1.A Case of Neutrophilic Eccrine Hidradenitis.
Joo Won KIM ; Ki Sung KIM ; Chong Hyeok KIM ; Chil Wan OH
Korean Journal of Dermatology 2000;38(8):1121-1123
Neutrophilic eccrine hidradenitis(NEH) is a self-limited inflammatory dermatosis primarily induced by chemotherapeutic agents. The pathogenesis and possible cause of NEH remain unknown. NEH may represent as a reaction pattern to chemotherapeutic agents or bacterial infection. Also it is developed on palmoplantar areas in healthy children or young adults. In some cases it is developed as a paraneoplastic phenomenon. We report a case of neutrophilic eccrine hidradenitis that developed in a patient suffering from aplastic anemia after or during treatment with cyclosporine, oxymetholone and folic acid.
Anemia, Aplastic
;
Bacterial Infections
;
Child
;
Cyclosporine
;
Folic Acid
;
Hidradenitis*
;
Humans
;
Neutrophils*
;
Oxymetholone
;
Skin Diseases
;
Young Adult
2.Dyskeratosis Congenita in a Girl.
Jun Sun YI ; Hoon KOOK ; Hee Jo PAIK ; So Youn KIM ; Ik Sun CHOI ; Suk Joo KIM ; Kyoung Ran SOHN ; Ho Song NAM ; Tai Ju HWANG
Korean Journal of Pediatric Hematology-Oncology 2003;10(2):293-298
Dyskeratosis congenita (DC) is a rare genetic disorder encompassing abnormal skin pigmentation, dystrophic nails, leukoplakia of mucous membranes and others. Bone marrow failure is the cause of early mortality. Moreover, DC is known for its predisposition to malignancy. X-linked recessive, autosomal dominant and autosomal recessive forms of the disease are recognized. We describe here a rare case of DC in a 4-year-old girl showing dark skin, dystrophic toe nails, and mild bone marrow failure. Autosomal recessive disease was suggested as the patient is female, and tests for DKC1 and hTR mutations were negative. Intermittent treatment with oxymetholone and prednisolone for about 26 months resulted in stable hemoglobin and platelet response.
Blood Platelets
;
Bone Marrow
;
Child, Preschool
;
Dyskeratosis Congenita*
;
Female*
;
Humans
;
Leukoplakia
;
Mortality
;
Mucous Membrane
;
Oxymetholone
;
Prednisolone
;
Skin
;
Skin Pigmentation
;
Toes
3.Aplastic Anemia Associated with Stomach.
Jee Sook HAHN ; Doe Young KIM ; Dong Ki NAM ; Young Sik LEE ; Sun Ju LEE ; Yun Woong KO ; Chang Hwan CHO
Yonsei Medical Journal 1988;29(3):263-269
Herein is presented a case of aplastic anemia associated with adenocarcinoma of the stomach which seem- ed to be coincidental. A 52 year-old man was admitted with a 3 year history of dyspnea. Three years previously, he was diagnosed as bone marrow hypoplasia and had been treated with oxymetholone for 1 year. After confirmation of aplastic anemia during the first admission, he was followed up with fluoxymesterone and steriods. One year later, he was readmitted with melena. Fibergastroscopy and an UGl study revealed a fungating mass on the antrum suggestive of stomach cancer. Following perioperative platelet transfusions and intensive supportive care, a subtotal gastrectomy was performed and there were no postoperative complications. Pathologic examinations disclosed a moderately well differentiated adenocarcinoma. This is the first report in Korea of adenocarcinoma of the stomach occurring in a patient with aplastic anemia. He survived 17.5 months after the surgery and 5.4 years after the onset of aplastic anemia. Gastrointestinal bleeding in aplastic anemia may be incorrectly ascribed to steriod use and overlooked, thus the need to fully investigate gastric pathology by endoscopy as well as radiology is streesed. In a patient with pancytopenia, the major surgical procedures are frequently evaded by both surgeons and internists due to the possibility of morbidity from bleeding and infection. In this case, intensive perioperative supportive care and surgery were combined to prolong the patient's survival time.
