Chronic myeloid leukemia (CML) is a malignant disease induced by the oncogenic signal of the BCRABL transcript resulting from the translocation between chromosome 9 and 22, t (9:22). This genetic alteration evoked the development of imatinib, a tyrosine kinas inhibitor (TKI) targeting BCR-ABL tyrosine kinase. This drug showed higher activity with durable response compared with conventional interferon therapy and became a standard therapy for newly diagnosed CML patients. Dasatinib and nilotinib, the next generation TKIs are used for patients with chronic phase CML as first line therapy as well after finding that these drugs exert faster and deeper response than imatinib. Resistance and intolerance to BCR-ABL TKIs are the obstacles to managing patients. Substantial new drugs are developed for targeting mutations resistant to BCR-ABL TKIs. More concern is paid to long-term management of patients showing complications when taking these drugs, and eventually stopping drugs in selected patient populations are being evaluated.
Benzamides ; Chromosomes, Human, Pair 9 ; Fusion Proteins, bcr-abl ; Humans ; Interferons ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Piperazines ; Protein Kinase Inhibitors ; Protein-Tyrosine Kinases ; Pyrimidines ; Thiazoles ; Tyrosine ; Dasatinib ; Imatinib Mesylate

No abstract available.
Pain Management

Iron overload is frequently found in transfused patients with chronic anemia and congenital hemochromatosis. Iron toxicity adversely affects organs, including the heart, liver, and endocrine glands. Iron chelation helps patients with transfusion-related iron overload improve organ dysfunction and prolong survival.Current Concepts: Iron overload is classified into primary and secondary causes. Acquired causes of secondary iron overload include ineffective erythropoiesis, chronic transfusion, and chronic liver diseases. The effectiveness of iron chelation therapy in reducing or maintaining body iron has been demonstrated in many studies of patients with transfusion-induced anemias. Deferasirox, a once-daily oral iron chelator for adult and pediatric patients with transfusion-induced iron overload, is available in Korea. The initial daily dose of deferasirox recommended by Korean guidelines is 20 mg/kg body weight, taken on an empty stomach at least 30 minutes before meals. Serum ferritin levels should be maintained below 1,000 ng/mL.Discussion and Conclusion: Iron chelation therapy should be considered when a patient has undergone large red blood cell transfusions, and there is evidence of organ iron overload to improve organ dysfunction and survival.

To determine whether the p53 expression might be a predictor for treatment sponse and overall survival in nodal non-Hodgkin's lymphoma (NHL), we analyzed e expression of p53 in 69 NHL patients. p53 protein expression was analyzed by munohistochemistry with long-term follow up (1-148 months: median 12.2). p53 pression was noted in 23/69 (33.3%) patients. Complete response (CR) rate to stemic chemotherapy was correlated with stage (I/II) (p=0.038), but not with 3 expression (p=0.2856). Poor overall survival was associated with stage =0.0010) or IPI score (p=0.0076), but not with p53 expression (p=0.8601). From ratification analysis by stage, in stage III/IV patients, the p53 positive oup had a trend to be associated with poor overall survival than the p53 gative group. Multivariate analysis revealed that p53 positive group was sociated with less CR rate compared to the p53 negative group (p=0.046), ereas overall survival was correlated with stage (p=0.0320), not with p53 atus. p53 expression was associated with less CR rate in patients with DLBL. rther studies with large numbers of samples and homogenous group of NHL are eded to determine the prognostic value of cell cycle regulator, p53 in NHL.
Antibodies, Monoclonal ; Cell Cycle Proteins/biosynthesis ; Female ; Gene Expression ; Human ; Immunohistochemistry ; Immunophenotyping ; Lymph Nodes/pathology* ; Lymph Nodes/metabolism* ; Lymphoma, Non-Hodgkin/pathology* ; Lymphoma, Non-Hodgkin/metabolism* ; Lymphoma, Non-Hodgkin/genetics ; Lymphoma, Non-Hodgkin/drug therapy ; Male ; Middle Age/Mpartment of Microbiology ; Prognosis ; Protein p53/immunology ; Protein p53/genetics ; Protein p53/biosynthesis*

Primary lymphoma of the central nervous system is a rare disease, occurring spontaneously and/or in conjunction with immunosuppressive state. Its incidence is increasing according to the increment of organ transplantation and AIDS. Recently we experienced a case of primary lymphoma occurred in central nervous system after renal transplantation in a 58-year-old women who had complained of persistent headache and left hemiparesis. CT scan of the brain showed two hyperdense mass lesions in right frontal and right basal ganglia areas. Immunohistochemical stain of the excised mass lesion revealed that tumor cells were derived from B cells. The patient was treated with discontinuance of immunosuppressive drug and irradiation, but expired due to pneumonia.
B-Lymphocytes* ; Basal Ganglia ; Brain ; Central Nervous System ; Female ; Headache ; Humans ; Immunosuppression ; Incidence ; Kidney Transplantation* ; Lymphoma ; Lymphoma, B-Cell* ; Middle Aged ; Nervous System* ; Organ Transplantation ; Paresis ; Pneumonia ; Rare Diseases ; Tomography, X-Ray Computed ; Transplants

