BACKGROUND: Several studies about myelodysplastic syndromes (MDS) have demonstrated that patients with high score of erythrocytic and total dysplasia showed a significantly lower degree of acute myeloid leukemia (AML) development. We analyzed correlation between bone marrow dysplasia and peripheral blood indices, and estimated the value of peripheral blood indices substituted for bone marrow examination to predict the progress of MDS to AML. METHODS: RBC count, MCV, RDW, WBC count, platelet count, MPV, and PDW were measured by Coulter Counter STKS (USA). We calculated the granulation score (G-score), percentage of peudo-pelger polymorphs (PPP) in the peripheral blood film, and examined the dysplasia in bone marrow aspirates. The reticulocyte survival study was performed with the venous blood collected in CPDA-1 under sterile conditions which was incubated immediately after collection at 37degrees C. RESULTS: G-score was inversly correlated with granulocytic and total dysplasia, but highly scored PPP showed a significantly lower degree granulocytic and total dysplasia. Reticulocyte survival curves showed variable pattern according to degree of erythrocytic and total dysplasia. Patients with a high degree of erythrocytic and total dysplasia showed significant difference compared with normal control group. MPV was increased in accordance with increase in megakaryocytic and total dysplasia. A lower score for erythrocytic and total dysplasia was observed in RAEB-t than in RA and RAEB. CONCLUSIONS: It is suggested that G-score, PPP, and MPV in peripheral blood as well as reticulocyte survival curve may be good markers for bone marrow dysplasia, and erythrocytic and total dysplasia in RAEB-t is lower than in RA and RAEB. Therefore, peripheral blood indices can be used to predict the progress of MDS to AML
Anemia, Refractory, with Excess of Blasts ; Bone Marrow Examination ; Bone Marrow* ; Humans ; Leukemia, Myeloid, Acute ; Myelodysplastic Syndromes* ; Platelet Count ; Reticulocytes

Translocations involving chromosome 21q22 are frequently observed in hematologic malignancies including acute myeloid leukemia (AML), most of which have been known to be involved in malignant transformation through transcriptional dysregulation of Runt-related transcription factor 1 (RUNX1) target genes. Nineteen RUNX1 translocational partner genes, at least, have been identified, but not Homeobox A (HOXA) genes so far. We report a novel translocation of RUNX1 into the HOXA gene cluster in a 57-year-old female AML patient who had been diagnosed with myelofibrosis 39 months ahead. G-banding showed 46,XX,t(7;21)(p15;q22). The involvement of RUNX1 and HOXA genes was confirmed by fluorescence in situ hybridization.
Core Binding Factor Alpha 2 Subunit ; Female ; Fluorescence ; Genes, Homeobox ; Hematologic Neoplasms ; Humans ; In Situ Hybridization ; Leukemia, Myeloid, Acute* ; Middle Aged ; Multigene Family* ; Primary Myelofibrosis

Balantidium coli is widely distributed in hogs, particularly in warm and temperate climates, and in monkeys in the tropics. B. coli is the only pathogenic ciliate and is the largest protozoan parasitizing humans. Some individuals with B. coli infections are totally asymptomatic, whereas others have symptoms of severe dysentery similar to those seen in patients with amebiasis. We report a 5-year-old girl with asymptomatic balantidiasis. The patient was suffering from herpes zoster for several days. She did not have symptoms of dysentery or urinary tract infection. Motile trophozoites of B. coli were observed in the urinary sediment. This is the first report of asymptomatic balantidiasis in Korea.
Amebiasis ; Balantidiasis ; Balantidium* ; Child, Preschool ; Climate ; Dysentery ; Female ; Haplorhini ; Herpes Zoster ; Humans ; Korea ; Trophozoites ; Urinary Tract Infections

BACKGROUND: We investigated the significance of plasma neutrophil gelatinase-associated lipocalin (pNGAL) level as an acute-phase reactant and an index for an increase in serum creatinine (sCr) level in patients with inflammatory diseases. METHODS: A total of 63 patients with systemic inflammatory response syndrome (SIRS) and 149 without SIRS were evaluated, and pNGAL level was determined using a fluorescence immunoassay. sCr levels were measured daily during three days, and the difference between the initial and follow-up sCr levels was defined as a delta sCr value. Serum albumin/sCr ratio (sACR) was calculated. High-sensitivity C-reactive protein (hsCRP) level was determined using a latex turbidometric method. RESULTS: The median pNGAL level in the SIRS group (154 ng/mL) was significantly higher than that in the non-SIRS (86 ng/mL) and control (62 ng/mL) groups (P<0.001, respectively). The area under the ROC curve (AUC) of pNGAL for diagnosing SIRS was 0.725 (95% CI, 0.664-0.781), which was not significantly different from that of hsCRP (0.749; 95% CI, 0.685-0.809; P=0.375). Multivariate regression analyses revealed that log-pNGAL was significantly associated with hsCRP (beta=0.546, P<0.001) and sACR (beta=0.351, P<0.001). The AUC of pNGAL for the positive delta sCr in 48-72 hr was 0.649 (95% CI, 0.542-0.746, P=0.023) in the SIRS group. CONCLUSIONS: pNGAL is comparable to hsCRP as an inflammation-related parameter, and its measurement may provide additional information for a potential increase in sCr during 48-72 hr in patients with SIRS.
Area Under Curve ; C-Reactive Protein ; Creatinine ; Fluorescence ; Follow-Up Studies ; Humans ; Immunoassay ; Latex ; Lipocalins* ; Neutrophils* ; Plasma ; ROC Curve ; Systemic Inflammatory Response Syndrome*

