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A Case of Acquired Amegakaryocytic Thrombocytopenia Responding to Immunosuppressive Therapy.

Yong Ha HWANG ; Soo Mee BANG ; Hye Sook HAHN ; Jeong Yeal AHN ; Eun Kyung CHO ; Dong Bok SHIN ; Jae Hoon LEE

Korean Journal of Hematology.2002;37(2):147-152.

Acquired amegakaryocytic thrombocytopenia is a relatively rare bone marrow failure disorder characterized by severe thrombocytopenia associated with a total absence or a marked reduction in the number of bone marrow megakaryocytes. We report a case of acquired amegakaryocytic thrombocytopenia. A 39-year old man admitted our hospital because of gingival bleeding and purpura on the thigh, his initial complete blood cell counts were white blood cell 5.6 103/micro liter hemoglobin 9g/dL, and platelet 1 103/micro liter On the bone marrow study, megakaryocyte was not observed and cytogenetic analysis of marrow was 46, XY, inv(9). (p11q13). Other autoimmune markers were negative. The patient received steroid therapy during 8 weeks, but there was no significant improvement and then he received immunosuppressive therapy with antithymocyte globulin and cyclosporin-A. Thereafter the platelet count increased to 80 103/micro liter, and this level continued for 10 months
Adult ; Antilymphocyte Serum ; Blood Cell Count ; Blood Platelets ; Bone Marrow ; Cytogenetic Analysis ; Hemorrhage ; Humans ; Leukocytes ; Megakaryocytes ; Platelet Count ; Purpura ; Thigh ; Thrombocytopenia*

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Nonmyeloablative Stem Cell Transplantation in a Patient with Refractory Acute Myeolgeous Leukemia with Central Diabetes Insipidus.

Young Shin KIM ; Inho KIM ; Hyun Min PARK ; Moon Hee LEE ; Chul Soo KIM

Korean Journal of Hematology.2002;37(2):143-146.

A 31-year-old man presented fatigue, polydipsia and polyuria. He was diagnosed as acute myelogenous leukemia (AML) FAB-M1, and a water deprivation test confirmed central diabetes insipidus (DI). A sella magnetic resonance imaging showed the thickening of pituitary stalk with contrast enhancement suggesting leukemic infiltration. He was treated with remission induction chemotherapy including cytosine arabinoside and idarubicin, and concurrent intrathecal methotrexate, cytosine arabinoside and hydrocortisone. But he was not achieved a remission. Reinduction chemotherapy was also failed to induce remission. He underwent a non-myeloablative allogeneic he matopoietic stem cell transplantation (NST) from HLA one antigen mismatched sibling donor for refractory AML. After transplantation, he had no evidence of leukemia and DI, He showed complete conversion of donor chimerism. By day 7 after NST, desmopressin (DDAVP) was no longer required and a follow-up at 9 months he has no evidence of relapse. We report a rare case recovered from diabetes insipidus associated with acute myelogenous leukemia after NST in Korea.
Adult ; Chimerism ; Cytarabine ; Deamino Arginine Vasopressin ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic* ; Drug Therapy ; Fatigue ; Follow-Up Studies ; Humans ; Hydrocortisone ; Idarubicin ; Korea ; Leukemia* ; Leukemia, Myeloid, Acute ; Leukemic Infiltration ; Magnetic Resonance Imaging ; Methotrexate ; Pituitary Gland ; Polydipsia ; Polyuria ; Recurrence ; Remission Induction ; Siblings ; Stem Cell Transplantation* ; Stem Cells* ; Tissue Donors ; Water Deprivation

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Two Cases of Intravascular Lymphomatosis.

Soon IL LEE ; Won Seog KIM ; Jeeyun LEE ; Seo Young SONG ; Joon Oh PARK ; Kihyun KIM ; Chul Won JUNG ; Young Hyuck IM ; Won Ki KANG ; Hong Ghi LEE ; O Jung KWON ; Ki Woong SUNG ; Hong Hoe KOO ; Young hyeh KO ; Sung Soo YOON ; Keunchil PARK

Korean Journal of Hematology.2002;37(2):138-142.

