• Title/Summary/Keyword: 특발성 혈소판 감소성 자반증

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Indication of Bone Marrow Aspiration in Acute Idiopathic Thrombocytopenic Purpura in Children (소아 급성 특발성 혈소판 감소성 자반증에서 골수흡인 검사의 적응)

  • Kim, Won-Duck;Hah, Jeong-Ok
    • Journal of Yeungnam Medical Science
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    • v.18 no.2
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    • pp.239-245
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    • 2001
  • Background: Acute idiopathic thrombocytopenic purpura(ITF) is one of the common hematologic disorders in children. Bone marrow aspiration (BMA) is often performed in children with acute ITP to rule out leukemia, aplastic anemia or other hematologic diseases. However, whether BMA is needed in children with typical clinical and hematological features of acute ITP have been questioned. This study was performed to examine the proper indication of BMA in acute childhood ITF. Materials and Methods: The medical records and BMA reports of children with the provisional diagnosis of acute ITP were reviewed from January 1984 to December 2000. Patients were divided into two groups, one with typical and another with atypical clinical and hematological features of acute ITP. Typical acute ITP group was characterized by the history of previous viral infection, well being appearance, no hepatosplenomegaly, no lymphadenopathy, normal Hb, WEC, neutrophil count and peripheral blood smear except thrombocytopenia. A platelet count of $50{\times}l0^9/L$ or lower was the cutoff level. Results: Total 120 children with the provisional diagnosis of acute ITP were included. One hundred eighteen of them were confirmed to have acute ITP by BMAs. Of these, 66 had typical and 54 had atypical features. All of typical features and 52 of 54 with atypical features of acute ITP were confirmed to have acute ITP by BMAs. Two patients with atypical features of acute ITP were diagnosed as aplastic anemia and myelodyspalstic syndrome, respectively, by BMAs. Conclusion: This study concludes that BMA is not needed for the children with typical features of acute ITP but it is needed for the children with atypical features of acute ITP to rule out other hematologic disorders.

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Megakaryocyte Colony Formation of Fetal Liver Cells (태아 간세포의 거핵구 집락형성)

  • Kwon, Byung O;Ju, Hye Young;Kim, Chun Soo;Jeon, Dong Seok;Kim, Jong In;Kim, Heung Sik
    • Clinical and Experimental Pediatrics
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    • v.45 no.2
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    • pp.247-255
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    • 2002
  • Purpose : This study was undertaken to obtain basic data about the megakaryocyte colony formation of fetal liver cells by using immunocytochemical staining and ex vivo culture with growth factors. Methods : The mononuclear cells were isolated from fetal liver and bone marrow with idiopathic thrombocytopenic purpura(ITP) and pancytopenia. These mononuclear cells were cultured in $MegaCult^{TM}-C$(Stem Cell Tech, Canada) media in the presence of growth factors and CFU-Megakaryocyte( CFU-Mk) colonies were counted on day 12. The expansion of CD34+ and CD41+ cell was analyzed by flow cytometry after 5 days incubation using flask culture. Results : The numbers of CFU-Mk colonies of mononuclear cells obtained from fetal liver in the 11th week gestational age were more than those in the 19th week specimens; growth factors could not enhance the colony expansion in all cases. Total numbers of CFU-Mk colony of fetal liver cells were higher than bone marrow from ITP or pancytopenia groups. The numbers of pure or large CFU-Mk colonies of fetal liver cells were also higher than bone marrow specimens. The rate of CD34+ cell expression of fetal liver was increased after flask culture and the enhancement effect of epression was seen only in cases which added thrombopoietin. The rate of CD41+ cell expression of fetal liver was increased after incubation, but the enhancement effect of growth factors was unclear. Conclusion : This study revealed good results about the megakaryocyte colony assay of fetal liver mononuclear cells using $MegaCult^{TM}-C$ media. This study suggests that the fetal liver could be a good source of megakaryocytic progenitor cells for clinical application in hematopoietic stem cell transplantation.

