Long-term follow-up results of cytarabine-containing chemotherapy for acute promyelocytic leukemia

Background/Aims@#We evaluated the feasibility and long-term efficacy of the combination of cytarabine, idarubicin, and all-trans retinoic acid (ATRA) for treating patients with newly diagnosed acute promyelocytic leukemia (APL). @*Methods@#We included 87 patients with newly diagnosed acute myeloid leukemia and a t(15;17) or promyelocytic leukemia/retinoic acid receptor alpha (PML-RARα) mutation. Patients received 12 mg/m2/day idarubicin intravenously for 3 days and 100 mg/m2/day cytarabine for 7 days, plus 45 mg/m2/day ATRA. Clinical outcomes included complete remission (CR), relapse-free survival (RFS), overall survival (OS), and the secondary malignancy incidence during a 20-year follow-up. @*Results@#The CR, 10-year RFS, and 10-year OS rates were 89.7%, 94.1%, and 73.8%, respectively, for all patients. The 10-year OS rate was 100% for patients that achieved CR. Subjects were classified according to the white blood cell (WBC) count in peripheral blood at diagnosis (low-risk, WBC < 10,000/mm3; high-risk, WBC ≥ 10,000/mm3). The low-risk group had significantly higher RFS and OS rates than the high-risk group, but the outcomes were not superior to the current standard treatment (arsenic trioxide plus ATRA). Toxicities were similar to those observed with anthracycline plus ATRA, and higher than those observed with arsenic trioxide plus ATRA. The secondary malignancy incidence after APL treatment was 2.7%, among the 75 patients that achieved CR, and 5.0% among the 40 patients that survived more than 5 years after the APL diagnosis. @*Conclusions@#Adding cytarabine to anthracycline plus ATRA was not inferior to anthracycline plus ATRA alone, but it was not comparable to arsenic trioxide plus ATRA. The probability of secondary malignancy was low.

Rare Presentation of Richter's Transformation to Diffuse Large B Cell Lymphoma: a Case Report / 대한내과학회지

Richter's syndrome refers to the development of aggressive lymphoma in a patient with chronic lymphocytic leukemia (CLL). It occurs in about 2% to 10% of patients with CLL. The most frequent manifestation of Richter's syndrome is diffuse large B cell lymphoma (DLBCL). Extranodal involvement is rare but can occur. The prognosis of Richter's syndrome is very poor. We herein report a case of a rare presentation of Richter's syndrome. A 42-year-old man diagnosed with CLL 2 years previously developed nodules on the bilateral thighs and buttocks. A positron emission tomography (PET)-CT scan revealed high fluorodeoxyglucose uptake in multiple lymph nodes, skeletal muscles, and the myocardium. An ultrasonography-guided biopsy confirmed Richter's syndrome from CLL to DLBCL. The patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. After six cycles of chemotherapy, we performed a PET-CT scan that revealed a complete response. However, 3 months later, the syndrome recurred. The patient was undergoing salvage chemotherapy at the time of this writing.

Immune thrombocytopenia associated with sarcoidosis / 영남의대학술지

Sarcoidosis is a systemic disease of unknown cause involving multiple organs and is characterized by noncaseating granuloma. Immune thrombocytopenia (ITP) is an autoimmune disease characterized by increased peripheral platelet destruction due to the presence of an antibody to the platelet and abnormal platelet production. There is no known pathogenesis that occurs concurrently with ITP and sarcoidosis. However, considering together of 2 known pathogenesis, abnormal immune response triggers either ITP or sarcoidosis. The disease that develops first stimulates secondary disease. After development of secondary disease, they stimulate each other. A few cases of ITP associated with sarcoidosis are well documented in English; however, the disease has rarely been reported in Korea. Here, we report on a case of ITP with sarcoidosis in a 29-year-old man. He suffered from easy bruising. The chest X-ray and the contrast-enhanced computed tomography scan showed bihilar lymphadenopathy and reticulonodular infiltrates. Bone marrow study and fluoroscopy-guided percutaneous needle biopsy were performed and the patient was diagnosed with sarcoidosis and ITP. He was put on 400 mg/kg of intravenous immunoglobulin for 5 days and administered oral steroids and further follow-up will be carried out. He has shown a good response without significant bleeding event. However, administration of more oral steroid and additional follow-up is required than for single disease, whether sarcoidosis or ITP.

Chronic myeloid leukemia as a secondary malignancy after diffuse large B-cell lymphoma

No abstract available.

