Performance Evaluation of the CS-5100 Coagulation Analyzer for Special Coagulation Parameters

Current Concepts in Primary Effusion Lymphoma and Other Effusion-Based Lymphomas

Primary effusion lymphoma (PEL) is a human herpes virus 8 (HHV8)-positive large B-cell neoplasm that presents as an effusion with no detectable tumor in individuals with human immunodeficiency virus infection or other immune deficiencies. PEL is an aggressive neoplasm with a poor prognosis. PEL cells show diverse morphologies, ranging from immunoblastic or plasmablastic to anaplastic. The immunophenotype of PEL is distinct, but its lineage can be misdiagnosed if not assessed thoroughly. PEL cells usually express CD45, lack B- and T-cell-associated antigens, and characteristically express lymphocyte activation antigens and plasma cell-associated antigens. Diagnosis of PEL often requires the demonstration of a B-cell genotype. HHV8 must be detected in cells to diagnose PEL. In most cases, PEL cells also harbor the Epstein-Barr virus (EBV) genome. Similar conditions associated with HHV8 but not effusion-based are called "extracavitary PELs." PELs should be differentiated from HHV8-negative, EBV-positive, body cavity-based lymphomas in patients with long-standing chronic inflammation; the latter can occur in tuberculous pleuritis, artificial pneumothorax, chronic liver disease and various other conditions. Despite their morphological similarity, these various lymphomas require different therapeutic strategies and have different prognostic implications. Correct diagnosis is essential to manage and predict the outcome of patients with PEL and related disorders.

Prognostic Significance of Absolute Lymphocyte Count/Absolute Monocyte Count Ratio at Diagnosis in Patients with Multiple Myeloma

BACKGROUND: Absolute lymphocyte count (ALC) in peripheral blood has recently been reported to be an independent prognostic factor in multiple myeloma (MM). Previous studies indicated that the absolute monocyte count (AMC) in peripheral blood reflects the state of the tumor microenvironment in lymphomas. Neither the utility of the AMC nor its relationship with ALC has been studied in MM. METHODS: The prognostic value of ALC, AMC, and the ALC/AMC ratio at the time of diagnosis was retrospectively examined in 189 patients with MM. RESULTS: On univariate analysis, low ALC ( or =490 cells/microL), and low ALC/AMC ratio (<2.9) were correlated with worse overall survival (OS) (p=.002, p=.038, and p=.001, respectively). On multivariate analysis, the ALC/AMC ratio was an independent prognostic factor (p=.047), whereas ALC and AMC were no longer statistical significant. Low ALC, high AMC, and low ALC/AMC ratio were associated with poor prognostic factors such as high International Staging System stage, plasmablastic morphology, hypoalbuminemia, and high beta2-microglobulin. CONCLUSIONS: Univariate analysis demonstrated that changes in ALC, AMC, and the ALC/AMC ratio are associated with patient survival in MM. Multivariate analysis showed that, of these factors, the ALC/AMC ratio was an independent prognostic factor for OS.

Omental Histiocytic Sarcoma: A Case Report

We report a case of perigastric histiocytic sarcoma (HS) involving the lesser omental sac in a 30-year-old man. HS is an exceedingly rare malignancy of mature tissue histiocyte. The tumor was a multi-lobulated, bulging enhancing mass in the lesser omentum with metastasis to lymph nodes and liver. The tumor consisted of diffuse non-cohesive proliferation of pleomorphic large oval to round neoplastic cells with giant cells showing vesicular chromatin and ample eosinophilic cytoplasm. In some areas, the tumor cells showed spindling with elongation of the nuclei and cellular shapes. Many of the tumor cells, especially giant forms contained phagocytosed lymphocytes. Immunohistochemical analysis of the tumor cells showed expression of leukocyte common antigen, CD68, lysozyme, vimentin, CD4, and CD163. Ki-67 index was 50-60%. After the operation, he was treated with chemotherapy, but the response was poor.

A Case of Histiocytic Sarcoma Diagnosed by Bone Marrow Biopsy in a Patient Suffering from Fever for 8 Months / 대한진단검사의학회지

Histiocytic sarcoma is a malignant proliferation of cells showing morphologic and immunophenotypic features similar to those of mature tissue histiocytes and is known for its rapid progression and poor prognosis. We describe a case of histiocytic sarcoma diagnosed by bone marrow biopsy. A 64-yr-old male was admitted for fever and weight loss that persisted for 8 months. The patient died undiagnosed on the 7th hospitalization day. A bone marrow biopsy performed just before the patient's death revealed diffuse proliferation of large pleomorphic neoplastic cells with large, round to oval nuclei, vesicular chromatin, and abundant foamy cytoplasm. These cells were positive for histiocytic markers, CD68, lysozyme, CD21, and S-100 protein, but negative for B-cell, T/NK-cell, and epithelial cell markers, thus confirming the presence of histiocytic sarcoma.

A Case of Chronic Neutrophilic Leukemia with Multiple Myeloma and an Increased Function of Her Neutrophils / 대한혈액학회지

We describe here a 64-year-old woman who simultaneously presented with chronic neutrophilic leukemia (CNL) and multiple myeloma (MM). The patient presented with mature neutrophilic leukocytosis, hepatosplenomegaly, the absence of Philadelphia chromosome and the BCR-ABL fusion gene, along with IgG kappa type monoclonal gammopathy in her serum and urine. The bone marrow aspirates showed hypercellularity with marked granulocytic hyperplasia and an increase in immature plasma cells. The neutrophil function tests showed increased phagocytosis, chemotaxis and respiratory burst activity, but there was normal microbial killing activity. The patient was treated with dexamethasone and pamidronate for MM and with hydroxyurea for CNL, and she was discharged from the hospital in an improved condition.

