• 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.

• 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.

• Tuberculosis and Respiratory Diseases
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• 제55권6호
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• pp.636-642
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• 2003

저자들은 기침, 호흡곤란 등의 호흡기 증세와 발열, 체중감소 등의 전신증상이 있으면서 흉부 방사선 소견상 폐렴이 의심되었으나 광범위 항생제에 반응이 없이 빠르게 진행하는 환자에서 개흉 폐생검을 통해 NK/T 세포 림프종을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Pediatric Infection and Vaccine
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• 제3권2호
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• pp.200-206
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• 1996

Virus associated hemophagocytic syndrome(VAHS), a class II histiocytosis syndrome, is characterized by high fever, liver dysfunction, coagulation abnormalities, and generalized histiocytic proliferation with marked hemophagocytosis in bone marrow and lymph nodes. VAHS is associated with several viral infections including Epstein Barr virus which has a relatively high mortality rate. We report a fatal case of Epstein Barr virus associated hemophagocytic syndrome and its diagnosis by mRNA in situ hybridization and polymerase chain reaction. A brief review of related literaure is also presented.

• Clinical and Experimental Pediatrics
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• 제45권5호
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• pp.664-668
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• 2002

저자들은 이에 가와사끼병 및 말단 조직괴저가 동반된 혈구 탐식증 1례를 보고하는 바이며, 가와사끼병 환자가 간비종대, 혈소판 감소를 보이는 경우에는 드물지만 혈구 탐식증의 가능성을 배제할 수 없으므로 확진을 위하여 혈청 중성지방의 측정과 골수 생검을 시행하여야 할 것으로 사료하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제65권6호
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• pp.522-526
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• 2008

결핵과 연관된 혈구 탐식 증후군은 매우 드물며 사망률이 50% 정도로 높은 질환이다. 저자들은 결핵성 심막염 진단 후 2달간 항결핵약을 복용하고 있는 환자에서 지속적인 혈소판 감소증을 보이고 골수검사에서 혈구탐식증을 보였으나 이전에 보고된 증례와는 다르게 경한 임상증상을 보이면서 항결핵약제 및 스테로이드와 etoposide의 병합 항암 치료에 빠른 호전을 보인 예를 경험하였기에 이를 문헌 고찰과 함께 보고하는 바이다.

• 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.

• Pediatric Infection and Vaccine
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• 제17권2호
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• pp.177-181
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• 2010

KD의 급성 열성기에 드물지만 MAS가 발생할 수 있다. 저자들은 19개월 여아의 KD 치료 중에 발생된 MAS를 경험하였다. 환아는 IVIG과 steroid 치료를 받았지만, 발열과 간비종대는 지속되었고 골수생검에서 혈구탐식구증이 관찰되었다. KD에 동반된 MAS를 인지하여 적기에 치료하기 위해서는 임상양상과 치료반응을 토대로, MAS의 가능성을 고려하는 것이 필요하다.

• Clinical and Experimental Pediatrics
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• 제59권10호
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• pp.421-424
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• 2016

Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3. We were unable to identify the cause for the HLH. He remained symptom-free until the development of back pain and right ankle joint pain with swelling at 15 years of age. He was diagnosed with HLA B27-positive AS with bilateral active sacroiliitis. He showed symptom aggravation despite taking naproxen and methotrexate, and the symptoms improved with etanercept. On admission, his laboratory data showed leukopenia with high ferritin and triglyceride levels. Bone marrow biopsy examination showed histiocytic hyperplasia with hemophagocytosis. There was no evidence of infection. He received naproxen alone, and his symptoms and laboratory data improved without any other immunomodulatory medications. Genetic study revealed no primary HLH or inflammasome abnormalities. In this case, underlying autoimmune disease should have been considered as the cause of recurrent MAS in the young patient once primary HLH was excluded.

• Pediatric Infection and Vaccine
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• 제16권2호
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• pp.215-219
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• 2009

5-14세에 호발하는 마이코플라즈마 폐렴은 20-25%에서 폐외 증상을 일으키는 것으로 알려져 있으며 이 중 혈액계 질환에는 용혈성 빈혈, 혈소판 감소증, 혈구포식세포 증식증(hemophagocytosis) 등이 있다. 마이코플라즈마 폐렴에서의 냉항체에 의한 자가면역성 용혈로 용혈성 빈혈이 발생할 수 있으며, 이 때 항체의 역가와 용혈의 정도에 상관관계가 있을 수 있다. 저자들은 마이코플라즈마 폐렴에 의해 용혈성 빈혈이 발현된 유전성 구상 적혈구증 1례를 경험하였기에 보고하는 바이다.