Clinical Significance of Antibodies Against Platelet HLA Class I in Children with Idiopathic Thrombocytopenic Purpura (소아 특발성 혈소판 감소증에서 항-HLA Class I항체의 임상적 의미)

  • Lee, Hong Jun;Yeom, Jung-Sook;Park, Ji Sook;Park, Eun Sil;Seo, Ji-Hyun;Lim, Jae Young;Park, Chan-Hoo;Woo, Hyang-Ok;Youn, Hee-Shang
    • The Korean Journal of Blood Transfusion
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    • v.24 no.3
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    • pp.233-240
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    • 2013
  • Background: A previous history of transfusion has been known to be associated with production of anti-HLA class I antibodies. However, platelet glycoproteins are the main target of idiopathic thrombocytopenic purpura (ITP). The mechanism of antibody production is known to differ significantly between glycoproteins and anti-HLA class I. The aim of this study was to evaluate the clinical significance of anti-HLA class I antibodies in childhood ITP. Methods: Enrollment for the normal control group targeted 48 people who visited Gyeongsang National University Hospital from 1990 to 2010, and 48 young children with ITP. Anti-glycoproteins and anti-HLA class I antibodies were tested using the Modified Antigen Capture Enzyme-linked immunosorbent assay (MACE) kit. Results: The positive rate of anti-HLA antibodies was significantly different [36/39 (92.3%) vs 29/46 (63%)] [ITP group vs normal control group] (P=0.002). The mean positive S/C ratio of anti-HLA antibodies was also significantly different (3.55 vs 1.51) [ITP group vs normal control group] (P=0.0000). The positive rate of anti-HLA did not differ significantly between the transfused group and the non-transfused group [12/12 (100%) vs 24/27 (88%)] [transfused ITP vs non-transfused ITP]. The mean positive S/C ratio of anti-HLA antibodies did not differ significantly between the transfused ITP group and the non-transfused ITP group (4.30 vs 3.25) [transfused ITP vs non-transfused ITP]. Consecutive testing showed that positive rate and positive S/C ratio of anti-HLA antibodies did not change significantly between sampling times in both groups [transfused ITP vs non-transfused ITP] (P=1.00 and P=0.15). Conclusion: Anti-HLA class I antibodies may be involved in childhood ITP. Transfusion did not affect the course of childhood ITP.

A Case of Idiopathic Thrombocytopenic Purpura in Pregnancy (임신과 동반된 특발성 혈소판 감소성 자반증 1례)

  • Kim, Mi-Sook;HwangBo, Ho-Joon;Lee, Young-Gi;Park, Yoon-Kee;Lee, Sung-Ho
    • Journal of Yeungnam Medical Science
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    • v.10 no.2
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    • pp.512-517
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    • 1993
  • Idiopathic thrombocytopenic purpura is an uncommon illness but most common form of thrombocytopenia in pregnancy. Corticosteroids, splenectomy, immunosuppressive drugs, and immunoglobulin therapy have been recommended for management. The optimal method of delivery is controversial. We have experienced a case of idiopathic thrombocytopenic purpura diagnosed previously and managed with corticosteroid and vincristine, which was followed by pregnancy, vaginal delivery and postpartum splenectomy.

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Renal Toxicity of High-dose Intravenous Immunoglobulin in Children with Kawasaki Disease and Idiopathic Thrombocytopenic Purpura (가와사끼병과 특발성 혈소판 감소성 자반증 환아에서 고용량 정주용 면역글로불린의 신독성 유무)

