재생 불량성 빈혈 환자에서 B 형 및 C 형 간염 바이러스 간염에 대한 분석 평가

김남진(Nam Jin Kim),임우현(Woo Hyun Lim),유상두(Sang Doo You),김명성(Myeong Seong Kim),서순팔(Soon Pal Suh),이현철(Hyun Chul Lee),황태주(Tae Joo Hwang),김세종(Sei Jong Kim) 대한내과학회 1998 대한내과학회지 Vol.55 No.3

Objective: Aplastic anemia is a rare but serious complication of viral hepatitis. Both aplastic anemia and viral hepatitis are more common in Korea than in the Western countries. It is necessary to study about the relationship between them. Methods: Twenty-three patients with aplastic anemia visiting Chonnam University Hospital from 1995 to 1996 were studied for positivity of hepatitis B virus (HBV) and hepatitis C virus (HCV) to investigate the association of hepatitis virus infection with aplastic anemia. The surface antigen of HBV (HBsAg) and anti-HCV in sera were tested by EIA(enzyme immunoassay), and the presence of HBV-DNA and HCV-RNA in both sera and bone marrow cells was examined by the polymerase chain reaction (PCR). Results: The positivities of HBsAg and anti-HCV in 23 patients with aplastic anemia were 4.3% (1 patient) and 8.7% (2 patients), respectively. The positivity of HBsAg is similar to that of HBsAg in general population of Korea. The positivity of anti-HCV is higher than that of anti-HCV in general population of Korea. One patient had HBV DNA and 3 patients had HCV HNA in their sera. All of the 3 hepatitis C viremic patients received 11 to 15 units of blood products in the past. None of the patients showed the evidence of recent viral hepatitis infection. HBV DNA and HCV RNA were not detected by the PCR in bone marrow cells in any of the patients. Conclusion: This study suggests that the HBV or HCV might not be a causative agent of aplastic anemia. The higher positivity of anti-HCV in the patients might be due to passive transmission of EICV after transfusion of blood products.

재생불량성 빈혈의 Antilymphocyte Globulin 요법 : Androgen 요법과의 비교 Comparison with Androgen Treatment

