, Sung Jin Kim, Ji Won Lee, Hyery Kim, Hyoung Jin Kang, Kyung Duk Park, Hee Young Shin, Jong-Hee Chae, Ji Hoon Phi, Jung-Eun Cheon, Sung-Hye Park, Hyo Seop Ahn
Clin Exp Pediatr. 2015;58(9):358-361.   Published online September 21, 2015

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: a novel mutation in RAB27A gene with different clinical features in 2 siblings: a diagnostic conundrum
Kirtisudha Mishra, Shilpy Singla, Suvasini Sharma, Renu Saxena, Vineeta Vijay Batra
Clin Exp Pediatr. 2014;57(2):91-95.   Published online February 24, 2014

Griscelli syndrome type 2 (GS2) is a rare autosomal recessive disease caused by mutations in the RAB27A gene. It is characterized by cutaneous hypopigmentation, immunodeficiency, and hemophagocytic lymphohistiocytosis. We describe 2 brothers who had GS2 with clinically diverse manifestations. The elder brother presented with a purely neurological picture, whereas the younger one presented with fever, pancytopenia, hepatosplenomegaly, and erythema nodosum....

Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement
Hee Jo Baek, Hoon Kook, Dong Kyun Han, Min-Cheol Lee, Tae Woong Jeong, Tai Ju Hwang
Clin Exp Pediatr. 2009;52(6):725-729.   Published online June 15, 2009
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...
Original Article
Clinical characteristics and prognostic factors for survival in hemophagocytic lymphohistiocytosis
Khi Joo Kim, Ki Hwan Kim, Taek Jin Lee, Jin-Kyong Chun, Chuhl Joo Lyu, Dong Soo Kim
Clin Exp Pediatr. 2008;51(3):299-306.   Published online March 15, 2008
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...
Review Article
Recent advances in histiocytic disorders
Jong Jin Seo
Clin Exp Pediatr. 2007;50(6):524-530.   Published online June 15, 2007
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,...
Case Report
A Case of Hemophagocytic Lymphohistiocytosis in a Child with Systemic Lupus Erythematosus
Ja Young Hwang, Suk Man No, Jin Lee, Pil Sang Jang, Young Hoon Kim, Jin Tack Kim, Joon Sung Lee
Clin Exp Pediatr. 2003;46(10):1029-1031.   Published online October 15, 2003
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...
Familial Hemophagocytic Lymphohistiocytosis
Dong Un Kim, Dae Kyun Koh, Yeon Dong Lee, Jae Kyun Hur, Kyoo Hong Cho, Suk Jin Kang
Clin Exp Pediatr. 1994;37(9):1279-1285.   Published online September 15, 1994
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...