  • Jung Ji Ah;Kim Hye Soon;Seo Jeong Wan;Lee Seung Joo
    • Childhood Kidney Diseases
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    • v.2 no.2
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    • pp.133-137
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    • 1998
  • Purpose : To investigate renal toxicity of high-dose intravenous immunoglobulin(IVIG) in children with Kawasaki disease and idiopathic thrombocytopenic purpura. Methods : 23 children with Kawasaki disease and 7 children with idiopathic thrombocytopenic purpura who were treated with high-dose IVIG(2 g/kg) were evaluated for the change of urine output, blood urea nitrogen(BUN), serum creatinine(Scr), creatinine clearance(Ccr), tubular reabsorption of phosphorus(TRP), fractional excretion of sodium(FENa), 24hour urine ${\beta}_2$-microglobulin/creatinine(${\beta}_{2}MG/cr$) ratio and urine microalbumin/creatinine(MA/cr) ratio at post-IVIG 1 and 3 day. Results : There was no significant change of urine output, BUN, Scr, Ccr, TRP, 24hour urine ${\beta}_{2}MG/cr$ and MA/cr ratio after high-dose IVIG treatment. Transient increase of FENa at post-IVIG 1 day was the only significant change. Conclusion : There was no significant renal toxicity of high-dose IVIG in children with Kawasaki disease and idiopathic thrombocytopenic purpura who had normal renal function.

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A Case of Idiopathic Thrombocytopenic Purpura (특발성 혈소판 감소성 자반증에 대한 고찰 및 증례 1예)

  • Hong, Sang-Hoon
    • The Journal of Internal Korean Medicine
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    • v.21 no.4
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    • pp.677-682
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    • 2000
  • Idopathic thrombocytopenic purpura(ITP) is the most common autoimmune hematologic disorder. The use of glucocorticoids and splenectomy have been the mainstays of treatment of ITP Some studies have shown that chinese herbal medicine treatment increase platelet count level of ITP patients. However, there is a lack of research considering oriental herbal medicine with ITP, in korea. A 33-year-old female who had diagnosed ITP on Mar 1999, was treated herbal medicine for decreased platelet count level$(34000/{\mu}l)$ that was identified on regular follow-up. this patient's platelet count level was get to normal$(139000/{\mu}l)$ by BuQiSheXue(補氣攝血) pattern herbal medicine that composed with Radix Astragali(黃?), Herba Agrimoniae(仙鶴草), Rhizoma Atractylodis Macrocephalae(白朮), Radix codonopsis(黨參), etc. I report the case with a review of literature.

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Comparison of Effectiveness between Two Different Protocols of Treatment of IV γ-globulin in Idiopathic Thrombocytopenic Purpura (특발성 혈소판 감소성 자반증에서 정주용 감마 글로불린의 투여 방법에 따른 효과 비교)

  • Jang, Jae Ho;Lee, Moonsouk;Park, Mira;Choeh, Kyuchul
    • Clinical and Experimental Pediatrics
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    • v.46 no.4
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    • pp.358-362
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    • 2003
  • Purpose : Several methods of IV ${\gamma}-globulin$(IVG) infusion are effective in the treatment of autoimmune disease, including idiopathic thrombocytopenic purpura(ITP). But it is not known which method is more effective in the treatment of ITP. The effectiveness of these two methods of IVG infusion was studied in terms of platelet recovery rate, side effects and recurrence rate. Methods : Forty seven patients with acute ITP in the department of pediatrics, Eulji University Hospital from January 1995 to June 2001 were enrolled. We assesssed the treatment effects of 47 patients blindly selected; IV ${\gamma}-globulin$ 2 g/kg/day in one day(treatment group A, n=25), 400 mg/kg/day in five days(treatment group B, n=22). Results : Treatment group A increased platelet count more rapidly on the 2nd, 4th and 6th day of treatment than treatment group B. Side effects like fever, chill and vomiting were more frequent in treatment group A than treatment group B. The platelet count on the sixth day of treatment showed a greater increase in the cases which had side effects than in the cases which did not. There was no difference between the two groups in the recurrence rate. Conclusion : IV ${\gamma}-globulin$ 2 g/kg/day in one day increases platelet count more rapidly than 400 mg/kg/day in five days, and is favorable for the prevention of a severe hemorrhagic episode like early intracranial hemorrhage.

