Erythrophagocytosis by Myeloid Cells in a Patient with Myeloproliferative Disorder

This report documents a case of myeloid erythrophagocytosis in a patient with myeloproliferative disorder. The patient had pancytopenia and his marrow was hyperplastic with erythrophagocytosis by myeloid cells of various stages, including myeloblasts. He was diagnosed to have a prefibrotic stage of chronic idiopathic myelofibrosis. The erythrophagocytosis by myeloid cells persisted even after 2 months of treatment for the primary disorder.

Autoimmune Hemolytic Anemia Predominantly Associated with IgA Anti-E and Anti-c

A patient with warm autoimmune hemolytic anemia (AIHA) due to predominance of immunoglobulin A (IgA) with an Rh specificity, considered to be the first case in Korea, is described. A 13-yr-old male patient with severe hemolytic anemia showed a weak reactivity (1+) in the direct antiglobulin test (DAT) by using anti-IgG antiglobulin reagent. This finding, however, could not fully explain the patient's severe AIHA. When anti-IgA reagent was used for the DAT, strong reactivity (4+) was observed and free anti-E and anti-c autoantibodies were also detected by anti-IgA and anti-IgG reagents. The patient's hemoglobin began to rise with the administration of steroids. Because RBCs coated with multiple types of immunoglobulins are associated with more severe hemolysis than those only with IgG, the DATs using anti-IgA and other reagents are needed for the correct diagnosis when the result of DAT is not compatible with patient's clinical manifestations.

Acute Promyelocytic Leukemia Developed in a Patient with Systemic Lupus Erythematosus / 대한진단검사의학회지

A 19-year-old, woman who had been diagnosed as systemic lupus erythematosus (SLE) a year ago, was admitted because of fever, dizziness, and sustained postoperative bleeding after a hemorroidectomy. On admission, a CBC revealed pancytopenia (Hb 6.2 g/dL, WBC 1,200/microL, platelets 11,000/microL) with a shift to themicroLeft, and the FDP and D-dimer were positive. She was treated for sepsis and disseminated intravascular coagulation. Granulocyte colony-stimulating factor (G-CSF) was administrated twice for severe neutropenia. An increase in WBC and immature myeloid cells, mainly hypergranular promyelocytes on the peripheral blood followed and was considered to be the effect of G-CSF. To evaluate the cause of pulmonary infiltrates, bronchoalveolar lavage (BAL) was performed on the 5th day of admission. The BAL fluid revealed many promyelocytes and myelocytes with occasional structures recognized as Auer rods. Acute promyelocytic leukemia (APL) was confirmed on the bone marrow study and chromosome analysis. Unfortunately, the patient died of septic shock on the 9th day of admission. We report here a very rare case of APL diagnosed in a SLE patient, the diagnosis of which was somewhat delayed due to the use of G-CSF and superimposed sepsis.

Blood Use According to Diagnoses in Hospitalized Adults of Ajou University Hospital / 대한임상병리학회지

BACKGROUND: There is paucity of data on the use of blood and its products with regard to the diagnoses of recipients in Korea. The objective of this study is to report the characteristics of the recipients and the usage in relation to diagnoses among Koreans. METHODS: We assessed the blood usage of adult patients (18 years or older) in a tertiary teaching hospital during the past 2 years (1998-2000). The red blood cells (RBCs), fresh frozen plasmas (FFPs) and platelets (PLTs, platelet concentrates and apheresis platelets) were evaluated in relation to the characteristics of the recipients and the discharge diagnoses according to the International Classification of Diseases (10th Ed). Data were extracted from the hospital information system. RESULTS: Approximately twenty percent of the hospitalized patients were transfused. RBCs, FFPs and PLTs were transfused 18.9%, 4.8% and 3.2% of hospitalized patients, respectively. Fifty-six percent of 54,049 RBCs and 64.9% of 19,549 FFPs were transfused in the patients with nonhematological neoplasms, disorders of the digestive system, injury and poisoning. Sixty-two percent of 50,621 PLTs were transfused in the patients with hematological and non-hematological neoplasms and disorders of the digestive system. CONCLUSIONS: This survey showed the trends of the transfusion practice as different from those for Caucasians and the usage of FFPs and PLTs was restricted for some recipients. These results could help in predicting the blood needs and medical costs for a variety of patients.

A Case of Juvenile Myelomonocytic Leukemia in a Six Months Old Infant / 대한혈액학회지

Myelodysplastic syndrome (MDS) in childhood is a rare hematologic malignancy and its classification has been the subject of some controversy. Cases of pediatric MDS are subdivided into those with features of adult-type MDS and those with myeloproliferative features occasionally observed in infancy and early childhood. There appears to be an international consensus to rename the disease juvenile myelomonocytic leukemia (JMML), which includes all leukemias of childhood previously classed as chronic myelomonocytic leukemia (CMML), juvenile chronic myelogenous leukemia (JCML), and infantile monosomy 7 syndrome. We experienced a 6-month-old female infant with JMML who developed extensive extramedullary hematopoiesis. The patient developed abdominal distention, hepatosplenome-galy, anemia, thrombocytopenia, and leukocytosis with significant monocytosis and was found to have a high hemoglobin F level of 30%. Her bone marrow biopsy section and aspirate smears revealed normocellularity with no increment of blast cells and no dysplastic changes. Cytogenetic analysis revealed a normal 46, XX karyotype. Her liver, spleen, lymph nodes, and appendix were found to be heavily infiltrated by partially differentiated myelomonocytic cells. These findings supported the diagnosis of JMML with extensive extramedullary hematopoiesis.

