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Introduction Diffuse large B cell lymphoma (DLBCL) of primary nodal (PN) or primary extranodal (PEN) origin may differ immunophenotypically, in that PEN lymphoma cells may originate from activated rather than germinal center B (GCB) cells. We evaluated the relationship between DLBCL clinicopathological features, including expression of B-cell differentiation markers, and primary tumor site. Patients and Methods Expression of CD10, Bcl-6, Bcl-2, and MUM1 was determined in paraffin-embedded tissues from 123 patients with DLBCL. Results Of the 123 patients with DLBCL, 40 (32.5%) had the GCB and 83 (67.5%) had the non-GCB phenotype. Fifty-one patients (42%) showed disease involvement at PEN sites, including 29 with disease in the gastrointestinal (GI) tract (14 in the stomach, 15 in the intestine). Of these 51 patients, 16 (31.4%) were classified with the GCB and 35 (68.5%) with the non-GCB subtype. There were no differences in the frequencies of GCB and non-GCB subtypes among primary sites. Of the 72 patients with PN DLBCL, 22 (31%) had the GCB and 50 (69%) had the non-GCB subtype. There were no differences in the frequencies of GCB and non-GCB subtypes between patients with PN and PEN DLBCL. Although lactate dehydrogenase (LDH) concentration > normal, stage >II, and rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisone (RCHOP) treatment were independent predictors of overall survival (OS), GCB subtype, and presence of PEN disease failed to predict survival upon multivariate analysis. Conclusion There was no difference in GCB and non-GCB phenotypes between patients with PN and PEN DLBCLs. Additional studies are needed to further assess molecular differences between the two groups.

Background The objective of this study was to explore whether individual variations in the concentration of growth factors (GFs) influence the biologic effects of platelet-rich plasma (PRP) on human mesenchymal stem cells (HMSCs). Methods The concentrations of 7 representative GFs in activated PRP (aPRP) were measured using ELISA. The effects of PRP on the proliferation and alkaline phosphatase (ALP) activity of HMSCs were examined using several concentrations of aPRP from 3 donors; the relationships between the GF levels and these biologic effects were then evaluated using 10% aPRP from 5 subgroups derived from 39 total donors. HMSCs were cultured in DMEM with the addition of aPRP for 4 or 12 days; then, DNA content and ALP activity were measured. Results The quantity of DNA increased significantly at a 10% concentration of aPRP, but the ALP activity was suppressed at this concentration of aPRP. The GF concentrations varied among donors, and 5 subgroups of characteristic GF release patterns were identified via cluster analysis. DNA levels differed significantly between groups and tended to be higher in groups with higher concentrations of transforming growth factor-beta1 (TGF-β1) and platelet-derived growth factors (PDGFs). DNA quantity was positively correlated with TGF-β1 concentration, and was negatively correlated with donor age. ALP activity was negatively correlated with PDGF-BB concentration. Conclusions The varying GF concentrations may result in different biologic effects; thus, individual differences in GF levels should be considered for reliable interpretation of the biologic functions and standardized application of PRP.