Graves' disease Associated with Idiopathic Thrombocytopenic Purpura and Iron Deficiency Anemia (특발성 혈소판 감소성 자반증 및 철 결핍성 빈혈을 동반한 Graves 씨병 1례)

  • Kim, Jong-Myung;Yun, Sung-Chul;Choi, Soo-Bong;Lee, Hyun-Woo;Kim, Kyeong-Dong;Kim, Chung-Sook;Nam, Hei-Ju;Choi, Won-Hee;Lee, Tae-Sook
    • Journal of Yeungnam Medical Science
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    • v.5 no.1
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    • pp.173-179
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    • 1988
  • Since 1931, sporadic reports have appeared noting an apparent association between hyperthyroidism and idiopathic thrombocytopenic purpura. Recent various studies suggested that these two diseases may share a similar immunologic background, but the the exact mechanism is still a matter of speculation. This 22-year old female patient visisted this hospital because of general weakness and purpura of legs for 2 months. The laboratory findings were compatible with Graves' disease associated with idiopathic thrombocytopenic purpura. The palatclet count was 16000/$mm^3$, hemoglobulin was 10.9g/dl and MCV was 60.1tL. T3 was 490.53ng/dL, T4 was 24ug/dL and free T4 was 5.66ng/dL. Antiplatelet antibody and anti-microsomal antibody were positive. The bone marrow findings were compatible with tron deficiency anemia and idiopathic thrombocytopenic purpura. The thyroid biopsy showed adenomatous goiter. She was administered with propyl thiouracil, Beta-blocker, iron and prednisolon. On the 10th hospital day, platelet count was 184000/$mm^3$, hemoglobulin was 12.0gm/dL and MCV was 67.5fL. On the 20th hospital day, T4 was 10.35ug/dL and free T4 was 2.30ng/dL. Therefore she was discharged and followed up.

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Changes in Mean Platelet Volume and Platelet Distribution Width after Therapy in Childhood Idiopathic Thrombocytopenic Purpura (소아 특발성 혈소판 감소성 자반증 환아에서 치료경과에 따른 평균 혈소판용적과 혈소판용적 분포폭의 변동)

  • Kim, Jong Tai;Lee, Kyung Won;Kim, Soon Nam;Kim, Moon Kyu
    • Clinical and Experimental Pediatrics
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    • v.45 no.4
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    • pp.505-511
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    • 2002
  • Purpose : Mean platelet volume(MPV) and platelet distribution width(PDW) are useful parameters in evaluating disorders of platelets. In cases with idiopathic thrombocytopenic purpura(ITP), they change as platelet count increases. In this study, we compared the values of MPV and PDW in ITP patients at diagnosis with those of normal children. We also studied whether the early changes in MPV may predict the clinical course. Methods : From December 1995 to May 2001, 71 patients with ITP were admitted to Ajou University Hospital. They were treated with IVIg 400 mg/kg for five days and MPV, PDW, platelet count were analysed. Normal control group(n=38) was compared. The study group was divided into acute and chronic forms, and also divided into group A, good early responders whose platelets increased more than $100,000/{\mu}L$ within 5 days and group B who did not. Results : Mean value of MPV at diagnosis in ITP patients was lower than the normal control group(P<0.05). In group A, MPV was abruptly increased on the first day after IVIg and then started to decrease. But in group B, MPV was steadily increased until the fourth day after IVIg. In the normal control group, there were inverse correlations between platelet count and MPV(r=-0.415, P<0.05), but in ITP patients, there were positive relationships between platelet count and MPV(r=0.646, P<0.05) at diagnosis. Conclusions : MPV at diagnosis of ITP was lower than the normal control. MPV and PDW could not predict the course of ITP patients, but MPV could distinguish good early responders. More research is needed to find out the reasons of decreased MPV at diagnosis of ITP.