Intravascular lymphomatosis is a rare lymphoma characterized by neoplastic proliferation of malignant cells within the lumen of small blood vessels, usually presenting in the central nervous system or on the skin. Intravascular lymphomatosis is manifested clinically by fever, dementia, cutaneous nodules or plaques, and occasionally, dyspnea. The diagnosis of intravascular lymphomatosis is difficult because of misleading clinical features mimiking vasculitis, infection, stroke, or other neoplasm. We report two cases of intravascular lymphomatosis pesented as fever and skin rash. Those are confirmed by involved tissue biopsy. All cases were treated by combination chemotherapy, but the response was not good. Infectious problems were complicated and disease were progressed.
Biopsy ; Blood Vessels ; Central Nervous System ; Dementia ; Diagnosis ; Drug Therapy, Combination ; Dyspnea ; Exanthema ; Fever ; Lymphoma ; Skin ; Stroke ; Vasculitis

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A Case of Bone Marrow Involvement of Hepatosplenic gamma delta-Cell Lymphoma.

Seong Kyu LEE ; Hee Yeon WOO ; Quehn PARK ; Sun Hee KIM ; Hong Gee LEE ; Young Hyeh KO

Korean Journal of Hematology.2002;37(2):134-137.

Hepatosplenic gamma delta-cell lymphoma is a rare histologic type of peripheral T-cell lymphomas, clinically characterized by predominant involvement of liver and spleen, no or little adenopathy, and an often aggressive course. We report a case of bone marrow involvement of hepatosplenicgamma delta-cell lymphoma in a 21- year-old woman who presented with fever, anemia, thrombocytopenia, and hepatosplenomegaly. A lymphoma was found subsequently by bone marrow biopsy and computed tomography scan of the abdomen and pelvis. Immunologic characterization of lymphoma cells in bone marrow revealed positivity for CD2, CD3, and CD16/56, and negativity for CD4, CD5, CD7, CD8, CD34, and terminal deoxynucleotidyl transferase (TdT). Conventional cytogenetic studies revealed the presence of isochromosome 7q. Using the PCR-SSCP technique, monoclonal gene rearrangement of the T-cell receptor gamma chain was demonstrated. Thus, we could make a confirmatory diagnosis as hepatosplenic gamma delta-cell lymphoma.
Abdomen ; Anemia ; Biopsy ; Bone Marrow* ; Cytogenetics ; Diagnosis ; DNA Nucleotidylexotransferase ; Female ; Fever ; Gene Rearrangement ; Humans ; Isochromosomes ; Liver ; Lymphoma* ; Lymphoma, T-Cell, Peripheral ; Pelvis ; Receptors, Antigen, T-Cell ; Spleen ; Thrombocytopenia

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Safety and Pharmacokinetics of Intravenous Busulfan as Conditioning prior to Allogeneic Stem Cell Transplantation.

Yoon Hee PARK ; Seok LEE ; Yoo Jin KIM ; Dong Gun LEE ; Chi Young PARK ; Ho Jin SHIN ; Hee Je KIM ; Chang Ki MIN ; Dong Wook KIM ; Jong Wook LEE ; Woo Sung MIN ; Chun Choo KIM

Korean Journal of Hematology.2002;37(2):127-133.