To the Editor, Diffuse large B-cell lymphoma (DLBCL) is an aggressive subtype of non-Hodgkin lymphoma. The occurrence of chronic myeloid leukemia (CML) as a secondary malignancy in patients diagnosed with DLBCL is extremely rare [1]. We report a unique case of secondary CML 10 years after chemotherapy and local irradiation for the treatment of tonsillar DLBCL. We also review the literature related to secondary CML. A 40-year-old male with a sore throat and a right tonsillar mass was evaluated at a local clinic in September 2000. A biopsy revealed a malignant lymphoma (DLBCL). He was then transferred to our hospital, where a staging work-up was performed. There were no metastatic lesions except for the right tonsillar mass. There was also no marrow invasion or chromosomal abnormalities in his bone marrow. The patient had a stage IA DLBCL. He received six cycles of CHOP regimen (750 mg/m2 cyclophosphamide, 50 mg/m2 adriamycin, and 2 mg vincristine on day 1, and 100 mg prednisolone on days 1 to 5) chemotherapy every 3 weeks. Chemotherapy was followed by radiotherapy to the right tonsillar region (total, 25 Gy). A complete response was achieved and sustained for 10 years. In July 2010, he sought evaluation at our hospital for easy fatigue and a febrile sensation of several months duration. His white blood cell count was 173,900/mm3, his hemoglobin concentration was 11.3 mg/dL, and platelet count was 735,000/mm3. Splenomegaly was noted on physical examination, but the remaining physical findings were nonspecific. Diffuse fluorodeoxyglucose (FDG) uptake in the bone marrow and mild and diffuse FDG uptake in the spleen were noted on a positron emission tomography and computerized tomography scan (Fig. 1). His platelet count was increased and a complete spectrum of granulocytic forms, from blasts to mature neutrophils, was noted on a peripheral blood smear (Fig. 2A). Hypercellular marrow with increased megakaryocytes and a markedly proliferated myeloid series were noted on a bone marrow study; however, the erythroid series was suppressed (Fig. 2B). A bone marrow chromosome analysis was then performed. Twelve cells were characterized by the presence of the Philadelphia translocation [t(9;22)(q34;q11)] in all analyzed mitoses; no other abnormalities were found (Fig. 3). PCR revealed a major BCR/ABL gene rearrangement in peripheral blood and bone marrow cells. The patient was diagnosed with CML. Figure 1 (A) Diffuse fluorodeoxyglucose (FDG) uptake in the bone marrow and mild and diffuse FDG uptake in the spleen with splenomegaly were noted on a positron emission tomography and computerized tomography (PET/CT) scan. (B) Abdominal CT revealed massive splenomegaly. ... Figure 2 (A) Platelet counts were increased and a complete spectrum of granulocytic forms, from blasts to mature neutrophils, was noted on the peripheral blood smear (H&E, ×400). (B) Hypercellular marrow with increased megakaryocytes and a markedly ... Figure 3 A representative karyotype shows 46, XY, t(9;22) (q34;q11.2). He was treated with 400-mg/day imatinib beginning on 29 July 2010. After treatment for 1 month, his white blood cell count was 5,660/mm3, hemoglobin concentration was 10.9 mg/dL, and platelet count was 257,000/mm3 in the peripheral blood, which indicated a complete hematologic response. After treatment for 3 months, the CR/ABL gene rearrangement was not detected in peripheral blood and bone marrow cells, and the Philadelphia chromosome was not detected during a cytogenetic study of the bone marrow. Therefore, a complete cytogenetic response was obtained after 3 months of therapy with imatinib. He continued to be treated with 400-mg/day imatinib until March 2013, and the complete cytogenic response was maintained. Secondary hematologic malignancies after neoplastic diseases are commonly reported as important late complications in cancer patients due to chemotherapy and/or radiotherapy. Advances in the treatment of malignancies have been accompanied by an increased frequency of secondary acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS); however, secondary CML is a rare event [2]. Recently, a relationship was reported between patients treated with cytotoxic therapy for malignancies and increased AML and MDS [3]. However, definitive evidence that the cytotoxic therapies used in cancer treatment increase the risk of CML has not been reported. AML and MDS are genetically diverse, and numerous genetic abnormalities have been linked to their onset. In contrast, CML is homogeneous; the BCR-ABL oncogene is the single genetic lesion that results in the development of CML [3]. This suggests that the risk of CML after exposure to a chemical incitant is markedly lower than the risk of AML or MDS. A recent study reported that 40% of patients with therapy-related AML (tr-AML) and MDS received chemotherapy, 14% received radiation therapy, and 46% underwent a combination of chemotherapy and radiation therapy for a prior malignancy. Of the patients treated with chemotherapy, 78% received alkylating agents, and 39% received topoisomerase II inhibitors. The most common primary tumors of tr-AML and MDS were Hodgkin and non-Hodgkin lymphoma [3]. In a similar study assessing patients with tr-CML, 44% of patients received combination therapy. In addition, the chemotherapy agents used to treat the primary disease included alkylating agents and topoisomerase II inhibitors. The most common primary tumor in this cohort of tr-CML patients was Hodgkin's lymphoma [1]. The occurrence of CML is increased in survivors of the atomic bomb detonations in Hiroshima and Nagasaki, and in patients irradiated for ankylosing spondylitis or cervical cancer [4,5], all of whom were exposed to high doses of ionizing radiation. This suggests that CML may be more likely to occur after acute high dose radiation exposure than other types of leukemia. A previous study reported that the median interval to diagnosis of CML in patients treated with combination chemotherapy and radiotherapy, radiotherapy alone, and chemotherapy alone was 60 months (range, 27 to 240), 53 months (range, 27 to 109), and 35 months (range, 23 to 148), respectively. There was no statistically significant difference between these groups of patients [1]. In our study, the interval between diagnosis of DLBCL and CML was 118 months. Our patient received six cycles of CHOP chemotherapy, including an alkylating agent, followed by local irradiation (total, 25 Gy) for the treatment of tonsillar DLBCL. However, the total dose of irradiation was low, and was divided into several fractions. In addition, there was a long interval to the diagnosis of CML (10 years). Therefore, it is unlikely that the treatment for DLBCL was related to the development of CML; however, tr-CML could not be excluded. Therefore, we were unable to discriminate between tr-CML and non-tr-CML in this patient. Nevertheless, chemotherapy or irradiation therapy for malignancies can cause tr-CML.

