No abstract available.
Humans ; Multiple Myeloma ; Plasma Cells ; Plasma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

Recent advances in chemotherapy have led to increased survival rates for patients with hematologic malignancies. However, standard chemotherapies, including alkylating agents for non-Hodgkin lymphoma, could induce therapy-related myeloid neoplasms (t-MNs), a group of disorders categorized by the World Health Organization in 2008. Here, we report a case of coexistence of bone marrow (BM)-involved refractory marginal zone B-cell lymphoma (MZL) and therapy-related myelodysplastic syndrome (t-MDS). Simultaneous presence of refractory lymphoma and t-MN in the BM is rare, and this is the first report in Korea. The patient received allogeneic hematopoietic stem cell transplantation (HSCT) to cure both the MZL and t-MDS. Since the HSCT, he has been stable for 21 months without any evidence of recurrence.
Alkylating Agents ; Bone Marrow ; Drug Therapy ; Hematologic Neoplasms ; Hematopoietic Stem Cell Transplantation ; Humans ; Korea ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Lymphoma, Non-Hodgkin ; Myelodysplastic Syndromes* ; Recurrence ; Survival Rate ; World Health Organization

Mutations in the calreticulin gene, CALR, have recently been discovered in subsets of patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF). We investigated Korean patients with ET and PMF to determine the prevalence, and clinical and laboratory correlations of CALR/JAK2/MPL mutations. Among 84 ET patients, CALR mutations were detected in 23 (27.4%) and were associated with higher platelet counts (P=0.006) and lower leukocyte counts (P=0.035) than the JAK2 V617F mutation. Among 50 PMF patients, CALR mutations were detected in 11 (22.0%) and were also associated with higher platelet counts (P=0.035) and trended to a lower rate of cytogenetic abnormalities (P=0.059) than the JAK2 V617F mutation. By multivariate analysis, triple-negative status was associated with shorter overall survival (HR, 7.0; 95% CI, 1.6-31.1, P=0.01) and leukemia-free survival (HR, 6.3; 95% CI, 1.8-22.0, P=0.004) in patients with PMF. The type 1 mutation was the most common (61.1%) type among all patients with CALR mutations, and tended toward statistical predominance in PMF patients. All 3 mutations were mutually exclusive and were never detected in patients with other myeloid neoplasms showing thrombocytosis. CALR mutations characterize a distinct group of Korean ET and PMF patients. Triple-negative PMF patients in particular have an unfavorable prognosis, which supports the idea that triple-negative PMF is a molecularly high-risk disease.
Adult ; Aged ; Aged, 80 and over ; Calreticulin/*genetics ; Disease-Free Survival ; Female ; Gene Frequency ; Genetic Association Studies ; Humans ; Janus Kinase 2/*genetics ; Male ; Middle Aged ; Mutation/genetics ; Primary Myelofibrosis/*genetics/mortality ; Receptors, Thrombopoietin/*genetics ; Republic of Korea ; Thrombocythemia, Essential/*genetics/mortality ; Young Adult

BACKGROUND: The presence of significant dysplasia in bone marrow (BM) aspirates helps to distinguish between hypocellular myelodysplastic syndrome (hMDS) and aplastic anemia (AA). Occasionally, diluted BM aspirates make it difficult to recognize dysplastic changes and can also negatively affect the detection of cytogenetic abnormalities in hMDS. We evaluated the usefulness of CD34 and p53 immunoreactivity for discriminating between hMDS and AA and for estimating survival outcomes in hMDS patients. METHODS: BM clot section (BMC) or BM biopsy (BMB) specimens were obtained from 64 hMDS/AA patients (33 with hMDS and 31 with AA) and seven controls. Immunohistochemical (IHC) staining for CD34 and p53 was performed by using the EnVision detection system (Dako, Denmark). We compared the results of IHC staining, BM findings, and chromosomal analyses, and determined overall survival outcomes. RESULTS: The number of CD34- and p53-positive BM cells was higher among the patients with hMDS than among the patients with AA (P<0.001 and P=0.001, respectively). hMDS patients with increased CD34-positive cells had significantly poorer survival outcomes compared with those with normal number of CD34-positive cells (P=0.013). CONCLUSIONS: CD34 and p53 IHC stains of BMC or BMB provide useful information for differentiating between hMDS and AA. CD34 IHC staining of BMC or BMB also provides useful information for estimating survival outcomes in hMDS patients.
Adolescent ; Adult ; Anemia, Aplastic/*diagnosis ; Antigens, CD34/*metabolism ; Bone Marrow/metabolism/*pathology ; Child ; Chromosome Aberrations ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Myelodysplastic Syndromes/*diagnosis/mortality ; ROC Curve ; Tumor Suppressor Protein p53/*metabolism