Adenocarcinoma/*complications/secondary/surgery
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Anemia, Aplastic/*complications/drug therapy
;
Case Report
;
Human
;
Male
;
Middle Age
;
Oxymetholone/therapeutic use
;
Stomach Neoplasms/*complications/surgery
4.Colonic Angioectasia in an Adolescent Boy with Hoyeraal-Hreidarsson on Long-Term Anabolic Steroid Therapy
Pediatric Gastroenterology, Hepatology & Nutrition 2018;21(1):68-71
Androgen therapy has proven efficacy in treating patients with bone marrow failure who are not candidates for bone marrow transplantation. Herein, we report on a case of colonic angioectasia secondary to oxymetholone use in an adolescent patient with Hoyeraal-Hreidarsson syndrome (HHS). A 13-year-old Caucasian male with HHS characterized by cerebellar hypoplasia, developmental delay, microcephaly, esophageal strictures and myelodysplasia presented with severe hematochezia from colonic angioectasia secondary to long-term oxymetholone therapy. These vascular lesions resolved spontaneously once this anabolic steroid was discontinued. While androgen therapy is often recommended for certain anemias and myelodysplastic syndromes, clinicians should be aware of the potential complication in developing these perceived uncommon colonic angioectasias. Moreover, pediatric gastroenterologists should familiarize themselves in identifying these vascular lesions by colonoscopy, especially among the high risk groups on long-term anabolic steroid therapy.
Adolescent
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Anemia
;
Bone Marrow
;
Bone Marrow Transplantation
;
Colon
;
Colonoscopy
;
Constriction, Pathologic
;
Gastrointestinal Hemorrhage
;
Humans
;
Male
;
Microcephaly
;
Myelodysplastic Syndromes
;
Oxymetholone
;
Testosterone Congeners
5.Infant with Fanconi Anemia Presenting with Myelodysplastic Syndrome.
Tae Hyung CHO ; Hoon KOOK ; Na Eun RYU ; Chang Jong KIM ; Jae Hyuk LEE ; Tai Ju HWANG
Korean Journal of Pediatric Hematology-Oncology 1998;5(2):322-327
PURPOSE: Fanconi anemia(FA) is a rare autosomal recessive disorder characterized by progressive bone marrow failure and congenital malformations. Patients with FA have aplastic anemia(> 90%), leukemia(10~15%), myelodysplasia(5%) and liver(5%) and other tumors(5%). In the International FA Registry study myelodysplasia in FA patients was detected at a median of 13 years. Presentation of FA with myelodysplasia in an infant should be extremely rare. CASE: A 3-month-old infant presented with anemia and poor feeding. The initial hemogram showed: hemoglobin, 4.6 g/dL; MCV, 104.1 fL/pg; white cell count, 4,300/microL; neutrophils, 450/microL; platelets, 23,000/microL. The bone marrow was normocellular, with findings of macrocytic anemia and dyserythropoiesis, and less than 5% of myeloid blasts, compatible with myelodysplastic syndrome(refractory anemia). The patient had multiple cafe-au-lait spots, hypopigmented nevi, broad nasal bridge, micrognathia, and thumb and toe anomalies. FA was confirmed by chromosomal hypersensitivity to diepoxybutane and mitomicin C. Supportive treatment with oxymetholone and prednisolone failed to improve hematologic and clinical findings. The patient succumbed to sepsis, pneumonia and meningitis due to Pseudomonas aeruginosa at 20 month of age. Clonal cytogenetic anomalies were not found. CONCLUSION: We reported here a rare case of FA presenting with myelodysplasia at the age of 3 month.
Anemia
;
Anemia, Macrocytic
;
Bone Marrow
;
Cafe-au-Lait Spots
;
Cell Count
;
Cytogenetics
;
Fanconi Anemia*
;
Humans
;
Hypersensitivity
;
Infant*
;
Meningitis
;
Myelodysplastic Syndromes*
;
Neutrophils
;
Nevus
;
Oxymetholone
;
Pneumonia
;
Prednisolone
;
Pseudomonas aeruginosa
;
Sepsis
;
Thumb
;
Toes
6.A Clinical Study of Fanconi's Anemia.