Germline TP53 mutations are the underlying genetic defect in Li–Fraumeni syndrome (LFS), a complex autosomal dominant disorder characterized by the early occurrence of multiple cancers [1]. Common ...

Chromosome 1 band p32 (1p32) aberrations are common in T lymphoblastic leukemia/lymphoma (T-ALL/LBL). Two types of 1p32 aberrations include translocations with different partners and submicroscopic interstitial deletion. Both aberrations are known to result in TAL1 gene deregulation. The t(1;5)(p32;q31) is a rare translocation of 1p32 in T-ALL. We now present the second case of t(1;5)(p32;q31) in T-ALL, which was present as a primary cytogenetic abnormality, with a review of the relevant literature. Interestingly, neither the translocation of the TAL1 gene nor aberrant expression of TAL1 protein was detected by fluorescent in situ hybridization (FISH) and by immunohistochemical staining in this case.

Trisomy 19 is frequently encountered in cases of chronic myeloid leukemia (CML) as a secondary abnormality: however, trisomy 19 rarely occurs as a sole chromosomal abnormality and, to date, it has only been reported in 48 hematopoietic malignancies, 1 case of adenocarcinoma and 1 case of astrocytic tumor. Here, we report two additional cases of trisomy 19 as a sole karyotypic aberration in myeloid malignancies. One of these cases involved a 6-month-old male who was diagnosed with acute myeloid leukemia minimally differentiated. His karyotype was 47,XY,+19[20]. He expired 5 days after diagnosis. Another case occurred in an 80-yr-old female who had refractory anemia with excess blasts. Her karyotype was 47,XX,+19[16]/46,XX[4]. Four months later, her peripheral blood smears suggested that the disease had progressed, but she refused further evaluation. Based on a review of the existing literature and the results of this report, trisomy 19 not only as a secondary abnormality but also as a sole karyotypic aberration is strongly associated with myeloid disorder; however, it is not preferentially found in specific FAB subgroups of myelodysplasic syndrome or acute myeloid leukemia.

TNF-alpha mediated apoptosis of the hematopoietic cells has been thought to contribute to the ineffective hematopoiesis observed in myelodysplastic syndrome (MDS). The combination of pentoxifylline (P) and ciprofloxacin (C) has been shown to reduce the serum levels of TNF-alpha, and an earlier trial of P and C with dexamethasone (D) provided good palliation for patients with MDS. The purpose of this study is to assess the hematologic response to PCD therapy for patients suffering with MDS. 21 of 25 patients who completed at least of 12 weeks of treatment were evaluable for the treatment efficacy. At baseline, the patient's median age was 60 yr (range: 18-75 yr). The diagnoses according to WHO classification included: RA (n=5), RCMD (n=10), RARS (n=1), RCMD/RS (n=1), RAEB (3), and CMML (n=1). 11 patients (52%) had at least single lineage response. 3 patients (11%) showed improvement of triple lineage cytopenia. There were no differences in the response rates between the FAB subtypes. The median time to response was 4 weeks (range: 2-12 weeks), and it is interesting that 9 of 11 patients who had a response remained without relapse for a median of 177 days (range: 78-634 days). These preliminary results indicate that anti-cytokine therapy with PCD is an effective and well tolerated palliative treatment for patients with MDS.

