• 대한두경부종양학회지
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• 제36권2호
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• pp.55-59
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• 2020

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening one syndrome of excessive immune activation. This immune dysregulation disorder is prominently associated with cytopenias and combinations of clinical signs and extreme inflammation symptoms. For survival, it is important to diagnose early and treat appropriately. We report a case of 10 years old boy who was admitted to the hospital with a month history of fever and cervical lymph node enlargement. There were signs of hemophagocytic histiocytosis in the lymph node and bone marrow. The etiology, diagnosis, and treatment of hemophagocytic lymphohistiocytosis are reviewed.

• Clinical and Experimental Pediatrics
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• 제59권1호
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• pp.43-46
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• 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, $2,020cells/{\mu}L$; hemoglobin, 10.2 g/dL; platelets, $52,000cells/{\mu}L$), hypertriglyceridemia (229 mg/dL), and hyperferritinemia (1,420 ng/mL). Bone marrow biopsy showed a hypocellular bone marrow with a large numbers of histiocytes and marked hemophagocytosis; based on these findings, she was diagnosed with hemophagocytic lymphohistiocytosis. Polymerase chain reaction (PCR) with both the bone marrow aspiration and sputum samples revealed the presence of Mycobacterium tuberculosis. Antitubercular therapy with immune modulation therapy including dexamethasone and intravenous immunoglobulin was initiated. The results of all laboratory tests including bone marrow biopsy and PCR with both the bone marrow aspiration and sputum samples were normalized after treatment. Thus, early bone marrow biopsy and the use of techniques such as PCR can avoid delays in diagnosis and improve the survival rates of patients with tuberculosis-associated hemophagocytic lymphohistiocytosis.

• Journal of Yeungnam Medical Science
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• 제38권3호
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• pp.245-250
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• 2021

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome-HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m²/day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.

• Journal of Korean Medical Science
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• 제33권50호
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• pp.319.1-319.5
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• 2018

The incidence of severe fever with thrombocytopenia syndrome (SFTS) has increased in Korea since a first report in 2013. We investigated whether SFTS existed before 2013 using real-time reverse transcription polymerase chain reaction and stored blood samples from febrile patients with thrombocytopenia. Four cases of SFTS were identified, with the earliest occurring in 2008.

• Clinical and Experimental Pediatrics
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• 제46권10호
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• pp.1029-1031
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• 2003

본 저자들은 전신성 홍반성 루푸스와 미만성 증식성 사구체신염으로 진단된 남아의 치료과정 중 발열과 혈구 감소증이 지속된 환아에서 이차성 혈구 탐식성 조직구 증식증을 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Pediatric Infection and Vaccine
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• 제31권1호
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• pp.122-129
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• 2024

Chediak-Higashi syndrome (CHS) is a rare haematological and immunodeficiency disorder that occurs in childhood leading to recurrent infections, bleeding tendencies and progressive neurological dysfunction. Partial oculocutaneous albinism occurs in almost all cases. The exact prevalence is unknown, and the disease is caused by over 70 identified mutations in the lysosomal trafficking regulator gene. The presence of a bright polychromatic appearance from hair shaft and abnormally large intracytoplasmic granules, especially within neutrophils and platelets in the bone marrow is highly suggestive. Treatment is largely supportive, and the only curative treatment is through an allogeneic hematopoietic stem cell transplant. Without transplant, most patients will enter an accelerated phase of hemophagocytic lymphohistiocytosis (HLH) which carries a high mortality rate. We present a young male with CHS who we had followed through and eventually developed a fulminant accelerated phase. We believe this is only the second reported case of CHS in Malaysia.

• Clinical and Experimental Pediatrics
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• 제52권6호
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• pp.725-729
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• 2009

중추신경계의 침범을 동반한 Epstein-Barr 바이러스 관련 혈구포식 림프조직구증은 조혈모세포이식을 시행하지 않으면 예후가 좋지 않다. 또한, 고식적인 강도의 전처치시 이식관련 사망률이 높다. 따라서 저자들은 진행성 중추신경계 침범을 보인 Epstein-Barr 바이러스 관련 혈구포식 림프조직구증 2례에서 저강도 전처치와 rituximab 후 타인 조혈모세포이식을 시행하여 치료하였기에 이를 보고하고자 한다.

• Clinical and Experimental Pediatrics
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• 제58권9호
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• pp.358-361
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• 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 condition. In the present report, we describe the case of a patient diagnosed with HLH, in whom the brain pathology, but not the bone marrow pathology, showed hemophagocytosis. As the diagnosis of HLH is difficult in many cases, a high level of suspicion is required. Moreover, the pathologic diagnosis of organs other than the bone marrow, liver, and lymph nodes may be a useful alternative.

• Journal of Yeungnam Medical Science
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• 제31권2호
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• pp.75-81
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• 2014

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease. Despite of proper treatment and improving treatment regimens, HLH patients still show a fatal prognosis. Therefore the evaluation of prognostic factor is important and there are many studies about hyperbilirubinemia as a prognostic factor in HLH. So we studied the prognostic value of hyperbilirubinemia in HLH children. Methods: A retrospective analysis was performed about 33 patients who were diagnosed with HLH at Pusan National University Hospital and Yangsan Pusan University Hospital between January 2000 to December 2012. We reviewed the clinical characteristics, laboratory findings, and results of treatment to identify hyper-bilirubinemia as a prognostic factor in HLH patients. Results: The median age of patients at diagnosis was 32 months. Most of patients presented with fever, pale appearance, abdominal pain and jaundice. Forty-eight point five percentage of patients showed normal serum bilirubiln level (<2.0 mg/dL) and 51.5% showed hyperbilirubinemia (${\geq}2.0mg/dL$). In normal serum bilirubin group, 1 patient (6.3%) was relapsed and 1 patient (5.9%) was relapsed in hyperbilirubinemia group. In the hyperbilirubinemia group, the mortality was higher than the normal bilirubin group but, there was no statistical significance. Conclusion: As a prognostic factor serum bilirubin at diagnosis in HLH patients, there was no significant correlation between hyperbilirubinemia and poor outcome. But, our study has a limitation that the number of patients is too small and almost showed good prognosis.

• The Korean Journal of Medicine
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• 제91권1호
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• pp.88-91
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• 2016

혈구포식 림프조직구증식증(HLH)은 대식세포와 림프구의 증식과 활성화가 조절되지 않아 발생하게 되는 드문 과염증 증후군으로, 치명적인 예후를 초래하는 경우가 많다. 저자들은 기저 질환이 없는 환자에서 B형 인플루엔자 감염과 연관되어 발생한 혈구포식 림프조직구증식증으로 인하여 사망한 증례를 경험하여 이를 보고하는 바이다.