A 13-year old girl visited emergency medical center presenting with nasal bleeding and gross hematuria. She had no growth retardation, nor history of abnormal bleeding. Her initial blood test results showed normal platelet counts, normal liver enzyme level but prolonged prothrombin time and activated partial thromboplastin time. On admission, she showed massive but intermittent bleeding until the 15th hospital day. Evaluation including coagulation factor assay was done and the results were compatible with vitamin K deficiency. She was treated with vitamin K intramuscular injection 7 times and intermittent transfusion of red blood cells, platelets and fresh frozen plasma. After that, all of her blood test results returned to normal levels including coagulation tests concomitent with resolving symptoms. In that there were no proof of underlying diseases that can cause vitamin K deficiency, she was diagnosed as idiopathic transient vitamin K deficiency.
Adolescent ; Blood Coagulation Factors ; Emergencies ; Epistaxis ; Erythrocytes ; Female ; Hematologic Tests ; Hematuria ; Hemorrhage ; Humans ; Injections, Intramuscular ; Liver ; Partial Thromboplastin Time ; Plasma ; Platelet Count ; Prothrombin Time ; Vitamin K ; Vitamin K Deficiency

Acquired pure red cell aplasia (PRCA) can be induced by various factors such as viral infection, thymoma, connective tissue disease, lymphoma, and adverse drug reactions. PRCA has not been reported in an adolescent in Korea for the past several decades. We recently experienced a case of acquired PRCA in an adolescent. A 14-year-old girl presented with pallor, dizziness, and mild fever. She had isolated normocytic normochromic anemia with reticulocytopenia in the peripheral blood and erythroblastopenia in the bone marrow. She was diagnosed with secondary acquired PRCA presumably induced by Mycoplasma pneumoniae infection during her clinical course, and she experienced spontaneous remission 11 weeks after initial diagnosis. Her clinical and hematologic statuses were normal as far as 20 months after her diagnosis.
Adolescent ; Anemia ; Bone Marrow ; Connective Tissue Diseases ; Diagnosis ; Dizziness ; Drug-Related Side Effects and Adverse Reactions ; Female ; Fever ; Humans ; Korea ; Lymphoma ; Mycoplasma Infections ; Mycoplasma pneumoniae ; Pallor ; Pneumonia, Mycoplasma ; Red-Cell Aplasia, Pure ; Remission, Spontaneous ; Thymoma

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease. The prognosis is poor in most cases with rapid progression despite administering chemotherapy. A 67-year-old man complained of skin rashes on his back and this spread to the trunk, face, arms and thighs, and he was initially diagnosed with cutaneous lupus erythematosus according to the skin biopsy. The skin rashes then became aggravated on a trial of low dose methylprednisolone for 3 months. Repeated skin biopsy revealed a diffuse infiltration of lymphoid cells with medium sized nuclei, positive for CD4 and CD56, negative for Epstein-Barr virus (EBV), indicating a diagnosis of BPDCN. Further workups confirmed stage IVA BPDCN involving the skin, multiple lymph nodes, the peripheral blood and the bone marrow. He was treated with six cycles of combination chemotherapy consisting of ifosphamide, methotrexate, etoposide, prednisolone and L-asparaginase, and he achieved a partial response. Herein we report on a rare case of BPDCN that was initially misinterpreted as cutaneous lupus erythematosus.
Aged ; Arm ; Biopsy ; Bone Marrow ; Dendritic Cells ; Drug Therapy, Combination ; Etoposide ; Exanthema ; Herpesvirus 4, Human ; Humans ; Lupus Erythematosus, Cutaneous ; Lymph Nodes ; Lymphocytes ; Methotrexate ; Methylprednisolone ; Prednisolone ; Prognosis ; Rare Diseases ; Skin ; Thigh