Recurrent acinic cell carcinoma in the parotid gland with cardiac metastasis and hypertrophic osteoarthropathy / 영남의대학술지

Acinic cell carcinoma (ACC) is an uncommon malignant tumor of the salivary glands that is difficult to diagnose. It grows slowly and shows distant metastasis rarely. We experienced a case of recurrent ACC in the parotid gland with cardiac metastasis and hypertrophic osteoarthropathy. The 29-year-old man had been suffering from severe multiple bones and joints pain for 2 months. Ten years earlier, he underwent superficial parotidectomy due to a right subauricular mass. The mass was diagnosed with ACC. After surgery, the tumor recurred twice. Then the patient was diagnosed with cardiac metastasis via positron emission tomography-computed tomography and trans-thoracic echocardiography. He also had hypertrophic osteoarthropathy with multiple bone metastasis. He was given palliative radiotherapy and conservative treatment. ACC in the parotid gland with cardiac metastasis and hypertrophic osteoarthropathy has not yet been reported in literature. From this case, it is recommended to evaluate multiple distant metastasis in the ACC of the parotid gland when joint and bone pain are present.

A Case of Type 1 Neurofibromatosis Associated with Multiple Metastatic Gastrointestinal Stromal Tumors / 영남의대학술지

Type 1 neurofibromatosis (von Recklinghausen's disease, NF-1) is an autosomal-dominant neurocutaneous-disorder characterized by systemic cafe'-au-lait spots, multiple cutaneous neurofibromas, axillary or inguinal freckling, and Lisch nodules (pigmented iris hamartomas). Approximately 10-25% of NF1 patients have gastrointestinal neoplasms. Gastrointestinal stromal tumor (GIST) in patients with neurofibromatosis is most commonly found in the small bowel and the stomach, and approximately 60% of such patients have multiple tumors or multiple tumor sites. Although, the increased incidence of GIST in patients with neurofibromatosis is well documented in pathology literature in English, but has rarely been documented in Korea. Here, we report a case of multiple GISTs in a 48-year-old woman accompanied by NF1. She was admitted to Yeungnam University Hospital with complaints of melena and dyspnea. A contrast-enhanced computed tomography (CT) scan revealed that multiple soft tissue masses were occupying the entire peritoneal cavity. An ultrasonogram- guided biopsy was performed and the tumors were found to have been composed of tumor cells that were positive for c-kit protein. The patient was put on Imatinib mesylate treatment, and further follow-up will be carried out.

Interstitial Lung Disease in a Patient with Dyskeratosis Congenita / 결핵및호흡기질환

Dyskeratosis congenita is a rare congenital disorder characterized by a triad of reticular pigmentation of the skin, dystrophic nails, and leukoplakia of the mucous membrane. Sometimes it is associated with bone marrow failure, secondary malignancy and interstitial lung disease. Though it is rare, Dyskeratosis congenita is diagnosed relatively easily when clinicians suspect it. It can be diagnosed just by gross inspection with care. Dyskeratosis congenita should be considered as one cause associated with interstitial lung disease. In Korea, interstitial lung disease with dyskeratosis congenita has not been reported. We report a case and review the literature.

Clinical Outcome of Rituximab-Based Therapy (RCHOP) in Diffuse Large B-Cell Lymphoma Patients with Bone Marrow Involvement / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: We investigated the clinical outcome of bone marrow (BM) involvement in patients with diffuse large B-cell lymphoma (DLBCL) who received rituximab-based therapy. MATERIALS AND METHODS: A total of 567 consecutive patients with newly diagnosed DLBCL treated with rituximab-CHOP (RCHOP) between November 2001 and March 2010 were included in the current study. All of the patients underwent a BM study at the initial staging and the clinical characteristics and prognosis of these patients with or without BM involvement were analyzed retrospectively. RESULTS: The total cohort included 567 patients. The overall incidence of BM involvement was 8.5%. With a median follow-up duration of 33.2 months (range, 0.1 to 80.7 months) for patients who were alive at the last follow-up, the five-year overall survival (OS) and event-free survival (EFS) rate in patients without BM involvement (76.3% and 67.5%, p<0.001) was statistically higher than that in patients with BM involvement (44.3% and 40.1%, p<0.001). In multivariate analysis, among total patients, BM involvement showed a significant association with OS and EFS. In univariate and multivariate analyses, even among stage IV patients, a significant association with worse EFS was observed in the BM involvement group. CONCLUSION: BM involvement at diagnosis affected the survival of patients with DLBCL who received RCHOP. Although use of RCHOP can result in significant improvement of the therapeutic effect of DLBCL, BM involvement is still a negative prognostic factor of DLBCL patients in the era of rituximab.