Thrombotic thrombocytopenic purpura (TTP) is a disseminated form of thrombotic micro-angiopathy. Although most cases are held to be idiopathic, its association with malignancy iswellrecognizedanditusuallyoccursattheterminalstageofcancer.Bonemarrownecrosis(BMN)is another rare disorder defined pathologically as the necrosis of myeloid tissue and medullarystromawithpreservationofbone.Whilehematologicmalignancyisthemostcommonunderlyingdisease associated with BMN, it can also be caused by solid tumors. Neither TTP nor BMNassociated with colon cancer has been reported. We describe here a patient with the rare asso-ciation of TTP and BMN displayed as the first manifestation of an advanced colon cancer. Theanemia and thrombocytopenia responded not to plasma exchange but to the combinationchemotherapy. This case indicates that metastatic cancer should be included in the differentialdiagnosis of TTP and BMN, and that the chemotherapy may improve the detrimental clinicalcourse.Key words: thrombotic thrombocytopenic purpura – bone marrow necrosis – colon cancer –microangiopathic hemolytic anemia

We evaluated the impact of functional polymorphisms in the vascular endothelial growth factor A (VEGFA) and vascular endothelial growth factor 2 (VEGFR2) genes on the survival of patients with diffuse large B cell lymphoma (DLBCL). Five potentially functional polymorphisms in the VEGFA (rs699947, rs2010963 and rs3025039) and VEGFR2 (rs1870377 and rs2305948) genes were assessed in 494 DLBCL patients treated with rituximab plus CHOP chemotherapy. The associations of genotype and haplotype with overall survival (OS) and progression-free survival (PFS) were analyzed. Of the five polymorphisms, VEGFR2 rs1870377T>A was significantly associated with both OS and PFS; in the dominant model, patients with the AA + TA genotypes had significantly better OS (P = 0.002) and PFS (P = 0.004) than those with the TT genotype. The association between significantly better OS and the AA + TA genotypes was observed separately in patients with low (0–2; P = 0.035) and high (3–5; P = 0.043) International Prognostic Index scores. Multivariate analysis showed that, relative to the AA + TA genotypes, the TT genotype was an independent prognostic factor for poor OS (HR, 1.71; 95% CI, 1.21–2.43; P = 0.002) and PFS (HR, 1.57; 1.13–2.17; P = 0.004). Other independent significant predictors of survival in patients with DLBCL were International Prognostic Index score, age > 60 years, lactate dehydrogenase concentration >normal, extranodal disease >1 and presence of B symptoms. The VEGFR2 rs1870377 polymorphism might affect survival in patients with DLBCL, suggesting that angiogenesis might be related to poor survival in these patients. (Cancer Sci 2012; 103: 497–503)

Korean National Health Insurance (NHI) was introduced in 1963 and became a nationwide health insurance after several law reforms in 1988. As a result of nationalization, coverage has expanded, and its service level has greatly improved since 1998 [1]. However, several diagnostic and therapeutic modalities for rare, incurable diseases such as malignant hematologic diseases are excluded from the reimbursement criteria, and this often complicates physicians' decision making in terms of evidence-based guidelines. The diagnosis and treatment of hematologic diseases have been rapidly advancing in recent years. However, NHI policy limitations hinder the addition of these advances to standard practice guidelines. Furthermore, the system's current principle of basic equal sharing regardless of disease severity will inevitably lead to a budget deficit. In this article, we aim to illuminate the insurance problems that arise in the treatment of hematologic diseases under the current NHI and propose solutions to these problems.