BACKGROUND: Ionizing radiation is a group 1 carcinogen according to the IARC(International Agency for Research on Cancer) classification. With the development of the radiation related industry, the number of radiation exposed workers has been increasing. There have been several reports on AML(Acute Myeloid Leukemia) on exposure to ionizing radiation; however, there are no reports of occupational malignant lymphohematopoietic disease related to non-destructive inspection. CASE REPORT 1: A 35-years-old male, who had worked for 10 years in non-destructive inspection, was diagnosed with myelodysplastic syndrome. He worked 8 hours a day, for three weeks per months, where he was exposed to 192Ir and 60Co radiation sources. Because he had not worn a film badge for monitoring his radiation exposure dose, the accurate exposure dose was not reported. The estimate exposure dose calculated via a chromosomal study was 1.20 Gy, which exceed the dose limits of Korean radiation dose standards, which are 50 and 100 mSv annually and quinquennially respectively. CASE REPORT 2: A 26-years-old male, who had worked for 2.5 years in the same company was also diagnosed with myelodysplastic syndrome. CONCLUSION: Non-destructive inspection is the main source of ionizing radiation in the workplace, which could be the cause of malignant lymphohematopoietic diseases. Therefore, more practical plans and guidelines are needed to prevent non-destructive inspectors from workplace radiation exposure.
Film Dosimetry ; Humans ; Leukemia ; Leukemia, Erythroblastic, Acute ; Male ; Myelodysplastic Syndromes ; Radiation, Ionizing

Although eosinophilic fasciitis (EF) may precede hematologic malignancy or Hodgkin's disease, association with peripheral T-cell lymphoma (PTCL) is extremely rare. Only four cases of EF preceding or concomitant PTCL have been reported in the world literature. We experienced the first Korean case of EF complicated by the later relapse of peripheral T-cell lymphoma. A 63-year-old Korean male has been followed at our outpatient clinic periodically after treatment for stage IV PTCL. He had been in complete remission for seven and a half years when he developed edema of both lower extremities followed by sclerodermatous skin change in both hands with peripheral eosinophilia. Biopsy from the left hand showed fibrous thickening of the fascia with lymphoplasmacytic and eosinophilic infiltrate, consistent with EF. Twenty-five months later, a newly developed lymph node from the left neck showed recurrence of PTCL. EF may occur as a paraneoplastic syndrome associated with the relapse of PTCL. Therefore, in a patient with EF, the possibility of coexisting and/or future occurrence of hematologic neoplasm should be considered.
Case Report ; Eosinophilia/pathology ; Eosinophilia/complications* ; Fasciitis/pathology ; Fasciitis/complications* ; Human ; Lymphoma, T-Cell, Peripheral/pathology ; Lymphoma, T-Cell, Peripheral/complications* ; Male ; Middle Age ; Recurrence

Pure red cell aplasia (PRCA) in adults is usually idiopathic, although some underlying conditions can cause PRCA. Immunosuppressive therapy (IST) is used to treat PRCA, but IST has side effects and may fail. The anti-CD52 monoclonal antibody alemtuzumab (ALM) was recently used to successfully treat therapy-resistant PRCA. We herein report successful treatment of secondary PRCA after erythropoietin therapy using ALM and cyclosporin A (CsA) in one patient. The total dose of ALM was 60 mg over 3 days (10, 20, and 30 mg, respectively) plus CsA for at least 6 months. The patient achieved a complete response 18 months after ALM-CsA treatment and his treatment could be changed to a different erythropoietin-stimulating agent.
Adult ; Antibodies, Monoclonal, Humanized ; Cyclosporine ; Erythropoietin ; Humans ; Red-Cell Aplasia, Pure

Extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) is usually indolent. Although it was reported recently that about 20-30% cases of MALT lymphoma presented with a disseminated disease at diagnosis, it was described as a disease localized at diagnosis and remaining stable for a prolonged period. However, only a few cases of MALT lymphoma involved the lung and gastrointestinal tract all at once. We report a case of a 73-year-old man with disseminated MALT lymphoma. He presented with non-productive cough, initial chest radiograph showed a nodule in the right lower lobe. The diagnosis of stage IV MALT lymphoma was made by CT scan, video-assisted thoracoscopic excisional biopy, gastrofiberscopic biopsy and bone marrow biopsy. The lymphoma involved the lung, stomach and bone marrow at the time of diagnosis. Because he refused chemotherapy, he discharged after Helicobacter pylori eradication without chemotherapy. Regular follow-up examination did not show any evidence of disease progression over 22 months.
Aged ; Biopsy ; Bone Marrow ; Cough ; Diagnosis ; Disease Progression ; Drug Therapy ; Follow-Up Studies ; Gastrointestinal Tract ; Helicobacter pylori ; Humans ; Lung ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Radiography, Thoracic ; Solitary Pulmonary Nodule* ; Stomach ; Tomography, X-Ray Computed

Anorectum is a rare location for malignant lymphoma. Involvement of is rare even for the lymphoma associated with acquired immune deficiency syndrome (AIDS), and AIDS has a relatively increased frequency of anorectal lymphoma. Most lymphomas in AIDS patients are of a B-cell origin, and T-cell lymphoma of the gastrointestinal tract is extremely rare. We report here on a case of anorectal and gastric peripheral T-cell lymphoma, unspecified (PTCLu) in a non-AIDS patient. A previously healthy 29-year-old man presented with hematochezia and tenesmus that he had suffered with for the previous 2 months. Sigmoidoscopy showed anal and rectal submucosal tumor. Multiple round-shaped, flat and elevated lesions were noted on the gastric antrum and body as well. He underwent excisional biopsy for the anal mass and the diagnosis was PTCLu. Biopsies of the gastric lesions gave the same diagnosis. There was no lymphoma involved in the bone marrow. At admission, no antibodies against human immunodeficiency virus were detected. He underwent systemic chemotherapy and upfront autologous stem cell transplantation.
Tomography, X-Ray Computed ; Stomach Neoplasms/*pathology/radiography ; Sigmoidoscopy ; Rectal Neoplasms/*pathology/radiography ; Male ; Lymphoma, T-Cell, Peripheral/*pathology/radiography ; Humans ; Gastroscopy ; Follow-Up Studies ; Diagnosis, Differential ; Biopsy ; Adult ; Acquired Immunodeficiency Syndrome/*diagnosis