Sung Ho CHO ; Hoon KOOK ; Geun Mo KIM ; Won Sang YOON ; Tae Hyung CHO ; Tai Ju HWANG
Korean Journal of Pediatric Hematology-Oncology 1997;4(1):70-77
BACKGROUND: Fanconi's anemia(FA) is an autosomal recessive disease characterized by aplastic anemia and congenital malformations. As up to 30% of patients have no physical stigmata, the modern diagnosis of FA rests on chromosomal breakage of patient's cells induced by chemical clastogens such as diepoxybutane(DEB) or mitomycin-C(MMC). METHODS: We reviewed the clinical manifestations, laboratory findings, diagnostic methods, treatment and outcome of 6 patients diagnosed to have a FA at the Chonnam University Hospital for the last 6 years. RESULTS: Six cases(16.2 %) were found to have FA among 37 aplastic children who were diagnosed during the same period. The mean age at diagnosis was 6.3 years which was the usual onset of hematologic findings. All patients had features of aplastic anemia, and had one or more anomalies, such as low birth weight, hyperpigmentation, cafeau-lait spots, mental retardation, developmental delay, peculiar face(broad nasal bases, epicanthal folds, micrognathia), polydactyly, microcephaly, short stature, and dislocation of hip. We found increased breaks in cultured cells with DEB and MMC in 5 cases tested. The median duration of follow-up was 30 months. Oxymetholone and prednisolone treatment was partially beneficial in three cases. Immunosuppressive treatment with ALG/ATG was not successful in two cases tried. Four cases are living now, without transfusion in three. Two patients were died of disseminated fungal infection and transplant-related problems, respectively. CONCLUSIONS: Fanconi's anemia should be sought carefully in any patients with aplastic anemia because the prognosis, treatment modality, and the approach to bone marrow transplantation are quite different when the hematologic disorder is inherited rather than acquired.
Anemia, Aplastic
;
Bone Marrow Transplantation
;
Cells, Cultured
;
Child
;
Christianity
;
Chromosome Breakage
;
Diagnosis
;
Dislocations
;
Fanconi Anemia*
;
Follow-Up Studies
;
Hip
;
Humans
;
Hyperpigmentation
;
Infant, Low Birth Weight
;
Infant, Newborn
;
Intellectual Disability
;
Jeollanam-do
;
Microcephaly
;
Mitomycin
;
Mutagens
;
Oxymetholone
;
Polydactyly
;
Prednisolone
;
Prognosis
7.Reassessment of a Dup (1)(q21q32), Trp (1)(q21q32) in a Case of Myelodysplastic Syndrome by CGH (Comparative Genomic Hybridization).
Jong Rak CHOI ; Kyung A LEE ; Quehn PARK ; Kyung Soon SONG ; Yun Woong KO
Korean Journal of Hematology 1998;33(1):110-116
Acquired partial duplication or triplication of the long arm of human chromosome 1 has been observed rarely in myelodysplastic syndrome (MDS). We describe a dup (1)(q21q32), trp (1)(q21q32) in a patient with refractory anemia of MDS. A 51-year-old man was admitted for dyspnea. Five months ago, he was diagnosed with myelodysplastic syndrome (RA) and iron deficiency anemia and had been treated with iron, vitamin B12, oxymetholone, and prednisolone. The karyotype of trypsin-Giemsa-banded metaphase cells obtained from bone marrow aspirates was 46, XY, dup (1)(q21q32)x2[5]/46,XY,trp (1)(q21q32)[2]/46, XY, dup (1)(q21q32), trp (1)(q21q32)[2] and confirmed the amplification of 1q21-1q32 by CGH (comparative genomic hybridization). In this assay, test and reference DNAs are labeled with FITC and Texas Red, respectively and co-hybridized to normal metaphase chromosomes. Ratio profiles of each individual chromosome were analyzed using a Quips-XL software (Vysis, Downers Grove, IL, USA). The thresholds of gain and loss were defined 1.2 and 0.8, respectively.
Anemia, Iron-Deficiency
;
Anemia, Refractory
;
Arm
;
Bone Marrow
;
Chromosomes, Human
;
Comparative Genomic Hybridization
;
DNA
;
Dyspnea
;
Fluorescein-5-isothiocyanate
;
Humans
;
Iron
;
Karyotype
;
Metaphase
;
Middle Aged
;
Myelodysplastic Syndromes*
;
Oxymetholone
;
Prednisolone
;
Texas
;
Vitamin B 12
8.Improvement in Erythropoieis-stimulating Agent-induced Pure Red-cell Aplasia by Introduction of Darbepoetin-alpha When the Anti-erythropoietin Antibody Titer Declines Spontaneously.