BACKGROUND: The unpredictable intestinal absorption and bioavailability of oral busulfan (BU) has limited the drug's use in high-dose pretransplant conditioning therapy. To overcome these problems, several trials for the evaluation of pharmacokinetics and clinical usefulness of an intravenous BU (IVBU) formulation have been reported. Here we present clinical and pharmacokinetic data on patients receiving IVBU as a component of conditioning regimens for allogeneic stem cell transplantation (SCT) in our center. METHODS: A total of 6 adult patients were entered onto this study. All patients were treated with IVBU (0.8mg/kg every 6 hours x 8~16)-containing conditioning regimen followed by HLA-identical allogeneic SCT. We also investigated the pharmacokinetics of IVBU using high-performance liquid chromatography in two cases. RESULTS: All patients achieved successful engraftment. No patient experienced hepatic veno-occlusive disease or neurologic toxicity. Five of 6 patients still alive in complete remission have been followed for 8~12 months after SCT. The measured maximum concentration for the first dose was 1,175ng/mL and 951ng/mL, and the half-life was 2.25h and 3.09h, respectively. The area under the plasma concentration-time curve was 4,596ng h/ mL and 3,067ng h/mL, respectively. There was no significant difference between the first and last dose pharmacokinetic parameters. CONCLUSION: We suggest that IVBU should be considered as appropriate replacement for oral BU in pretransplant conditioning therapy prior to SCT in Korea. Further studies with sizable patients are needed to define the role of IVBU in SCT setting.
Adult ; Behavior Therapy ; Biological Availability ; Busulfan* ; Chromatography, Liquid ; Half-Life ; Hepatic Veno-Occlusive Disease ; Humans ; Intestinal Absorption ; Korea ; Pharmacokinetics* ; Plasma ; Stem Cell Transplantation* ; Stem Cells*

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Risk Factors of Hemorrhagic Cystitis after Allogeneic Stem Cell Transplantation.

Ho Jin SHIN ; Jong Wook LEE ; Chi Young PARK ; Yoon Hee PARK ; Yoo Jin KIM ; Seok LEE ; Chang Ki MIN ; Hee Je KIM ; Dong Wook KIM ; Woo Sung MIN ; Chun Choo KIM

Korean Journal of Hematology.2002;37(2):120-126.

BACKGROUND: Hemorrhagic cystitis (HC) is one of major causes of morbidity during hematopoietic stem cell transplantation (HSCT), occurring in 7~52% of transplant recipients. We have analyzed the incidence, risk factors, and complications of early (< or = 4 weeks post- transplant) and late-onset HC (4 weeks~100 days post-transplant). METHODS: To investigate the risk factors and complications of HC, we retrospectively analyzed 378 patients who underwent allogeneic HSCT in Catholic Hematopoietic Stem Cell Transplantation Center between January 1998 and December 2000. Urine specimens of patients with HC were requested for the detection of virus by polymerase chain reaction (PCR) and culture. RESULTS: HC occurred in 28 patients with an incidence of 7.4%, and 24 among 28 patients (86%) had severe HC (> or = grade II) with urinary obstruction and renal failure (n=2). One patient with grade IV HC died of pneumonia associated with persistent HC. Early and late- onset HC developed at median 9 (2~20) and 55 (31~100) days post-transplant, respectively. Median duration of HC was 16 (3~153) days. Of 23 evaluable patients for study in urine, BK virus was detected in 52% by culture and in 61% by PCR, whereas adenovirus in 18% by PCR. By univariate analysis, disease of aplastic anemia (P=0.03) and non-use of radiation in conditioning regimen (P=0.003) were risk factors for early-onset HC, while the use of busulfan in conditioning regimen (P= 0.02) and grade II-IV acute graft-versus-host disease (GVHD) (P=0.00001) for late-onset HC. By multivariate analysis, use of busulfan (RR=16.62, P=0.002) and aplastic anemia than other disease (RR=9.6, P=0.008) were unfavorable factor for early-onset HC, as only grade II-IV acute GVHD (RR=6, P=0.001) for late- onset HC. CONCLUSION: More than half of patients with HC developed after allogeneic HSCT were associated with urinary excretion of BK virus. Because of HC is one of the important causes of morbidity after allogeneic HSCT, special attention should be paid to attempting the prevention of HC in patients with high-risk for the development of HC.
Adenoviridae ; Anemia, Aplastic ; BK Virus ; Busulfan ; Cystitis* ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Incidence ; Multivariate Analysis ; Pneumonia ; Polymerase Chain Reaction ; Renal Insufficiency ; Retrospective Studies ; Risk Factors* ; Stem Cell Transplantation* ; Stem Cells* ; Transplantation

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Hematopoietic Stem Cell Transplantation for Paroxysmal Nocturnal Hemoglobinuria.

