Hepcidin Level and Iron Parameters in Patients with Chronic Kidney Disease

BACKGROUND: Hepcidin, a key regulator of iron homeostasis, is associated with iron metabolism imbalance in patients with chronic kidney disease (CKD). However, serum hepcidin level in anemic patients with CKD presents a contradictory picture. We investigated the relationship between serum hepcidin-25 level and iron parameters in patients with CKD. METHODS: We defined and categorized patients with CKD according to the Kidney Disease Outcomes Quality Initiative (KDOQI) guidelines. We analyzed the relationship between serum hepcidin-25 level and iron parameters [serum iron, total iron-binding capacity (TIBC), unbound iron-binding capacity (UIBC), transferrin saturation, and ferritin levels] according to the CKD stage and clinical and laboratory characteristics. RESULTS: Hb level, TIBC, and UIBC decreased and ferritin level increased (Ptrend0.05). CONCLUSIONS: Serum hepcidin-25 level was not found to be associated with iron parameters or clinical status of CKD patients in our study. Determination of hepcidin-25 levels may not provide more information than conventional iron parameters in monitoring iron metabolism in CKD patients. However, further studies are needed to establish the clinical utility of hepcidin measurement in CKD patients.

A Case of B-cell Lymphoma, Unclassifiable, with Features Intermediate between Diffuse Large B-cell Lymphoma and Burkitt Lymphoma in a Korean Child

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL) (intermediate DLBCL/BL), is a heterogeneous group with some features resembling DLBCL and others resembling BL. Here, we report a case of intermediate DLBCL/BL in a Korean child. A 2-yr-old male was admitted for evaluation and management of left hip pain. Immunohistochemistry of a biopsy of the femur neck revealed tumor cells positive for CD20, CD10, BCL2, BCL6, and Ki67. A bone marrow (BM) aspirate smear revealed that 49.3% of all nucleated cells were abnormal lymphoid cells, composed of large- and medium-sized cells. Immunophenotyping of the neoplastic cells revealed positivity for CD19, CD10, CD20, and sIg lambda and negativity for CD34, Tdt, and myeloperoxidase (MPO). Cytogenetic and FISH analyses showed a complex karyotype, including t(8;14)(q24.1;q32) and IGH-MYC fusion. Intensive chemotherapy was initiated, including prednisone, vincristine, L-asparaginase, daunorubicin, and central nervous system prophylaxis with intrathecal methotrexate (MTX) and cytarabine. One month after the initial diagnosis, BM examination revealed the persistent of abnormal lymphoid cells; cerebrospinal fluid cytology, including cytospin, showed atypical lymphoid cells. The patient was treated again with cyclophosphamide, vincristine, prednisone, adriamycin, MTX, and intrathecal MTX and cytarabine. The patient died of sepsis 5 months after the second round of chemotherapy.

Reticulocyte Indices for the Differential Diagnosis of Anemia of Chronic Disease and Iron Deficiency Anemia in Adult Women with Microcytic Hypochromic Anemia

BACKGROUND: Iron deficiency anemia (IDA) is the most common anemia followed by anemia of chronic disease (ACD). Reticulocyte indices have been shown to be helpful indicators for detecting IDA. We investigated whether RBC and reticulocyte indices can be used to differentiate ACD from IDA. METHODS: A total of 85 women showing microcytic hypochromic anemia (38 IDA and 47 ACD cases) were enrolled. IDA was defined as those with ferritin level of 450 microg/dL. ACD was defined as ferritin level of > or =6 microg/dL, TIBC of or =24.6 pg could be used to differentiate ACD from IDA with 85.1% sensitivity and 81.6% specificity. CONCLUSIONS: The reticulocyte indices, especially CHr, are useful for the differential diagnosis of microcytic hypochromic anemias, ACD and IDA.