Fine Needle Aspiration Cytology of the Plasmablastic Lymphoma in Human Immunodeficiency Virus(HIV) Negative Patient: A Case Report / 대한세포병리학회지

Plasmablastic lymphoma(PBL) is a recently described aggressive B-cell neoplasm, which usually manifests as a localized disease of the oral mucosa in individuals infected with human immunodeficiency virus(HIV). Recently, we encountered a case of plasmablastic lymphoma manifesting in the left maxillary sinus and cervical lymph node of a previously healthy HIV-negative man, 48 years of age. we conducted a fine-needle aspiration smear of the cervical lymph node, and this was found to be highly cellular with numerous large cells exhibiting eccentrically positioned nuclei, prominent nucleoli, and moderate quantities of basophilic cytoplasm. A biopsy of the mass in the maxillary sinus evidenced diffuse growth of similar plasmablastic cells. These tumor cells were negative for the leukocyte common antigens, CD20, CD3, CD30, and EMA. However, the cells tested positive for CD79a and CD138/syndecan-1. The tumor cells also exhibited L-light-chain restriction. The Ki-67 proliferation index was measured at almost 100%. The patient was diagnosed with plasmablastic lymphoma. After three cycles of combination chemotherapy and radiotherapy, the patient went into complete remission, and currently remains in this state.

Reorganization of Automated Outpatient Laboratory to improve Total / 대한진단검사의학회지

BACKGROUND: Asan Medical Center ran a fully automated outpatient laboratory to serve outpatient departments with a rapid turnaround time (TAT; one hour from reception to reporting) for frequently requested test items and thus to make `one stop service' possible. As the number of samples increased, the TAT gradually became longer and eventually showed over one hour for almost all items. METHODS: In October 1998, reorganization of the outpatient laboratory took place. Newly introduced were a priority system for samples, a plasma separate tube instead of the serum separate tube, an on-line simultaneous sample reception system at the time of sampling, and a real-time monitoring system for TAT. RESULTS: With the sample priority system, samples processed for one stop service were 61.0% (476 priority samples out of 780 total samples) for routine hematology, 59.2% (527 out of 890) for routine chemistry, 40.1% (122 out of 304) for urinalysis, 43.2% (89 out of 206) for coagulation tests, and 47.9% (75 out of 157) for diabetic tests. We monitored samples processed as `one stop service' with the real time monitoring system for TAT; among the samples processed as such, 80.1% of rou-tine hematology, 91.9% of routine chemistry, 99.5% of urinalysis, 92.6% of coagulation tests, and 97.6% of diabetic tests showed TAT less than one hour. Average TAT, from the specimen acquisition to the reporting, decreased dramatically after introduction of the system. Average TAT of routine hematology decreased to 53.9 minutes, routine chemistry to 54.6 minutes, urinalysis to 35.2 minutes, coagulation tests to 46.6 minutes, and diabetic tests to 31.9 minutes. And the patient satisfaction index for the outpatient laboratory rose 15% from 81% to 96%. CONCLUSIONS: The outpatient laboratory of AMC shortened the TAT substantially without sacrificing quality and fully met the needs of patients and clinicians after reorganization.

Prognostic factors affecting response to chemotherapy and survival duration in Korean patients with multiple myeloma / 대한내과학회지

BACKGROUND: New therapeutic modalities such as high dose chemotherapy and stem cell support have been tried to prolong the survival period of the patients with multiple myeloma. However, little is known about the criteria for the application of those new therapies. There are only a few reports for the prognostic factors of multiple myeloma in Korea. The purpose of this study is to analyze the prognostic factors affecting chemotherapy response and survival in patients with multiple myeloma. METHODS: We retrospectively analysed the clinical records of 122 patients who were newly diagnosed as multiple myeloma by SWOG criteria, between November, 1989 and April, 1997 at Asan Medical Center. RESULTS: 1) The peak incidence was the 7th decade and male to female ratio was 1.3:1. The most common presenting symptom at first diagnosis was bone pain. 2) Initial clinical stage was as followed: stage I in 17.2% , stage II in 16.4% and 66.4% in III. The immunoglobulin classes were IgG in 51.6%, light chain only in 25.4%, IgA in 16.4%, IgD in 4.1%, and non-secretory type in 2.5%. Plasma cell types in bone marrow were classified as plasmablastic type in 45.9%, plasmacytic type in 54.1%. 3) Eighty two patients who recieved chemotherapy more than 3 cycles were evaluable for chemotherapy response. Overall response rate was 69.5%. Factors affecting response to chemotherapy were serum creatinine level, plasma cell type, total plasma cell percentage and plasmablast percentasge of total nucleated cells in bone marrow. 4) For total 122 patients, overall median survival period was 21 months, and estimated 5 year survival rate was 23.5%. Factors affecting survival were serum creatinine, corrected calcium, albumin, beta2-microglobulin level, response to chemotherapy, total plasma cell percentage and plasmablast percentage in bone marrow. CONCLUSION: Bone marrow findings at initial diagnosis are significantly associated with response to chemotherapy and survival duration.

A Case of 13 Ring Chromosome Syndrome

We have experienced a case of 13 ring chromosome in a 40-month-old girl who demonstrated psychomotor retardation with delayed speech, growth retardation, hearing loss(left), microcephaly, trigonocephaly with flat occiput, hypertelorism, epicanthal folds, microophthalmia, broad prominamt nasal bridge, high arched palate, micrognathia, large auricles and other anomalies. Cytogenetic studies of peripheral blood lymphocytes with differential staining of chromosomes revealed 46, XX, r13. Her parents' karyotypes were normal. We reported the case with the review of the associated literatures.