Simultaneous Actinomycosis with Mucormycosis in Maxillary Sinus / 영남의대학술지

Actinomycosis is a rare subacute-to-chronic infection that causes sinus fistula, tract, or abscess due to the invasion surrounding the soft tissue. Actinomyces colonize the mouth, colon, and vagina. Mucosal disruption may lead to infection at virtually any site in the body. Cervicofacial infection accounts for 50-60% of all actinomycosis cases. The mandible and nasopharynx are the sites of predilection, but maxillary sinus infection is rare. Reported herein is a case involving a 57-year-old female with acute myeloid leukemia who had simultaneous actinomycosis with mucormycosis in the maxillary sinus.

Long-Term Complete Remission in an Acute Myeloid Leukemia Patient with Isolated Central Nervous System Relapse after Allogeneic Hematopoietic Stem Cell Transplantation / 영남의대학술지

Allogeneic hematopoietic stem cell transplantation (HSCT) is considered the optimal curative treatment for acute myeloid leukemia (AML), but some patients develop bone marrow relapse due to remnant leukemia, and few patients develop extramedullary relapse without bone marrow relapse. Isolated extramedullary relapse (IMER) is defined as extramedullary relapse without bone marrow relapse. IMER has been reported in various sites, including the skin, soft tissue, and central nervous system(CNS). Isolated CNS relapse is relatively rare and is associated with poor prognosis due to the absence of an optimal treatment for it. Reported herein is a case involving an adult AML woman who suffered from isolated extramedullary relapse in the CNS after allogeneic HSCT. She was treated with intrathecal chemotherapy and whole-brain and spine radiotherapy, followed by systemic chemotherapy. She is currently well, with no evidence of leukemia recurrence for over six years.

Early or Late Gefitinib, Which is Better for Survival?: Retrospective Analysis of 228 Korean Patients with Advanced or Metastatic NSCLC / 영남의대학술지

BACKGROUND: The optimal timing of treatment with EGFR-tyrosine kinase inhibitors (EGFR-TKI) in NSCLC patients has not yet been determined. METHODS: We separated 228 patients with advanced/metastatic NSCLC treated with gefitinib into an early gefitinib group (patients who received gefitinib as first- or second-line treatment) and a delayed gefitinib group (patients who received gefitinib as third or fourth-line treatment) and attempted to determine whether the timing of gefitinib treatment affected clinical outcomes. RESULTS: Median overall survival (OS), progression free survival (PFS), and median OS from first-line treatment of advanced/metastatic disease (OSt) for 111 patients in the early gefitinib group were 6.2 months, 3.3 months, and 11.6 months. However, median OS, PFS, and OSt for 84 patients in the delayed gefitinib group were 7.8 months, 2.3 months, and 22.7 months. No differences in OS and PFS were observed between the 2 groups. However, OSt was significantly longer in the delayed gefitnib group. Timing of gefitinib therapy was one of the independent predictors of OSt. Hb > or = 10 g/dl, and having never smoked, and ECOG performance status < or =1 were independent predictors of better PFS. CONCLUSION: Deferral of gefitinib therapy in patients with advanced or metastatic NSCLC may be preferable if they are able to tolerate chemotherapy.

Prognostic Factors in Patients with Localized Soft Tissue Sarcoma / 대한내과학회지

BACKGROUND/AIMS: We evaluated the independent prognostic factors for overall and disease-free survival in the treatment of soft tissue sarcoma. METHODS: Sixty-seven medical records were retrospectively reviewed. All the patients had presented with localized soft tissue sarcoma and had been treated with conservative surgery, followed by additional therapy or surgery. All were treated at the Yeungnam University Hospital between January 2000 and December 2006. Univariate and multivariate analyses were used to evaluate factors affecting overall and disease-free survival. RESULTS: The median age of the study sample was 50 (range, 15~70) years. Twenty patients (30%) had liposarcoma and nine (13%) had leiomyosarcoma. Seventeen patients (25%) had a tumor measuring greater than 10 cm. Nineteen patients (28%) had positive resection margins. The median overall survival was 62.7 months (95% CI: 25.7~99.4) and the median disease-free survival was 36.1 months (95% CI: 17.9~54.2). Factors that significantly affected the median overall survival were a tumor size greater than 10 cm and performance status. Positive resection margins and tumor site were independent prognostic factors associated with diseasefree survival. Other factors, such as histopathology, gender, age, and tumor grade, did not have prognostic value. Additionally, the chosen modality of treatment after surgery was not an independent predictor of the median overall or disease-free survival. CONCLUSIONS: Tumor size, positive resection margins, tumor site, and performance status were statistically significant independent predictors of poor prognosis.