A Case of Non-secretory Myeloma with Crystal-storing Histiocytosis / 대한진단검사의학회지

Crystal-storing histiocytosis (CSH) is a rare event observed in association with lymphoproliferative diseases, and mainly occurrs in plasma cell dyscrasias. It is presumed to be an intra-lysosomal accumulation of the secreted paraproteins. Crystal formation can be seen inside histiocyte-like cells with phagocytosed crystalline inclusions in the bone marrow and extramedullary sites. CSH is a rare morphological entity with poor prognostic implications and may be confused with Gaucher or pseudo-Gaucher cells. Herein we report a case of non-secretory myeloma associated with CSH showing a poor clinical course. A 79-yr-old male presenting with dizziness was evaluated in hematology department for anemia. Laboratory tests revealed Hb of 4.9 g/dL and beta2-microglobulin of 21,000 ng/mL (reference range, 0-370). Presence of monoclonal protein was not detected on protein electrophoresis and immunofixation in serum and urine. However, serum free light chain assay showed an increased kappa-light chain level of 126 mg/L (reference range, 3.3-19.4) resulting in an increased kappa/lambda ratio. The bone marrow touch print showed numerous plasma cells and crystal-laden histiocytes and immunohistochemical stainings on bone marrow biopsy revealed positivity for CD38, CD56 and kappa in the plasma cells and CD68 and kappa in crystal-laden histiocytes.

Impact of Revised Penicillin Breakpoints for Streptococcus pneumoniae (CLSI M100-S18) on the Penicillin Susceptibility Rate / 대한임상미생물학회지

BACKGROUND: In January 2008, the Clinical and Laboratory Standards Institute (CLSI) published revised penicillin breakpoints for Streptococcus pneumoniae according to clinical presentation and the route of penicillin administration. The aim of this study was to evaluate the impacts of the new penicillin breakpoints on the susceptibility rates of S. pneumoniae isolated from blood. METHODS: A total of 156 non-duplicated S. pneumoniae strains recovered from blood of hospitalized patients were collected between January 2003 and December 2008. Penicillin and cefotaxime susceptibility tests were performed using an E-test (AB Biodisk, Solna, Sweden). Results of the penicillin susceptibility tests were analyzed using the former and new CLSI guidelines. RESULTS: Of the 156 S. pneumoniae strains isolated from blood, penicillin susceptibility under the former CLSI guidelines resulted in 42.3% susceptible, 42.3% intermediate, and 15.4% resistant states. According to the new CLSI guidelines (nonmeningitis, parenteral), 87.8% of isolates were susceptible, 9.6% were intermediate, and 2.6% were resistant to penicillin. CONCLUSION: When the new CLSI guidelines are applied, the penicillin susceptibility rate of S. pneumoniae strains isolated from blood is considerably increased. This suggests that penicillin should still be useful for the treatment of nonmeningeal pneumococcal infections and that the use of broad-spectrum antimicrobials should not replace this treatment.

Characteristics of CTX-M Type Extended Spectrum beta-lactamase Producing Non-typhoi dal Salmonella Isolates / 감염과화학요법

BACKGROUND: Extended-spectrum beta-lactamase (ESBL)-producing Salmonella have been increasingly reported worldwide. ESBL-producing Salmonella is of particular concern since children cannot be treated with quinolones. This study was conducted to determine the phenotypic and genetic characteristics of ESBL-producing Salmonella in a tertiary hospital. MATERIALS AND METHODS: Four clinical ESBL-producing isolates of non-typhoidal Salmonella were collected during 2001 to 2009. Antimicrobial susceptibility was determined by disk diffusion test and VITEK-II system. ESBL production was tested by ESBL phenotypic confirmatory test. TEM, SHV, CTX-M1, CTX-M2, CTX-M8, and CTX-M9 type ESBL genes were detected by PCR amplification, and PCR products were subjected to direct sequencing. RESULTS: Phenotypic confirmatory test showed that 4 of the 300 non-typhoidal Salmonella isolates were ESBL-producing: 3 S. Enteritidis and 1 S. Typhimurium. All 4 isolates were recovered during the past 1 year period. All 3 S. Enteritidis harbored CTX-M-15, while the S. Typhimurium harbored CTX-M-14. All CTX-M-15-producing S. Enteritidis isolates showed resistance both to cefotaxime and ceftazidime, while the CTX-M-14-producing S. Enteritidis were resistant only to cefotaxime. CONCLUSIONS: ESBL-producing nontyphoidal Salmonella has emerged recently and the type of ESBL has switched from TEM and SHV to CTX-M.