Ho Jin SHIN ; Hee Je KIM ; Chi Young PARK ; Yoon Hee PARK ; Yoo Jin KIM ; Seok LEE ; Chang Ki MIN ; Dong Wook KIM ; Jong Wook LEE ; Chun Choo KIM ; Woo Sung MIN

Korean Journal of Hematology.2002;37(2):114-119.

BACKGROUND: Paroxysmal nocturnal hemogolbinuria (PNH) is an acquired clonal hematological disorder characterized by intermittent episodes of hemolysis, a predisposition to venous thrombosis, defective hemopoiesis, and occasionally, transition to leukemia. Because PNH is a stem cell disorder, treatment with androgen or corticosteroids may only be a palliative measure, and allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment. We report here our experience of eight PNH patients who underwent HSCT. METHODS: Between January 1997 and July 2001, 8 patients, aged 16 to 47 (median 27.5), underwent HSCT for PNH at the Catholic Hemopoietic Stem Cell Transplantation Center. Median time from diagnosis to HSCT was 60.5 months (range 5-165 months). Two different conditioning regimens included busulfan (16mg/ kg) and cyclophosphamide (120mg/kg) or TBI 1,200cGy and cyclophosphamide (120mg/kg). Graft-versus-host disease (GVHD) prevention was cyclosporine with methotrexate (MTX)(N= 6), FK506 with MTX (N=1) or cyclosporine with T-cell depletion of donor marrow (N=1). RESULTS: Four deaths occurred in the first post-transplant year. Deaths were from graft failure (N=1), pneumonia (N=1) and veno- cclusive disease/thrombotic thrombocytopenic purpura (N=2). Four patients (50%) remain alive at a median of 26 months (range 1-60 months) and 5-year probability of survival was 66.7% after HLA-matched sibling HSCT. Grade I or II acute GVHDs occurred in 3 patients and chronic GVHD did not develop in 5 patients other than 3 patients who died within 100 days post-transplant. CONCLUSION: This study suggests that HSCT is an effective therapeutic option for PNH. Further studies are needed to decide the appropriate conditioning regimens to overcome treatment-related mortality after transplantation.
Adrenal Cortex Hormones ; Bone Marrow ; Busulfan ; Cyclophosphamide ; Cyclosporine ; Diagnosis ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Hemoglobinuria* ; Hemoglobinuria, Paroxysmal ; Hemolysis ; Humans ; Leukemia ; Methotrexate ; Mortality ; Pneumonia ; Purpura, Thrombocytopenic ; Siblings ; Stem Cell Transplantation ; Stem Cells ; T-Lymphocytes ; Tacrolimus ; Tissue Donors ; Transplants ; Venous Thrombosis

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Allogeneic Hematopoietic Stem Cell Transplantation Using Intensified Conditioning Regimen and High dose Stem Cells in Severe Aplastic Anemia .

Chi Young PARK ; Woo Sung MIN ; Ho Jin SHIN ; Yoo Jin KIM ; Yoon Hee PARK ; Hee Je KIM ; Seok LEE ; Chang Ki MIN ; Dong Wook KIM ; Jong Wook LEE ; Jong Won PARK ; Chun Choo KIM

Korean Journal of Hematology.2002;37(2):106-113.