Clinical Significance of p53, MDM2 and Cyclin D1 Protein Expression in Patients with Multiple Myeloma / 대한혈액학회지

BACKGROUND: Dysregulation of cell proliferation contributes to the pathogenesis of multiple myeloma (MM) and the number of S phase plasma cells is known to be one of the most important prognostic factors in MM. We analysed the cell cycle progression in MM using the expression of G1/S phase cell cycle regulators, such as p53, murine double minutes (MDM2) and cyclin D1. METHODS: The expressions of p53, MDM2 and cyclin D1 were evaluated by immunohistochemical staining with monoclonal antibodies, using bone marrow sections obtained from 48 patients with MM and 20 normal controls. RESULTS: The expressions of p53, MDM2 and cyclin D1 were demonstrated in 17 (35.4 %), 40 (83.3%) and 28 (58.3%) of 48 patients with MM, respectively. The expressions of cyclin D1 and p53 were positively correlated each other (P<0.05). However, no significant difference in MDM2 expression was found between the cyclin D1-positive and -negative groups. All of the control group showed negative expression. The expression of cyclin D1 and p53 in patients with MM correlated well with clinical and histologic stages (P<0.05). Even if MDM2 was upregulated in most patients with MM, no correlation was found with clinical or histologic stages. Serum beta2-microglobulin levels were reversely correlated with p53 expression, not with MDM2 or cyclin D1. After chemotherapy, all 5 patients with objective response showed decreased staining of these three proteins, comparing 10 of 13 patients with partial response or treatment failure showed no change or an increased degree of staining. No differences were observed in the survival rates between the groups with and without expression of each three proteins. CONCLUSIONS: These findings suggest that the expression of p53, MDM2 and cyclin D1 was increased in patients with MM and the expression rates of p53 and cyclin D1 were increased with the progression of the clinical and histological stages. It is considered that the detection of cell cycle regulatory proteins are important for understanding the biology of the malignant plasma cells, monitoring the results of treatment and determining the prognosis in MM.

Lactic Acidosis Associated with Acute Lymphoblastic Leukemia / 대한신장학회잡지

Occurence of lactic acidosis with adequate tissue oxygenation(type B lactic acidosis) has been described in association with leukemia, lymphoma, small cell carcinoma and breast cancer. However, no such case has been reported in Korea. Therefore, we report a case of type B lactic acidosis in a man with rapidly progressing acute lymphoblastic leukemia which had been transformed from lymphoma.

A Comparative Study of Biological and Analytical Variabilities in Automated Blood Cell Analysis / 대한임상병리학회지

BACKGROUND: The National Committee for Clinical Laboratory Standards (NCCLS) recommends that the analytical variability must not exceed 25% of the biological variability in automated blood cell analysis. This study was conducted to determine whether routine automated blood cell analysis by Coulter STKS (Coulter Corp., Miami, FL, U.S.A) comforms with the NCCLS's recommendations. METHODS: Routine CBC analysis with STKS was performed on 22 healthy volunteers. The tests included calculating WBC, RBC, hemoglobin, MCV, platelet, MPV, and percentages of the granulocytes, lymphocytes, and monocytes. Blood samples were collected twice in one week interval to study the total variability. For the analytical variability, blood samples from 12 subjects were tested twice immediately after venipuncture for within-run variability, and samples from 10 subjects were tested immediately and 6 hours after venipuncture for within-day variability. The analytical variability was calculated as the sum of within-run and within-day variabilities. The biological variability was calculated by subtracting the analytical variability from total variability. The ratios of analytical and biological variabilities were calculated by dividing the analytical variability by the biological variability. RESULTS: Ratios of analytical and biological variabilities were as follows: 0.22 for WBC, 0.20 for RBC, 0.21 for hemoglobin, 0.39 for platelet, 1.98 for MPV, 0.07 for %granulocyte, 0.11 for %lymphocyte, and 1.81 for %monocyte. The ratio for MCV was not obtained because the analytical variability exceeded total variability. CONCLUSIONS: The analytical variability did not exceed 25% of the biological variability in all test categories except platelet, MPV and the percentage of monocyte. Thus, it is recommended that the analytic variability of all test categories be reduced so as to be in conformity with the NCCLS' recommendations.

Bone Marrow Examination: Adventures in Diagnostic Hematology

No abstract available.