Primary non-Hodgkin's lymphoma of bone (PLB) is rare, and generally presents as a single extensive and destructive bone lesion. Histopathologically, most cases present as diffuse large B-cell lymphoma, and T-cell lymphoma is rare. By contrast, multiple myeloma is a disease defined as the neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin. We report a case of multiple myeloma that developed during treatment of PLB in a type of T-cell. A 48-yr-old man was diagnosed as T-cell PLB, stage IE, 18 months ago. The patient received the chemoradiotherapy and salvage chemotherapy for PLB. However, the lymphoma progressed with generalized bone pain, and laboratory findings showed bicytopenia and acute renal failure. On bone marrow biopsy, the patient was diagnosed as having multiple myeloma newly developed with primary T-cell lymphoma of bone. In spite of chemotherapy, the patient died of renal failure.
Bone Neoplasms/*complications/diagnosis/therapy ; Fatal Outcome ; Humans ; Kidney Failure, Acute/etiology ; Lymphoma, T-Cell/*complications/diagnosis/therapy ; Male ; Middle Aged ; Multiple Myeloma/*complications/diagnosis/therapy

Dermatomyositis (DM) is an uncommon inflammatory myopathy with characteristic rash accompanying, or more often preceding, muscle weakness. There is a well-recognized association between DM and several cancers, such as ovarian cancer, breast cancer, melanoma, colon cancer, and non-Hodgkin lymphoma. We report the first case of cancer of unknown primary site associated with DM. A 62-yr-old woman presented to us with both shoulder painful swelling and facial edema. She was diagnosed previously as cancer of unknown primary site, histologically confirmed with squamous cell carcinoma in a pelvic mass. For the following days, she complained of erythematous face followed by progressive weakness of the proximal muscles of upper and lower limbs. The laboratory tests showed an increased muscle enzyme and acute phase reactants. The electromyogram showed the typical findings of DM. After the treatment with high dose steroid and methotrexate, the proximal motor weakness improved, and she received palliative radiation therapy.
Carcinoma, Squamous Cell/complications/diagnosis/pathology ; Dermatomyositis/*complications/diagnosis/pathology/therapy ; Female ; Humans ; Middle Aged ; Neoplasms, Unknown Primary/*complications/diagnosis/pathology

Essential thrombocythemia (ET) is a clonalmyeloproliferative disorder that can rarely transform into acute leukemia in 1~5% of cases. A recent study has found that a significant proportion of leukemic cases from ET were associated with a cytogenetic abnormality (17p deletion). Herein, we report two cases of acute myeloid leukemic transformations harboring a 17p abnormality from a series of 119 ET patients. The first case, a 48-year-old female, developed acute myeloid leukemia with maturation (AML-M2) accompanying myelodysplasia was diagnosed 6.1 years after the initial diagnosis of ET. She was treated with hydroxyurea. Her karyotype showed a monosomy 17. The second case, a 61-year-old male, developed acute megakaryoblastic leukemia (AML-M7) with a very complex hyperdiploidy including addition of 17p13 that developed 6.5 years after the initial diagnosis. He was treated with hydroxyurea and anagrelide. The immunohistochemistry showed p53 overexpression in both cases. Our cases support the specificity of chromosome 17 abnormality and p53 overexpression in acute leukemic transformation from ET.
Chromosome Aberrations ; Chromosomes, Human, Pair 17* ; Diagnosis ; Female ; Humans ; Hydroxyurea ; Immunohistochemistry ; Karyotype ; Leukemia ; Leukemia, Megakaryoblastic, Acute ; Leukemia, Myeloid, Acute ; Male ; Middle Aged ; Monosomy ; Sensitivity and Specificity ; Thrombocythemia, Essential*