In B lymphoblastic leukemia/lymphoma (B-ALL/LBL), t(9;22)(q34;q11.2) and t(1;19)(q23;p13.3) are recurrent cytogenetic abnormalities. The concurrent occurrence of both abnormalities is very rare, and only 3 cases have been previously reported. Here, we report a case of adult B-ALL with ider(9)(q10)t(9;22)(q34;q11.2) and der(19)t(1;19)(q23;p13.3). A literature review revealed that ider(9) (q10)t(9;22) is a rare variant of t(9;22) with a deletion of the short arm of chromosome 9. Fifteen cases of ider(9)(q10)t(9;22) have been reported. This abnormality is specific to precursor B-lymphoid neoplasms, such as B-ALL or B-lymphoid blast phase of CML, and is associated with disease progression or short survival. The cytogenetic abnormality t(1;19) is also specific to B-ALL. In most instances of t(1;19), TCF3 is fused to PBX1; however, a few cases have identical translocations but no TCF3-PBX1 fusion, as was observed in our patient. We describe the first case of ider(9)(q10)t(9;22) in combination with TCF3-PBX1 negative t(1;19). The patient underwent imatinib therapy in addition to intensive chemotherapy, but failed to achieve remission.

Partial trisomy 2p is a rare but relatively well-defined syndrome with distinctive clinical features, including marked psychomotor delay, dysmorphic face, and congenital heart disease. The phenotype of trisomy 18p is variable, from normal appearance to moderate mental retardation. Most cases of trisomy 2p and trisomy 18p result from the inheritance of an unbalanced segregant from a balanced parental translocation or due to de novo duplication. Here, we present the first report of a combined partial trisomy 2p and trisomy 18p due to a supernumerary marker chromosome (SMC). The final karyotype of the patient was 47,XX,+der(18)t(2;18)(p23.1;q11.1)[22]/46,XX[8]. The patient had typical dysmorphic features of partial trisomy 2p23-pter syndrome and congenital heart disease. SMCs are remarkably variable in euchromatic DNA content and mosaicism level. The precise identification of the origin and composition of SMCs is essential for genotype-phenotype correlation and genetic counseling.

This study is conducted to determine the effect of different kinds of bone substitutes and collagen on the concentration of platelet-derived growth factor (PDGF) and transforming growth factor β-1 (TGF β-1) in platelet-rich plasma (PRP). PRP is treated with thrombin, hydroxyapatite (HA), and thrombin, HA alone, collagen-grafted HA, calcium metaphosphate (CMP), and collagen-grafted CMP. The concentrations of PDGF-AB and TGF β-1 are measured. After PRP treated with HA and CMP, the concentrations of PDGF and TGF β-1 are not significantly different from the concentration of them in PRP alone. The concentrations of PDGF in PRP with collagen-grafted HA and collagen-grafted CMP are significantly higher than that of PRP with HA and CMP. The concentrations of PDGF and TGF β-1 in PRP with collagen-grafted CMP are higher than with collagen-grafted HA. The results of multiple regression analysis show that PDGF increased with the use of collagen and thrombin, and is higher in native whole blood with higher platelet counts. However, PDGF decreased with the use of HA. In conclusion, HA and CMP do not seem to be able to activate platelets by themselves. However, if they had collagen grafted onto them, they could activate platelets and release growth factors.

A therapy-related myelodysplastic syndrome (t-MDS) during the course of Waldenstrom's macroglobulinemia (WM) has been observed in rare patients. In most of them, the condition developed after treatment with alkylating agents. We experienced a 65-year-old male patient who was diagnosed as WM. He was treated with intermittent oral chlorambucil for 12 months and three cycles of fludarabine, and complete response was achieved after fludarabine treatment. During routine outpatient follow-up, severe anemia occurred. His bone marrow aspirate showed dysplastic hemopoiesis with ringed sideroblasts and siderocytes, which is consistent with MDS (refractory anemia with ringed sideroblasts). Cytogenetic analysis showed complex chromosomal abnormalities including 5q deletion, 12p deletion and monosomy 18. When decision is made to treat WM with chlorambucil and/or fludarabine, a potential risk for t-MDS or therapy-related acute myeloid leukemia should be considered and a close hematologic monitoring is needed.