김광원,최정일,정병천,이재태,이규보,황기석 대한내과학회 1992 대한내과학회지 Vol.42 No.1

1970년 1월부터 1990년 8월 사이에 경북대학교 병원에서 재생불량성 빈혈로 진단받은 환자들중 ALG 치료를 받은 33명과 androgen 치료를 받은 88명, 그리고 수혈 등 보존적 치료만을 한 15명을 대상으로 각 치료에 따른 치료 반응도와 생존율을 비교하고 그 예후인자를 살펴보았다. 1) ALG 치료와 androgen 치료의 치료반응도를 비교해 보면 ALG 치료군이 42%였고 androgen 치료군은 38%로 두군 사이에 유의한 차이가 없었다. 2) ALG 치료군에서는 30세 이상에서 30세 미만보다 반응도가 높았고(58% 대 33%), T4/T8이 1미만인 5명 중 4명이 반응하였는데 반하여 T4/T8 비율이 1이상인 8명에서는 반응이 없었다. 진단으로부터 치료까지의 기간이나 호중구 수치는 치료반응과 상관이 없었다. 3) ALG 요법에서 치료반응 환자와 무반응 환자간의 말초혈액 소견이나 골수소견, 혈청 철소실 반감시간들을 비교하면 치료반응군에서 T4/T8 비율이 낮았고, 혈철 철 소실 반감시간이 비교적 짧았다. 4) 6개월 내에 사망한 조기 사망자에서는 중증 재생불량성 빈혈이 62%로서 많이 분포되어 있었다. 5) 전대상 환자의 생존율은 2년 생존율이 64%, 10년 생존율이 50%였다. 2년 생존율을 치료방법으로 구분하면 ALG 치료군이 78%로서 angrogen 치료군 66%, 보조적 치료군 27%에 비하여 유의하게 높았으며(p=0.0000), ALG 치료군 중에서도 치료반응군에서 생존율 94%가 치료무반응군 47%에 비하여 월등히 높았고 비중증 재생불량성 빈혈군의 2년 생존율이 77%로서 중증 재생불량성 빈혈의 43%에 비하여 유의하게 높았다(p=0.0000). 6) ALG 치료에 따른 부작용은 발열 및 완, 자반증, 발적, 치주출혈, 주사부의 동통, 관절통 등이 있었다. 이상의 결과를 보아 ALG 치료는 angrogen 치료나 보존적 요법에 비해 재생불량성빈혈 환자의 생존율을 높일 수 있고, 그중에서도 조기 사망률이 높은 중증 재생불량성빈혈 환자에게 또한 연령이 많은 환자에서 우선적으로 사용할 수 있는 것으로 판단된다. In order to evaluate the therapeutic efficacy of antilymphocyte globulin (ALG) in the aplastic anemia, the authors investigated 136 cases of aplastic anemia that comprised 33 cases of ALG treatment and 103 cases of non-ALG treatment in Kyungpook National University Hospital from january 1, 1970 to August 31, 1990. The response rate of ALG was 42%, while the response rate of androgen was 38%, which showed little significant difference. In responded patients showed a lower T4/T8 ratio and relatively short plasma radioiron disappearance half time. Severe aplastic anemia was 62% of the early death group which died within 6 months after diagnosis. A two year survival rate was 64% in the all cases, but the 2 year survival rate was 78% in the ALG treated group, and 27% in the support treated group, that revealed a significant difference. The responders to ALG treatment had remarkably high survival rate in comparison to nonresponders. The non-severe aplastic anemia had a higher survival rate than severe aplastic anemia. The side reactions of ALG were: fever and chills, purpura, erythema, gum bleeding, pain on the injection site, arthralgia and so on. We observed that ALG was more effective in the treatment of aplastic anemia than the androgen and supportive therapy only and so concluded that the ALG is useful in severe aplastic anemia of old age.

재생불량성빈혈 환자에서의 수술 시행예에 대한 고찰

한지숙(Jee Sook Hahn),정경섭(Kyung Sup Chung),이선주(Sun Ju Lee),고윤웅(Yun Woong Ko),조장환(Chang Hwan Cho),박정수(Cheong Soo Park) 대한내과학회 1991 대한내과학회지 Vol.40 No.1

With improved management in the patient of aplastic anemia, complications that require surgical intervention are becoming more frequent. In the past, conventional management of complications that require surgical intervention in patients with pancytopenia were mainly conservative and nonoperative. Because of the presumed high mortality and morbidity, surgery has often been avoided. However, advance in supportive techniques such as transfusion of platelet and leukocyte and introduction of new broad-spectrum antibiotics has made surgical approach possible. Fourteen cases of aplastic anemia who underwent major or minor surgery for surgical complications or diagnostic purpose in the department of internal medicine from December 1972 to February 1989 were evaluated. The results obtained were as follows. 1) The incidence of surgery in 217 cases of aplasic anemia was 6.5 percent. Their median age was 29.5 years with female preponderance (M:F=1:2.5). The severe aplastic anemia (SAA) were 4 cases and non-severe aplastic anemia (non-SAA) 10 cases. 2) Eighteen surgical procedures have been performed, of which the major surgery were 10, and minor surgery 8 cases. Of the major surgery 3 (30%) occasions were undertaken in 2 SAA cases. 3) The types of major surgery included 2 cases of appendectomy, and 1 case of subtotal gastrectomy, cholecystectomy with T-tube drainage, cholecystostomy, hernioplasty, amputation of leg in each, and 3 cases of Cesarean section. Those of minor surgery presented 3 cases of incision and drainage, 1 case of implantation and removal of intravenous injection port, skin biopsy, wound repair, and dilatation and evacuation in each. 4) Of the 10 major surgical procedures, seven were emergent surgery and three elective surgery. The median duration from the diagnosis to the major surgical procedure was 0.5 months (4 days~47.3 months), and the median survival of the surgery was 12.3 months (4 days~38+ months). 5) The mean platelet count before the major surgery was 27,400/㎣ (8,000~94,000/㎣), and it rose to 94,400/㎣ (34,000~166,000/㎣) after average 6.8 units of platelet transfusion. 6) Three surgical complications were encountered in 2 cases, comprising of 1 case of sepsis, and pneumonia, and atelectasis in another 1 without any bleeding complications. The one SAA case complicating the both sepsis and pneumonia was dead within 30 days after surgery. 7) Total 3 cases were dead, whose causes were infection, stomach cancer, and periampullary cancer, respectively. It is concluded that the surgical intervention should be strongly considered in patients with aplastic anemia requiring surgery unless they are terminally ill, which could be attributable to the development of supportive care and would offer enough chance for definite treatment of aplastic anemia and reasultant longer survival. The surgeon and the hematologist must work together to facilitate a major operation when it is indicated.