Treatment of Hemangiopericytoma-Associated Hypoglycemia with Glucocorticoid Therapy / 영남의대학술지

Non-islet cell tumor-induced hypoglycemia (NICTH) is associated with mesenchymal tumor types, including hemangiopericytoma, fibrosarcoma, mesothelioma, and neurofibroma, as well as carcinoma of the liver, adrenal glands, and kidneys. Non-islet cell tumors induce hypoglycemia by overproducing an abnormal form of insulin-like growth factor II (IGF II). Complete removal of the tumor or reduction of the tumor mass is a successful therapeutic strategy in cases of NICTH. However, if the tumor re-grows, curative resection is nearly impossible, and hypoglycemia occurs repeatedly. Glucocorticoids are effective in terms of long-term relief from hypoglycemia through promotion of gluconeogenesis in the liver, tumor suppression, production of 'big'-IGF-II, and correction of the attendant biochemical abnormalities involving the growth hormone (GH)-IGF axis. We found that administration of corticosteroid therapy to a patient suffering from NICTH resulted in improvement of hypoglycemia associated symptoms.

Paratesticular Alveolar Rhabdomyosarcoma with Multiple Lymph Nodes Metastasis Successfully Treated with Chemotherapy / 영남의대학술지

Rhabdomyosarcomas are soft tissue sarcomas; while extremely rare in adults, they are one of the most common neoplasms in children and adolescents. Histologically, they can be classified into embryonal(ERMS), alveolar(ARMS), pleomorphic, and undifferentiated types. The ARMS type is very rare, and is associated with a poor prognosis. Common primary sites of ARMS are the trunk and extremities. We report on a case of paraaortic, supraclavicular, and axillary lymph node metastasis from paratesticular ARMS treated with VAC(vincristine, dactinomycin, cyclophosphamide)/ IE(ifosfamide, etoposide) chemotherapy in a young adult. Administration of six cycles of chemotherapy with VAC/ IE resulted in complete remission. The patient has maintained complete remission over the past 27 months.

A Novel Missense Mutation Asp506Gly in Exon 13 of the F11 Gene in an Asymptomatic Korean Woman with Mild Factor XI Deficiency / 대한진단검사의학회지

Factor XI (FXI) deficiency is a rare autosomal recessive coagulation disorder most commonly found in Ashkenazi and Iraqi Jews, but it is also found in other ethnic groups. It is a trauma or surgery-related bleeding disorder, but spontaneous bleeding is rarely seen. The clinical manifestation of bleeding in FXI deficiency cases is variable and seems to poorly correlate with plasma FXI levels. The molecular pathology of FXI deficiency is mutation in the F11 gene on the chromosome band 4q35. We report a novel mutation of the F11 gene in an 18-year-old asymptomatic Korean woman with mild FXI deficiency. Pre-operative laboratory screen tests for lipoma on her back revealed slightly prolonged activated partial thromboplastin time (45.2 sec; reference range, 23.2-39.4 sec). Her FXI activity (35%) was slightly lower than the normal FXI activity (reference range, 50-150%). Direct sequence analysis of the F11 gene revealed a heterozygous A to G substitution in nucleotide 1517 (c.1517A>G) of exon 13, resulting in the substitution of aspartic acid with glycine in codon 506 (p.Asp506Gly). To the best of our knowledge, the Asp506Gly is a novel missense mutation, and this is the first genetically confirmed case of mild FXI deficiency in Korea.

Comparative Study on the Infection Rates of Protected Environment versus Non-Protected Environment in Acute Myeloid Leukemia during Remission Induction Chemotherapy / 영남의대학술지

BACKGROUND AND PURPOSE: Patients with acute leukemia experience prolonged periods of neutropenia due to their disease or its treatment. For this reason, they often develop serious infectious complications. Although antibiotic therapy has improved in recent years, the fatality rate from infection remains high. For the control of infection, protected environment was developed. But because of economic issue, most of chemotherapy with acute myeloid leukemia have conducted in non-protected environment. So this study compared the rate of complete remission, days with neutropenia, rate of fever, rate of positive culture, rate of overt infection and use of antibacterial and antifungal agents with patients within non-protected environment and protected environment, retrospectively. Patients with acute myeloid leukemia during first remission induction chemotherapy were eligible for this study. METHODS: Retrospective analysis was conducted between patients in non-protected (25 patients) and protected environment (14 patients) with acute myeloid leukemia during remission induction chemotherapy. RESULTS: Rate of overt infection, rate of fever, rate of positive culture and rate of use of antibiotics were significantly high in patients within non-protected environment compared with patients within protected environment. There were no differences in rate of complete remission and days of neutropenia. CONCLUSIONS: This study suggests protected environment for patients with acute myeloid leukemia during remission induction chemotherapy could reduce rate of overt infection, and rate of use of antibiotics.