BACKGROUND: Graft rejection as well as graft versus host disease is the main cause of failure after allogeneic hematopoietic stem cell transplantation (HSCT) in patient with severe aplastic anemia. Graft rejection is associated with multiple transfusions before transplant. We added procarbazine (PCB) to the cyclophosphamide (CY) and antithymocyte globulin (ATG) conditioning regimen to overcome rejection since a synergistic immunuosuppressive effect between alkylating agents and ATG had been reported. In heavily transfused patients, G-CSF primed bone marrow+T cell depleted peripheral blood stem cell was used as the source of stem cells. Here we report the outcome in severe aplastic anemia patients receiving the intensified pretransplant conditioning and high dose stem cells to overcome graft rejection in these high risk patients. METHODS: Between January 1995 and December 2000, among 113 patients with severe aplastic anemia who underwent allogeneic- HSCT after conditioning with CY+ATG+PCB, 90 patients were enrolled and divided into three groups. Thirty eight patients who received a small amout of transfusion were transplanted with bone marrow stem cells alone (group 1). In heavily transfused patients, 32 patients transplanted with bone marrow stem cells alone (group 2), and 20 patients transplanted with high dose stem cells (group 3). We evaluated the effect and outcome of a high dose stem cell transplantation, retrospectively. RESULTS: The median age of all patients was 29 years (range 16 to 50) and median follow up duration was 30 months (range 1 to 80). Male to female ratio was 61 : 52. Six-year estimated overall survival of all patients was 88.4%. Sixteen patients (14.1%) experienced in graft rejection. A significant statistical significancy was observed with result that the lowest rejection incidence (5%) was seen in group 3, compared with 28.2% in group 2 (P= 0.02) and 13.1% in group 1. The incidence of acute and chronic graft versus host disease among patients with sustained engraftment was respectively 9.4%, 15.6% in group 2, and 20%, 20% in group 3 (P=0.4, P=0.78). Six- year estimated overall survival was 78% in group 2, and 90% in group 3 (P=0.24). CONCLUSION: These results suggest that allogeneic-HSCT with high dose stem cells is an effective treatment to overcome graft rejection in heavily transfused severe aplastic anemia patients
Alkylating Agents ; Anemia, Aplastic* ; Antilymphocyte Serum ; Bone Marrow ; Cyclophosphamide ; Female ; Follow-Up Studies ; Graft Rejection ; Graft vs Host Disease ; Granulocyte Colony-Stimulating Factor ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Incidence ; Male ; Procarbazine ; Retrospective Studies ; Stem Cell Transplantation ; Stem Cells*

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The Treatment of Adult Acute Myelogenous Leukemia with AD Induction Therapy Followed by Consolidation Therapy with Intermediate Dose Ara-C; Long Term Follow-up Results.

Dae Ho LEE ; Soo Mee BANG ; Joo Young JUNG ; Jin Seok AHN ; Chul Won JUNG ; Kyung Hae JUNG ; Sung Soo YOON ; Myung Don OH ; Seonyang PARK ; Byoung Kook KIM ; Kang Won CHOE ; Han Ik CHO ; Noe Kyeong KIM

Korean Journal of Hematology.2002;37(2):97-105.

BACKGROUND: This study was to evaluate the therapeutic efficacy of consolidation therapy based on intermediate dose Ara-C in patients with newly diagnosed acute myelogenous leukemia (AML) in Seoul National University Hospital. And also, this study was to assess the toxicities of the treatment. METHODS: We have reviewed retrospectively our experience of patients with newly diagnosed non-M3 AML between January 1993 and July 1997. They were treated with induction chemotherapy with Ara-C 200mg/m2/d over 24 h for 7 days and daunorubicin 45mg/m2/d daily for 3 days. The patients achieving complete remission (CR) are to receive the 3 courses of consolidation chemotherapy based on intermediate dose of Ara-C 1,000mg/m2 given over 2h every 12 h for a total of eight to ten doses. Patients having HLA-matched sibling donors with informed consent could receive allogeneic bone marrow transplantation (BMT). RESULTS: One hundred and fifteen patients were reviewed. The median age was 41 years (range, 16-69) and median follow-up was 75 months. The CR rate was 72.2%. The median disease-free survival (DFS) of patients receiving consolidation therapy and allogeneic BMT was 21 months and 26.5 months, respectively. The overall survival (OS) was 13 months for patients not-receiving consolidation therapy, 21 months for consolidation therapy, and 31 months for allogeneic BMT, respectively. The rate of treatment-related mortality of consolidation therapy was 14% and cause of all deaths was infection. But in allogeneic BMT, that mortality rate was 42%; 2 infections, 2 veno-occlusive diseases and 1 cyclophosphamide-induced cardiomyopathy. CONCLUSION: Patients receiving consolidation therapy with intermediate dose Ara-C had longer DFS and OS. But their DFS and OS was not superior to that of patients receiving allogeneic BMT. In addition, that result was inferior to that of patients receiving high dose Ara-C based consolidation therapy, compared with other previous studies. However, this study was retrospective and so further prospective study will be required for comparing different doses of Ara-C consolidation therapy versus BMT.
Adult* ; Bone Marrow Transplantation ; Cardiomyopathies ; Consolidation Chemotherapy ; Cytarabine* ; Daunorubicin ; Disease-Free Survival ; Follow-Up Studies* ; Humans ; Induction Chemotherapy ; Informed Consent ; Leukemia, Myeloid, Acute* ; Mortality ; Retrospective Studies ; Seoul ; Siblings ; Tissue Donors