Abstract 5157 Background: With the development of diagnostic technique, an accurate diagnosis of hereditary hemolytic anemia (HHA)- red blood cell (RBC) membranopathy, hemoglobinopathy, RBC enzymopahty – have been made. Therefore, we surveyed the prevalence and characteristics of patients diagnosed as HHA during recent five years in Korea. Methods: Through the use of questionnaires, information on the clinical and laboratory findings of HHA diagnosed from 2007 to 2011 in Korea was collected. The globin gene analysis (direct sequencing) and RBC enzyme analysis was performed at the representative laboratories. A total of 203 cases were collected in this study by the Korean Hereditary Hemolytic Anemia Working Party of the Korean Society of Hematology. Results: Patients number of RBC membranopathy, hemoglobinopathy, and RBC enzymopahty was 125, 47, and 31, respectively. Percentage of patients with dominant family history was 57% in patients with hereditary spherocytosis (n=116) and dominant symptoms were anemia, jaundice, splenomegaly and gallstones. Osmotic fragility test and flow cytometric method for detection of RBC membrane defect were performed about 60% of patients. RBC membrane protein analysis using sodium dodecyl sulfate polyacrylamide gel electrophoresis was performed on 59 patients. Of the 47 cases of hemoglobinopathies, 36 cases (77%) were β-thalassemia minor, 10 cases (21%) were α-thalassemia minor and one case (2%) was unstable Hb, Hb M-Saskatoon (beta 64 His-→Tyr). Median age at diagnosis was 7 years (range: 6 months–58 years). Eleven of 47 cases (23%) had family history of HHA. As all thalassemia patients were thalassemia minor, they presented with mild jaundice or pallor. Of the 31 patients were diagnosed as RBC membranopathy, pyruvate kinase deficiency was 3 cases and glucose-6 phosphate dehydrogenase deficiency was 2, and other various forms were reported. Conclusions: We could confirm that accurate diagnosis has been made in more patients using elegant diagnostic technique. However, more defined diagnostic approaches were needed in this rare disease and further systematic supporting systems for patients and their families were warranted in public health aspect. Disclosures: No relevant conflicts of interest to declare.

Objectives : The aim of this study is to examine the cognitive function change related to aging, the incidence of cognitive impairment, and the association between apolipoprotein E polymorphism and cognitive impairment through a follow-up of the elderly with normal cognitive ability at baseline. Methods : Two hundred and fifteen subjects aged 65 and over were surveyed in February, 1998 (baseline survey), and their cognitive function was assessed again in 2003 1st follow-up) and the once again in 2006 (2nd follow-up). Ninety one subjects completed all surveys up through the 2nd follow-up and their cognitive function scores using MMSE-K (Korean Version of the Mini-Mental State Examination) and the distribution of apolipoprotein E allele were analyzed. Results : The cognitive function scores decreased with aging and the difference between baseline and the 2nd follow-up scores of the study increased with the age group. The incidence rate of cognitive impairment through an 8-year follow-up was 38.5% and higher in older age groups. Age was the only significant factor for incidence of cognitive impairment, but there was no significant association between apolipoprotein E genotype and incidence of cognitive impairment. Conclusions : The cognition of the elderly decreased with aging and the association of apolipoprotein E genotype with incidence of cognitive impairment was not significant in this study. To confirm the association between apolipoprotein E polymorphism and incidence of cognitive impairment further studies will be needed.

According to the World Health Organization (WHO) classification system, cases with t(8;21)(q22;q22) should be diagnosed as acute myeloid leukemia (AML) even with a blast count of less than 20 percent in blood or bone marrow. It is an uncommon manifestation, moreover hypocellularity is rarely observed in this subtype of leukemia. Here, we report a case of t(8;21) in a patient with marked hypocellularity of less than 5 percent and a blast count of less than 20 percent. This patient responded relatively well to chemotherapy. An allogeneic bone marrow transplantation was performed with good engraftment. This case suggests that hypocellular AML with a t(8;21) has as good a prognosis as hypercellular AML with t(8;21).