임신 중 진단된 재생불량성 빈혈의 임상적 특징 및 예후

권지영 ( Ji Young Kwon ),조윤성 ( Youn Sung Jo ),안현영,이귀세라 ( Gui Se Ra Lee ),이영 ( Young Lee ),신종철 ( Jong Chul Shin ),이종건 ( Jong Kun Lee ),나종구 ( Jong Gu Rha ),김수평 ( Soo Pyung Kim ) 대한주산의학회 2006 大韓周産醫學會雜誌 Vol.17 No.2

목적 : 임신 중 발생한 재생불량성 빈혈의 임상적 특정 및 임신과 신생아에 미치는 예후를 알아보고자 본 연구를 시행하였다. 방법 : 1996년 1월부터 2005년 6월까지 본원에서 분만한 산모 중 임신 중 재생불량성 빈혈을 처음으로 진단받은 산모 14명을 대상으로 임상적 특정 및 분만기록과 신생아기록을 조사하였다. 결과 : 14명 중 11명(78%)에서 임신 제2 삼분기 이후에 재생불량성 빈혈을 진단받았다. 발견 당시부터 중증의 재생불량성 빈혈이었던 경우 3명을 포함하여 총 8명이 임신 기간 중 중증의 재생불량성 빈혈이었다. 모든 경우에서 임신 중 약물요법을 시행하지 않았다. 산전 진찰기간 중 수혈을 받은 경우는 7명 이었고, 분만 전후에 수혈을 받은 경우는 13명이었다. 분만 후 추적 관찰이 가능했던 12명에서 1명이 분만 후 완전관해가 되었으며 8명은 부분관해가 되었다. 2명은 골수이식 후 완전관해가 되었다. 조산이 1예가 있었으며 1예에서는 신생아 빈혈이 있었다. 결론 : 임신 중 재생불량성 빈혈의 예후는 기존의 보고에 비해서 매우 향상되었으며 혈색소와 혈소판 수혈을 통한 보존적인 치료가 매우 중요하다. Objective: Pregnancy-associated aplastic anemia remains a rare occurrence. The aim of this study was to examine the maternal and fetal outcomes of pregnancy-associated aplastic anemia treated with supportive care. Methods: From January 1995 to December 2004, a total of 14 women newly diagnosed with pregnancy-associated aplastic anemia were recruited for the study. Results : Eleven (78%) of the 14 women were diagnosed with pregnancy-associated aplastic anemia during the second or third trimester. There were eight severe cases; three of which were diagnosed at the initial presentation. All 14 women had conservative management with transfusions but not specific immunological or hormonal therapies during pregnancy. Blood transfusions were performed prenatally in seven mothers and perinatally in 13. Of the 12 patients eligible for follow-up, one achieved complete remission and another eight showed partial remission after delivery. During the follow up period, there was no case of maternal-fetal death in our series. The pregnancies were continued uneventfully in most cases. Conclusions : This study demonstrated favorable maternal and neonatal outcomes with transfusion support alone for pregnancy-associated aplastic anemia. Therefore, pregnancy continuation with meticulous blood support should be considered, rather than therapeutic termination, for women with pregnancy-associated aplastic anemia.