A Case of Spine Origin Chondroblastoma Metastasis to Lung / Journal of the Korean Cancer Association, 대한암학회지

Chondroblastoma is a rare benign cartilaginous neoplasm that accounts for approximately 1% of all bone tumors and characteristically arises in the epiphysis of a long bone, particularly the humerus, tibia, and femur. Chondroblastoma can affect people of all ages. It is, however, most common in children and young adults between the ages of 10 and 20 years. Although most chondroblastomas are cured by limited surgical procedures, occasional lesions behave more aggressively and may even metastasis. In this case a young man with pulmonary metastatic chondroblastoma on spine is presented. Unlike previously published examples of metastatic chondroblastoma, these metastasis developed before any operative manipulation of the primary tumor. And primary tumor site was also unusual. The histologic characteristics of the primary, metastatic tumors were those of a conventional chondroblastoma.

Inpatient consultations by the hematology department over a 1-year period / 대한내과학회지

BACKGROUND/AIMS: Although inpatient hematology consultations have recently increased, the reasons for consultation, as well as the impact of inpatient hematologic consultations, are not clear. Therefore, we assessed patient profiles, the reasons for hematology consultations, and diagnoses at our hospital. METHODS: We evaluated 177 consecutive patients with hematology problems seen between March 2004 and February 2005. For each, we assessed the reason for the consultation, patient diagnosis, and outcome. RESULTS: Of 28,854 inpatients in our hospital, 177 (0.6%) required consultations for hematology problems. Of these 177 inpatients, 85 (48%) were male and 92 (52%) were female; their median age was 55 years (range 16-85 years), with the most frequent age group being patients 61-70 years old. Hematology consultations were requested by the neurosurgery (25%), general surgery (11%), internal medicine (10%), neurology (10%), and orthopedics (9%) departments. The most common reasons for consultation were anemia (29%), thrombocytopenia (21%), pancytopenia/bicytopenia (16%), coagulation abnormalities (14%), and thrombocytosis (5%). In one-third of these consultations, the hematologic abnormality was detected after admission and most were resolved successfully. The most common cause of anemia was iron deficiency (43%). Drug-induced thrombocytopenia and pancytopenia were also common (20%), and were thought to be due to anticonvulsants and antibiotics. Bone marrow examination was required in 19 patients (11%). Careful examination detected bleeding of the gastrointestinal tract, or liver or hematologic diseases, such as chronic myeloproliferative disorders, in some of these patients. CONCLUSIONS: Although it provides a small cross-sectional survey of the types of patient and problems seen by hematologists during in-patient consultations, this information may help to improve patient care and the quality of hematology consultations.

F-18 FDG PET/CT Findings of Subcutaneous Panniculitis - Like T- Cell Lymphoma : A Case Report / 핵의학분자영상

F-18 FDG PET is a metabolic imaging modality that is efficacious in staging and assessment of treatment response for variety of lymphomas. We report usefulness of F-18 FDG PET/CT in evaluating severity of the disease and response to therapy in a patient with subcutaneous panniculitis- like T-cell lymphoma (SPTCL). Here we describe a case of SPTCL in 24-year-old man who had wide spread firm and tender nodular lesions with increased F-18 FDG uptake. After chemotherapy follow up F-18 FDG PET/CT image shows disseminated malignancy and then the patient died with hemophagocytic syndrome. This report suggests that F-18 FDG PET/CT may be useful in determining disease activity at the time of initial diagnosis, after treatment, and evaluating a suspected outcome of SPTCL.

A case of posttransplantation lymphoproliferative disorder in hematopoietic stem cell transplantation / 대한내과학회지

Posttransplantation lymphoproliferative disorder (PTLD) is a potentially fatal complication of hematopoietic stem cell transplantation (HPSCT) that occurs relatively early after the transplantation. The occurrence of PTLD is usually associated with Epstein-Barr virus (EBV) infection in patients who are treated by aggressive immunosuppressive therapy. PTLD presents as diverse manifestations, ranging from reactive lymphoid hyperplasia to high grade malignant lymphoma. In the present work, we describe a case of PTLD that was successfully treated with R-CHOP (rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone) chemotherapy in a 21-year-old female who received HLA-matched sibling HPSCT for severe aplastic anemia. At 120 days after transplantation, she had symptoms of dysphagia and a foreign body sensation in the throat and was diagnosed as PTLD, diffuse large B cell lymphoma involving a tonsil, cervical lymph nodes, an adrenal gland, and the liver. Three courses of R-CHOP resulted in complete remission.