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Clinical Significance of the Chromosomal Abnormalities and Immunophenotype in Acute Myeloid Leukemia.

Hyuk Chan KWON

Korean Journal of Hematology.2002;37(2):89-96.

BACKGROUND: Acute myeloid leukemia (AML) is a hematologic malignant disease characterized by an uncontrolled proliferation of myeloid cells in marrow and arrest in their maturation. Immunophenotyping is a widely used method to diagnose and classify leukemia, and cytogenetic studies can help providing the clues of disease progression and monitoring remission status after chemotherapy. METHODS: From August 1997 to July 2000, 68 patients with AML were treated with ara-C and idarubicin for remission induction, then followed by consolidation therapy. A panel of monoclonal antibodies (CD5, CD7, CD10, CD19, CD22, CD14, CD13, CD33, HLA-DR) were used to characterize immunologic phenotypes by flow cytometry. Chromosomal abnormalities by high resolution banding technique were evaluated. Patients were divided into three groups (favorable : A, intermediate : B, unfavorable : C). RESULTS: The incidence of chromosomal abnormalities was 57% (39/68), and the proportion of patients per groups were 21% (14/68) for group A, 57% (39/68) for group B, and 22% (15/68) for group C. The median follow- up duration of the 68 evaluable patients was 12.7 months. The complete remission (CR) rate was 63.2% (43/68). The CR rate in group A, B, and C were 92.9% (13/14), 66.7% (26/39) and 26.7% (4/15), respectively (P=0.005). The AML patients who expressed CD19 (P=0.048) or did not express CD14 (P=0.013) had better CR than other groups. The median leukemia free survival duration of group A and B were 39.5 months and 11.9 months, respectively, and the leukemia free survival duration of group C has not been reached at median value (P=0.038). The median overall survival duration was 12.3 months, the survival duration of group A has not been reached at median value, and the median survival duration of group B and C were 12.0 months and 1.5 months, respectively (P=0.001). The AML patients without CD7 (P=0.029), without CD14 (P=0.023), or with CD19 (P=0.047) showed a better survival than other groups. In multivariate analysis, karyotype proved to be a significant prognostic factor (P=0.018). CONCLUSION: This study demonstrated that karyotype was an important prognostic factor in AML patients. Among surface marker expressions, CD7, CD14, CD19 may be used as a prognostic factor. Further study will be needed to confirm the role of immunophenotyping in AML.
Antibodies, Monoclonal ; Bone Marrow ; Chromosome Aberrations* ; Cytarabine ; Cytogenetics ; Disease Progression ; Drug Therapy ; Flow Cytometry ; Humans ; Idarubicin ; Immunophenotyping ; Incidence ; Karyotype ; Leukemia ; Leukemia, Myeloid, Acute* ; Multivariate Analysis ; Myeloid Cells ; Phenotype ; Remission Induction

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