Constitutional trisomy 8 is a relatively rare aneuploidy; most identified cases are mosaic with a normal cell line. The phenotype is highly variable from apparently normal to severe disability. The proportion of abnormal cells is dramatically different between tissues and the severity of the phenotype is not directly related to the level of mosaicism. Therefore, it is very difficult to provide a definitive prognosis. We report here a case of constitutional trisomy 8 mosaicism with agenesis of the corpus callosum, congenital heart disease and micrognathia. The trisomy 8 cell line was not detected by prenatal cytogenetic study. This is the fourth reported case of constitutional trisomy 8 mosaicism in Korea.

The involvement of central nervous system is rare in acute promyelocytic leukemia (APL). We report a APL patient of a 41 yr-old Korean male who presented with fever and petechia. Complete molecular remission was achieved with all-trans retinoic acid (ATRA), idarubicin, and cytarabine. Ten months later, he complained of a mild headache. The results of the physical examination and the complete blood counts were normal. The examination of cerebrospinal fluid showed the presence of promyelocyte. Bone marrow studies showed cytogenetic remission but with molecular relapse. He was treated with intrathecal and systemic chemotherapy.

A congenital factor VII deficiency is a rare disorder with an estimated incidence in the western contries of one in 500,000. Because factor VII is important in initiation the coagulation cascade, a factor VII deficiency can result in significant bleeding with prolongation of the prothrombin time. We present a case of a factor VII deficiency with a subdural hematoma in an 18-year-old boy whose plasma activity of factor VII was ≤10%. Previously, he did not have any symptoms, such as hemarthrosis, easy bruising or bleeding after a minor trauma. He was administered fresh frozen plasma and a trephination was performed. His sister also had 51% lower level of factor VII.

Simultaneous deficiency of Vitamin and iron induces that the bone marrow erythroid megaloblastosis and peripheral blood macroovalocytosis are masked because of countervailing the tendency of iron deficiency to produce hypochromic microcytic erythrocytes. We report two cases of Vitamin deficiency anemia with low mean corpuscular volume (MCV) due to combined iron deficiency anemia with review of literature.

The aim of this study was to evaluate domestic enzyme immunoassay(EIA) kit 'LG RCD 3.0' (LG) for the detection of antibody to hepatitis C virus(anti-HCV) in comparision with Axsym HCV version 3.0(Axsym), Cobas Core anti-HCV EIA(Cobas). Cobas kit shows better clear distinction between positive and negative by signal/cutoff ratio(S/C), but it also reveal relatively high false positive rate. The concordance rate of test results between LG and Axsym was 96.2%, between LG and Cobas was 95.5%, and total agreement between three EIA kit was 93.9%. LG were relative poor distinction between positive and negative results, but it could be applied clinically as a screening tool for hepatitis C in general population. The SIC of one false negative result by LG was 0.91, and false positive were less than 4.0, therefore we concluded it is necessary to confirm by immunoblotting assay when SIC were between 0.8 and 4.0.

Four subtypes of hepatitis B surface antigen are useful in the epidemiologic studies of the route of virus transmission and clinical significance of simultaneous occurance of hepatitis B surface antigen and antibody to hepatitis B surface antigen in the same serum as well as useful marker for population migration. The sera were obtained from 214 HBs Ag positive patients who are diagnosed as chronic liver disease and following up in the Yeungnam university hospital. The subtypes were determined by solid-phase sandwich EIA using monoclonal antibodies. Among 214 specimens, the subtype adr was 93.9%, adw was 2.8%, ayr was 0.9%, ar was 0.9%, adwr was 1.4% and ayw was not detected. There were no correlation between subtype pattern and disease. In summary, the subtype adr was prominent in our study and the difference of subtype pattern by severity of disease was not significant. However, to determine the prognostic value of HBs Ag subtype and relationship between subtype and disease progression, long-term follow up will be needed.