Hajeong LEE ; Jaeseok YANG ; Hyosang KIM ; Ju Won KWON ; Kook Hwan OH ; Kwon Wook JOO ; Yon Su KIM ; Curie AHN ; Jin Suk HAN ; Suhnggwon KIM
Journal of Korean Medical Science 2010;25(11):1676-1679
Anti-erythropoietin antibodies usually cross-react with all kinds of recombinant erythropoietins; therefore, erythropoiesis-stimulating agent (ESA)-induced pure red-cell aplasia (PRCA) is not rescued by different ESAs. Here, we present a case of ESA-induced PRCA in a 36-yr-old woman with chronic kidney disease, whose anemic condition improved following reintroduction of darbepoetin-alpha. The patient developed progressive, severe anemia after the use of erythropoietin-alpha. As the anemia did not improve after the administration of either other erythropoietin-alpha products or erythropoietin-beta, all ESAs were discontinued. Oxymetholone therapy failed to improve the transfusion-dependent anemia and a rechallenge with ESAs continuously failed to obtain a sustained response. However, her anemia improved following reintroduction of darbepoetin-alpha at 3 yr after the initial diagnosis. Interestingly, anti-erythropoietin antibodies were still detectable, although their concentration was too low for titration. In conclusion, darbepoetin-alpha can improve ESA-induced PRCA when the anti-erythropoietin antibody titer declines and its neutralizing capacity is lost.
Adult
;
Anemia/drug therapy/etiology
;
Antibodies/*blood/immunology
;
Bone Marrow Cells/pathology
;
Drug Hypersensitivity/immunology
;
Erythropoietin/*analogs & derivatives/therapeutic use
;
Erythropoietin, Recombinant/adverse effects/*immunology/therapeutic use
;
Female
;
Glomerulonephritis, IGA/complications
;
Hematinics/adverse effects/immunology/*therapeutic use
;
Humans
;
Kidney Failure, Chronic/complications
;
Oxymetholone/therapeutic use
;
Red-Cell Aplasia, Pure/chemically induced/*drug therapy/immunology
9.Direct and Indirect Effects of Androgens on Survival of Hematopoietic Progenitor Cells In Vitro.
Seong Woo KIM ; Jin Hee HWANG ; Jae Min CHEON ; Nam Sook PARK ; Sang Eun PARK ; Su Jin PARK ; Hwan Jung YUN ; Samyong KIM ; Deog Yeon JO
Journal of Korean Medical Science 2005;20(3):409-416
Androgens remain a common treatment for certain type of anemia, based upon its myelostimulating effects; however, it has not been established whether androgens affect apoptosis of hematopoietic progenitor cells (HPCs). We investigated the effects of the androgens, such as testosterone, 5beta-dihydrotestosterone (5-DHT), and oxymetholone, on apoptosis of normal hematopoietic progenitor cells in vitro. Androgens did not rescue normal bone marrow (BM) CD34+ cells and colony-forming cells (CFCs), other than mature erythroid CFCs, from apoptosis induced by serum- and growth factor deprivation. Oxymetholone did not affect growth factor-mediated survival of normal CD34+ cells or its inhibition by interferon-gamma (IFN-gamma). In a standard methylcellulose clonogenic assay, low concentrations of oxymetholone and 5-DHT stimulated the clonal growth of colony-forming unit (CFU)-erythroid, but did not affect growth of CFU-granulocyte/macrophage or burst-forming unit-erythroid. Oxymetholone and 5-DHT stimulated the production of stem cell factor in normal bone marrow stromal cells (BMSCs) via transcriptional regulation. In agreement with this, oxymetholone-treated BMSCs better supported the survival of HPCs. These data indicate that survival-enhancing or growth-stimulatory effects of androgens on hematopoietic progenitor cells are minimal and mostly restricted to mature erythroid progenitors, and its myelostimulating effects could be attributed, at least in part, to the stimulation of production of hematopoietic growth factors in BMSCs.
Androgens/*pharmacology
;
Antigens, CD34/analysis
;
Apoptosis/drug effects
;
Blotting, Northern
;
Blotting, Western
;
Bone Marrow Cells/cytology/drug effects/immunology
;
Cell Survival/drug effects
;
Cells, Cultured
;
Chemokines, CXC/genetics/metabolism
;
Colony-Forming Units Assay
;
Cytokines/genetics/pharmacology
;
Dihydrotestosterone/pharmacology
;
Dose-Response Relationship, Drug
;
Flow Cytometry
;
Gene Expression/drug effects
;
Hematopoietic Stem Cells/cytology/*drug effects/metabolism
;
Humans
;
Oxymetholone/pharmacology
;
RNA, Messenger/genetics/metabolism
;
Research Support, Non-U.S. Gov't
;
Reverse Transcriptase Polymerase Chain Reaction
;
Testosterone/pharmacology
;
Time Factors