A Case of Ethosuximide-Induced Aplastic Anemia Successfully Treated with Methylprednisolone Pulse Therapy

강현식,김선형 대한소아혈액종양학회 2018 Clinical Pediatric Hematology-Oncology Vol.25 No.2

Aplastic anemia may develop secondary to environmental exposure to entities such as chemicals, medical drugs, and infectious agents. Fatal complications from antiepileptic medications may occur despite careful and appropriate use. We report the case of a 9-year-old girl with a presenting diagnosis of aplastic anemia following treatment with ethosuximide for absence seizures. Aplastic anemia can now be cured with stem cell transplantation or immunosuppressive therapy. In this case, however, because of the impossibility of bone marrow transplantation and the specific needs of the patient’s parents, three courses of methylprednisolone pulse therapy were administered. Following the therapy, there was improvement in pancytopenia and complete remission in the bone marrow. No adverse side effects of therapy were observed. The authors suggest that methylprednisolone pulse therapy may be a treatment for acquired aplastic anemia.

The Effect of Juakium-derivative(左歸飮加味方) on Aplastic Anemia

Lee,Jang-hoon,Woo,Hong-jung,Kim,Sun-min INSTITUTE OF ORIENTAL MEDICINE KYUNG-HEE UNIVERSIT 1997 JOURNAL OF ORIENTAL MEDICINE Vol.2 No.1

Aplastic anemia is a fatal disease characterized by a peripheral pancytopenia and markedly decreased hematopoietic activity in the bone marrow. It may be inherited but more commonly may develop as a reaction to a drug. 5-Fluorouracil(5-FU) is a chemotherapeutic mainly for stomach cancer, but the suppression of bone marrow is a controversial side effect of this drug. This study is to evaluate the effect of Juakium-derivative on aplastic anemia induced by 5-FU on mice. Forty 5-week old ICR mice were divided into four groups: normal, 5-FU only, 5-FU+Juakium-derivative and Juakium-derivative. 100mg/Kg of 5-FU was administered to induce aplastic anemia in the 5-FU only group. And 1000mg/kg of Juakium derivative was supplementarily administered in the 5-FU+Juakium-derivative group. Hematologic findings, body weight, organ weight and histopathological findings were observed. In mouse body weight, the weight of Juakium-derivative+5-FU group and 5-FU only group was decreased compared to the normal group. But the weight of the Juakium-derivative+5-FU group was higher than that of the 5-FU only group. In the hematologic study, the Juakium-derivative+5-FU group showed improvement in WBC, RBC, Hbc, Hct, and PLT compared with the 5-FU only group. The weight of extracted spleen and thymus in the Juakium-derivative+5-FU group was significantly higher than that of the 5-FU only group. The weight of extracted live and heart exhibited no significant difference between the Juakium-derivative+5-FU group and the 5-FU only group. In the histopathologic findings, compared with 5-FU only group, the Juakium-derivative+5-FU group showed few megakaryocytes in the cortex of the spleen and well-preserved lymphatic nodules. Furthermore the atrophy of the thymus was milder in the Juakium-derivative+5-FU group than in the 5-FU only group. Juakium-derivative has a therapeutic effect on aplastic anemia induced by 5-FU, and it is expected that combined administration of Juakium-derivative and 5-FU can decrease the side effects of 5-FU. Therefore, Juakium-derivative is recommendable for clinical use in the treatment of aplastic anemia. Further study on the supplemental herbs in this prescription is required.