t(9;22) with 5'ABL1 Deletion and t(6;19) in Biphenotypic Acute Leukemia

Biphenotypic acute leukemia (BAL) is a rare type of leukemia, comprises 4% of all acute leukemias. It is more common in adults and the clinical features, as related to marrow dysfunction, are similar to those found in other patients with acute leukemia. BAL commonly shows a dimorphic blast population with, one resembling lymphoblasts and the other resembling myeloblasts. The majority of BAL patients express B-lymphoid and myeloid markers. BAL can be diagnosed by morphologic studies and by a comprehensive panel of immunological markers, as well as cytogenetic/molecular studies, such as fluorescence in situ hybridization (FISH) and reverse transcriptase-polymerase chain reaction (RT-PCR). In addition, its prognosis is relatively poor. We present here a 27 year-old female patient who showed lymphoblasts and myeloblasts on her marrow studies and these cells were positive for myeloid and B-lymphoid markers on the immunophenotypic studies. Chromosome analysis revealed 46,XX,t(6;19)(p23;p13.1),t(9;22)(q34;q11.2). A major (b3a2) type of BCR-ABL1 mRNA transcript was detected by RT-PCR, and a 5'ABL1 deletion was identified by FISH.

A Case of Primary Autoimmune Myelofibrosis

Myelofibrosis is usually observed in association with hematologic malignancies or metastatic solid tumors, but it has rarely been reported in patients who suffer with autoimmune disorders. Autoimmune myelofibrosis is a distinct clinicopathologic entity and it can occur alone or in association with autoimmune disorders, and the final result is chronic peripheral cytopenia. Primary autoimmune myelofibrosis, in which the autoimmune myelofibrosis is not preceded by a well-defined autoimmune disease, has recently been defined as a distinct clinicopathologic syndrome. We report here on a case of an 18-year-old woman who was diagnosed with primary autoimmune myelofibrosis, and she manifested peripheral pancytopenia, positivity for autoantibodies and Grade III myelofibrosis without having any preceding autoimmune or hematologic disorders.

Evaluation of Platelet Indices for Differential Diagnosis of Thrombocytosis by ADVIA 120 / 대한진단검사의학회지

BACKGROUND: For the diagnosis of essential thrombocythemia (ET), no single clinical or laboratory finding of diagnostic value is available and a differential diagnosis of other myeloproliferative neoplasms or reactive thrombocytosis (RT) is needed. Following recent developments in automated blood cell analyzers, various platelet indices can now be measured. In this study, we analyzed whether platelet counts and 6 platelet indices can be used for the differentiation of ET from RT in patients with a platelet count of 600x10(3)/microliter or more. METHODS: The subjects studied were 31 patients with ET and 224 patients with RT. The platelet counts, mean platelet volume (MPV), plateletcrit (PCT), platelet distribution width (PDW), mean platelet mass (MPM), mean platelet component concentration (MPC) and large platelets (LPLT) were measured by ADVIA 120 (Bayer Diagnostics, USA). The mean values of each item were compared between the two patient groups and the sensitivity and specificity of each item in the diagnosis of ET were determined by ROC curve analysis. RESULTS: In essential thrombocythemia, all parameters except MPC were significantly higher than in reactive thrombocytosis. For the diagnosis of ET, the sensitivity and specificity were: 74.2% and 84.4%, when the platelet count was > or = 820x10(3)/microliter; 80.6% and 80.0%, when the plateletcrit was > or =0.63%; and 64.5% and 99.1%, respectively, when LPLT was > or = 23x10(3)/microliter. CONCLUSIONS: The platelet counts and platelet indices are useful for the differential diagnosis of thrombocytosis. The plateletcrit and LPLT are particularly useful for the diagnosis of ET when the platelet count is markedly increased.