Idiopathic hyperammonemia is a rare and serious complication of intensive cytoreductive chemotherapy for hematologic malignancies as well as after autologus or allogeneic bone marrow transplantation (BMT). A 42-year-old woman with Philadelphia chromosome positive acute lymphoblastic leukemia was undertaken unrelated BMT. Fourteen days later, the patient developed hallucination, mental confusion, lethargy, incoordination and stupor. Laboratory tests revealed normal serum aminotransferases. The serum ammonia level is moderately increased. Parenteral alimentation was discontinued to lower protein intake and treatment with lactulose, metronidazole, carnitine, and flumazenil was started. Also, hemodialysis was performed. Despite of these appropriate therapies, she died of idiopathic hyperammonenia at twenty-two days after allogeneic BMT.
Adult ; Ammonia ; Ataxia ; Bone Marrow Transplantation* ; Bone Marrow* ; Carnitine ; Drug Therapy ; Female ; Flumazenil ; Hallucinations ; Hematologic Neoplasms ; Humans ; Hyperammonemia* ; Lactulose ; Lethargy ; Metronidazole ; Philadelphia Chromosome ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Renal Dialysis ; Stupor ; Transaminases

BACKGROUND: Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia in its morphology as well as molecular or genetic profiles, conferring a good prognosis owing to the active roles of all-trans-retinoic acid (ATRA) and anthracyclines. METHODS: Patients diagnosed as APL from March 1997 to April 2006 were analyzed on their clinical features, laboratory profiles, methods of treatment including remission induction, consolidation and maintenance, treatment outcomes, and treatment-related morbidity. RESULTS: Chemotherapy naive were all the 12 patients in our study consisting of 3 males and 9 females. All patients showed typical morphologic feature of APL with cytogenetic abnormality, t(15;17), and PML/RAR alpha fusion gene was confirmed in 10 patients by FISH or PCR. The combination of cytarabine with daunorubicin (n=2) or idarubicin (n=9) was used as an induction regimen with concurrent ATRA administration. For consolidation therapy, cytarabine with anthracycline (n=4) or idarubicin monotherapy (n=8) was used with ATRA. Cytogenetic and molecular remissions were documented after induction chemotherapy (n=11) or first consolidation therapy (n=1). Maintenance therapy with ATRA was done in 11 patients. CR was obtained in 12 patients, with median remission duration of 30.5+ months (range 2 to 86+) at a median follow up duration of 33.5+ months (range 4 to 89+). One patient relapsed after completion of maintenance therapy and died of infection during reinduction chemotherapy. CONCLUSION: Herein is the report of ten years' experience of our hospital in the treatment of APL with favorable results as seen by high CR rate and fewer complications.
Anthracyclines ; Chromosome Aberrations ; Cytarabine ; Cytogenetics ; Daunorubicin ; Drug Therapy ; Female ; Follow-Up Studies ; Humans ; Idarubicin ; Induction Chemotherapy ; Leukemia, Myeloid, Acute ; Leukemia, Promyelocytic, Acute* ; Male ; Polymerase Chain Reaction ; Prognosis ; Remission Induction ; Tretinoin

BACKGROUND: Serum insulin-like growth factor-I (IGF-I) and insulin-like growth factor binding protein-3 (IGFBP-3) levels are known markers of growth hormone (GH) secretion. The clinical utility of serum IGF-I and IGFBP-3 testing, however, remains controversial. The aims of this study were to evaluate the usefulness of IGF-I and IGFBP-3 as indicators of GH secretion through the GH stimulation test and to investigate whether a decrease in serum IGF-I levels in children with short stature, regardless of the cause, can be used as a screening test for short stature. METHODS: A total of 262 children presented with short stature, precocious puberty, or premature thelarche and were grouped into 7 tiers based on the 2007 growth chart. Serum IGF-I and IGFBP-3 levels and GH stimulation were analyzed using an immunoradiometric assay, and the data from 68 children who were below the 3rd percentile for height were used to evaluate the usefulness of IGF-I and IGFBP-3 as markers of GH status. RESULTS: GH deficiency was confirmed by the GH stimulation test in 25 of the 68 children, and 15 (15/25, 60%) and 4 (4/25, 16%) of them showed a decrease in IGF-I and IGFBP-3 levels, respectively. The sensitivity and specificity for predicting GH secretion were 60% and 16%, respectively, for IGF-1 and 41.9% and 97.7%, respectively, for IGFBP-3. Decreased serum IGF-I levels were more frequently observed in children below the 25th percentile than in those in the 25th to 95th percentiles. CONCLUSIONS: IGF-I and IGFBP-3 levels have been used as a screening tool for GH secretion in children with short stature, but based on the results of the GH stimulation test in the current study, the levels of IGF-I and IGFBP-3 might not be useful as markers of GH secretion. Evaluating serum IGF-I levels alone is not a sufficient screening test for children with a short stature.
Child* ; Growth Charts ; Growth Hormone ; Humans ; Immunoradiometric Assay ; Insulin-Like Growth Factor Binding Protein 3* ; Insulin-Like Growth Factor I* ; Mass Screening ; Puberty, Precocious ; Sensitivity and Specificity