중증 재생불량성 빈혈환자의 구강악안면외과적 관리

윤현중,박재억,Yoon, Hyun-Joong,Park, Je-Uk 대한구강악안면외과학회 2001 대한구강악안면외과학회지 Vol.27 No.5

Aplastic anemia is a hematopoietic disorder characterized by marked reduction or absence of erythoid, granulocytic, and megakariocytic cells in the bone marrow with resultant pancytopenia. To control of infection & bleeding secondary to leukopenia and thrombocytopenia, the inflammatory lesions in oral & maxillofacial area should be removed. Material & Method: The extractions were performed on 21 patients with severe aplastic anemia. The initial, pre-operative and postoperative CBCs were checked up. Amount and kind of transfused platelet in each patient and increment of platelet level were recorded. The complications were documented. Result : A mean of 2.9 teeth were extracted from each patient(ranging between 1 and 13). Furthermore, surgical extractions including ostectomy and odontectomy of the third molar were performed on 11 patients. The preoperative WBC levels presented between $600/{\mu}L$ and $5000/{\mu}L$(mean $2376/{\mu}L$). The WBC values decreased by an average of $145/{\mu}L$ per patient after extractions had been performed. The teeth of 16 patients were extracted under 10.0g/dL, and the mean change in postoperative hemoglobin levels in comparison with preoperative hemoglobin levels was -0.06 per patient. The initial platelet levels were between 1000/(L and $81,000/{\mu}L$(mean $20,174/{\mu}L$). In five patients, extractions were performed with platelet levels less than $50,000/{\mu}L$. Conclusion : The results suggest that more active and preventive treatments in the oral and maxillofacial area are possible and are necessary to remove the infectious foci on the patients with severe aplastic anemia. We report the results of our experiences and literature reviews in treatment of the patients with severe aplastic anemia in our department.

재생 불량성 빈혈(Aplastic anemias) 환자의 치주 치료 증례

배규현,한수부,김우성,이혜아,김동균,Bae, Kyoo-Hyun,Han, Soo-Boo,Kim, Woo-Sung,Lee, Hye-Ja,Kim, Dong-Kyoon 대한치주과학회 1998 Journal of Periodontal & Implant Science Vol.28 No.1

Aplastic anemia is a disease characterized by general lack of bone marrow activity; It may affect not only the red blood cells but also the white blood cells and platelets, resulting in pancytopenia. Spontaneous gingival hemorrhage is present in some cases and it is related to the blood platelet deficiency. This case report presents the periodontal treatment of a patient with aplastic anemia. A 43-year-old female was referred for continuous gingival bleeding after periodontal treatment. Periodontal findings revealed generalized gingival imflammation, oozing of blood from gingival crevice, and it was diagnosed as adult periodontitis. Root planing and extraction of the upper left third molar with poor prognosis were put into operation after elevation of the platelet count with platelet transfusion. The extraction socket was sutured with 3-0 silk. Bleeding continued even after digital compression at the upper right second premolar, second molar, and left canine areas, which presented severe inflammation. Although platelets were transfused repeatedly, platelet count did not stay elevated since survival rate of the transfused platelets were low due to alloimmunization. Thrombin gauze packing was not effective. Bleeding ceased 3 days after treatment with transfusion of donor platelets. 20 days after the treatment, the gingiva was generally healthy except upper right second premolar and lateral incisor areas. The result of periodontal treatment was good, but bleeding control after treatment was troublesome. In the periodontal treatment of patient with aplastic anemia, elevation of the platelet count with platelet transfusion seems to be the best method for hemorrhage control.

중증 재생불량성 빈혈환자의 구강악안면외과적 관리

윤현중,박재억 大韓口腔外科學會 2003 大韓口腔外科學會誌 Vol.27 No.5

후천성 재생불량성빈혈의 세포유전학적 소견

이경아,김선희 대한임상병리학회 2001 대한임상병리학회지 Vol.21 No.4