Many investigators have reported that collagen gel contraction reflects the mechanism of wound contraction. In 1995, Tsai et al. reported that hypertrophic scar-derived fibroblasts in a connective tissue model possessed the greatest contraction potency when compared with those of normal skin and normal oral mucosa-derived CTMs. In this study, we studied the effect of collagen gel contraction by growth factors such as epidermal growth factor, platelet-derived growth factor, transforming growth factor-bata1, and transforming growth factor-bata3, Skin fibroblasts used in this study were obtained from the explant of rat skin culture. Fibroblasts were cultured in Dulbecco's modified Eagle's medium containing 10% fetal bovine serum. Growth factors were added per FPCL in the desired concentrations and we measured the collagen gel diameters in growth factor-treated FPCL on day 1,2,3, and 4 respectively after starting incubation. We examined the effects of EGF, PDGF, TGF-bata1, TGF-bata3 and the effects of combinations of TGF-bata1 + EGF, TGF-bata1 + PDGF, and TGF-bata1 + TGF-bata3 to contract a collagen gel. EGF has little influence on collagen gel contraction. TGF-bata1 and TGF-bata3 increase the collagen contraction. TGF-bata1 enhanced the contractility of collagen gel according to the concentrations. While TGF-bata3 alone had stimulatory contraction effects at low dose, high doses of TGF-bata3 decreased the potency of collagen gel contraction. A combination of TGF-bata1 and EGF minimally decrease TGF-bata1 activity. A combination of TGF-bata1and PDGF had an effect similar to TGF-bata1 activity. A combination of TGF-bata1 and TGF-bata3 decreased TGF-bata1 activity. According to reports that FPCL contraction is equivalent to the process of wound contraction, growth factors which enhance gel contraction may be related to wound contraction and wound healing. TGF-bata1 is reported to enhance scar formation in fetal wound. EGF accelerates wound healing and inhibits the promotion of hypertrophic scar formation. Compared to the effect of collagen gel contraction in this study, the combination of TGF-bata1 and TGF-bata3 that inhibited the promotion of collagen gel contraction are thought to diminish the formation of scar tissue. As well, EGF that has not enhanced collagen gel contraction is thought to diminish the production of scar tissue. We will study the interactive effects of TGF-bata3, EGF and TGF-bata1 on the contraction of collagen gels in the future.
Animals ; Cicatrix ; Cicatrix, Hypertrophic ; Collagen* ; Connective Tissue ; Epidermal Growth Factor ; Fibroblasts ; Gels ; Humans ; Intercellular Signaling Peptides and Proteins* ; Platelet-Derived Growth Factor ; Rats ; Research Personnel ; Skin ; Wound Healing ; Wounds and Injuries*

Sacral pressure sores have been treated by a variety of surgical methods. complete treatment needs wide excision and coverage with healthy tissue which has constant and sufficient blood supply. Use of gluteus maximus muscle flap with or without overlying skin is a revolutionary method because of the reliability of blood supply. However, it is technically a little bit complicated, and future reconstruction for recurrent decubitus is especially limited in paraplegic patients. The development of gluteal perforator-based flap with para-sacral perforator introduce a new treatment modality for the sacral pressure sores. Total 10 cases of sacral pressure sores were treated with gluteal perforator-based flap. There were minimal postoperative complications except wound dehiscence in one case. This flap has a many advantage of no transection or sacrifice of the gluteus maximus muscle, elevation time for the flap is short, reliable blood flow of the perforator, large rotation arc and no post-operative hindrance to walking in patients who are not paraplegic. The disadvantages of this perforator-based flaps are the anatomical variation in the location of perforators and the need for technically careful dissection.
Humans ; Postoperative Complications ; Pressure Ulcer* ; Skin ; Walking ; Wounds and Injuries

BACKGROUND: There has been some reports demonstrating the geographic heterogeneity in frequency of specific cytogenetic aberrations, but little is known about the types, frequency and prognostic value of specific chromosomal abnormalities in Korean adults with acute myelogenous leukemia. To evaluate cytogenetic characteristics of Korean adults with acute myelogenous leukemia, following study was performed. METHODS: Cytogenetic studies using high-resolution banding technique were done on newly diagnosed 38 patients with de nono acute myelogenous leukemia (AML) admitted to Asan medical center from January 1995 to May 1996. RESULTS: 1) Sixteen patients (42.1%) had clonal chromosomal abnormalities. 2) The t (8;21) was detected in 8 patients (21.1%). The t (15;17) was detected in 2 patients. The inv (16), del (11)(q23) and 7q- were detected in 1 patient each. Trisomy 21 as a sole abnormality was detected in 1 patient and complex abnormalities were detected in 2 patients. risomy 8 was not detected. 3) Eight (50%) of the 16 patients with AML-M2 had t (8;21), 2 of the 3 patients with AML-M3 had t (15;17) and 1 patient with AML-M4Eo had inv (16). 4) The median age of patients with chromosomal abnormalities was significantly younger than that of patients with normal karyotype (34 vs. 48 years, P=0.003). Other clinical andlaboratory characteristics were not significantly different between abnormal and normal cytogenetic groups. 5) The complete remission rate of patients with chromosomal abnormalities was lower than that of patients with normal karyotype, but the difference of CR rates was not statistically significant (78.1 vs. 84.2%, P=NS). CONCLUSION: These results suggest that lower incidence of trisomy 8, higher incidence of t (8;21) and stronger association between t (8;21) and AML-M2 than usually described may be the cytogenetic characteristics of acute myelogenous leukemia in Korean adult.
Adult* ; Chromosome Aberrations ; Chungcheongnam-do ; Cytogenetics ; Down Syndrome ; Humans ; Incidence ; Karyotype ; Leukemia, Myeloid, Acute* ; Population Characteristics ; Trisomy