• The Korean Journal of Nuclear Medicine
• /
• v.11 no.1
• /
• pp.71-75
• /
• 1977

A case is reported of aplastic anemia occuring during Methimazole therapy. Since this seems to be a kind of self-limiting disease having considerably rapid recovery, much more detailed supportive therapy is considered to be crucial rather than any other corticosteroid or the androgen therapy.

• The Korean Nurse
• /
• v.26 no.3 s.141
• /
• pp.74-82
• /
• 1987

• 대한핵의학회:학술대회논문집
• /
• 1966.11a
• /
• pp.90.3-91
• /
• 1966

• Clinical and Experimental Pediatrics
• /
• v.46 no.11
• /
• pp.1139-1142
• /
• 2003

Human parvovirus(HPV) B19 infection causes erythema infectiosum in children, sometimes red cell aplastic crisis with hemolytic anemia and chronic bone marrow failure in immunocompromised hosts. HPV B19 is directly cytotoxic for erythroid progenitor cells and inhibits erythropoiesis. Infrequently, HPV B19 inhibits hematopoiesis of three cell lineages and causes transient pancytopenia in patients with hemolytic disorders. We report three patients with hereditary spherocytosis who developed transient aplastic crisis. A HPV B19 infection was confirmed by IgM anti-B19 parvovirus titers and characteristic findings of bone marrow examination as the causative agent associated with severe pancytopenia. Three patients recovered spontaneously after a short period of supportive care with red cell transfusions and intravenous immunoglobulin.

• Tuberculosis and Respiratory Diseases
• /
• v.67 no.4
• /
• pp.345-350
• /
• 2009

Erdheim-Chester disease (ECD) is a proliferative non-Langerhans cell histiocytosis of multiple organs. This is a rare disease of unknown etiology with a high mortality. We present the case report of a 26-year-old man diagnosed with ECD. He was referred to our hospital with elevated levels of aminotransferases. Although the diagnosis was uncertain, the patient was lost to follow up at that time. One year later, the patient returned to the hospital with generalized edema. Although a specific bone lesion was not found, the patient was experiencing the following: glomerulonephritis, aplastic anemia, hepatitis, and lung involvement. A lung biopsy was performed: the immunohistochemical stain were positive for CD68 and negative for S-100 protein and CD1a. We diagnosed as the patient as havinf ECD. Approximately 50% of ECD cases present with extraskeletal involvement. ECD should be considered as part of the differential diagnosis when multiple organs are involved.

• The Journal of Internal Korean Medicine
• /
• v.23 no.2
• /
• pp.212-221
• /
• 2002

Objectives : Acording to the fact that Juakium has been frequently used in a lot of prescriptions to tonify bone marrow, We examined the hematopoietic effects of Juakium-derivative on aplastic anemia. Methods : After C57BL/6 mice were oral administrated with Juakium-derivative and injected with benzene, we counted the number of WBC, RBC, hemoglobin, platelet, nucleated cells and other erythroid parameters and weighed the spleen. Also we measured the expression of $CD34^+$ cell activity and analyzed the bone marrow tissue and spleen tissue histologically. Results : The results are summerized as follow: 1. The Juakium-derivative plus benzene group showed the improvement in the number of WBC, RBC, hemoglobin. platelet, nucleated cells and other erythroid parameters, compared with the benzene only group. 2. The spleen weight and the percent of $CD34^+$ cell of Juakium-derivative plus benzene group was higher, compared with the benzene only group. 3. The bone marrow tissue and spleen tissue of the Juakium-derivative plus benzene group showed the decrease of the infarcted area compared with the benzene only group. Conclusions : These results suggest that Juakium-derivative has hematopoietic effects on aplastic anemia induced by benzene through increasing the blood cells and stimulating the activity of $CD34^+$ cells. Therefore it is expected that Juakium-derivative can be used clinically to the patient with hematopoietic system disorder.

• Radiation Oncology Journal
• /
• v.30 no.4
• /
• pp.165-172
• /
• 2012

Purpose: To retrospectively evaluate the outcome and toxicity of total lymphoid irradiation (TLI) based conditioning regimen for allogeneic hematopoietic stem cell transplantation (HSCT) in severe aplastic anemia (SAA) patients who experienced an engraftment failure from prior HSCT or were heavily transfused. Materials and Methods: Between 1995 and 2006, 20 SAA patients received TLI for conditioning of HSCT. All patients were multi-transfused or had long duration of disease. Fifteen (75%) patients had graft failure from prior HSCT. In 18 (90%) patients, the donors were human leukocyte antigen identical siblings. The stem cell source was the peripheral blood stem cell in 15 (75%) patients. The conditioning regimen was composed of antithymocyte globulin plus TLI with a median dose of 750 cGy in 1 fraction. The graft-versus-host disease (GVHD) prophylaxis used cyclosporine with methotrexate. Results: With a median follow-up of 10.8 years, graft failures developed in 6 patients. Among them, 3 patients received their third HSCT to be engrafted finally. The Kaplan-Meier overall survival rate was 85.0% and 83.1% at 5 and 10 years, respectively. The incidence of acute and chronic GVHD was 20% and 20%, respectively. None of the patients have developed a malignancy after HSCT. Conclusion: In our study, TLI based conditioning in allogeneic HSCT was feasible with acceptable rates of GVHD in SAA patients who experienced graft failure from prior HSCT or was at a high risk of graft rejection. We achieved relatively better results of engraftment and survival with a long term follow-up.

• Clinical and Experimental Pediatrics
• /
• v.58 no.6
• /
• pp.199-205
• /
• 2015

Severe aplastic anemia (SAA) is a life-threatening disorder for which allogeneic hematopoietic stem cell transplantation (HSCT) is the current available curative treatment. HSCT from matched sibling donors (MSDs) is the preferred therapy for children with acquired SAA. For patients who lack MSDs, immunosuppressive therapy (IST) is widely accepted as a first-line treatment before considering HCT from an unrelated donor (URD). Given the recent progress in HSCT using URDs for childhood SAA, well-matched URDs became a realistic alternative for pediatric patients who have no suitable related donors and who are refractory to IST. However, it is quite challenging to treat patients with refractory SAA who lack suitable related or URDs. Even though haploidentical HSCT from genetically mismatched family members seemed to be an attractive procedure with the amazing benefit of readily available donors for most patients, early attempts were disappointing because of refractory graft-versus-host disease (GVHD) and excessively high transplant-related mortality. Recent advances with effective ex vivo depletion of T cells or unmanipulated in vivo regulation of T cells, better supportive care, and optimal conditioning regimens have significantly improved the outcome of haploidentical transplant. Besides considerable progress in the treatment of malignant diseases, recent emerging evidences for haploidentical HSCT in SAA has provided additional therapeutic options for patients with refractory diseases. Further improvements to decrease the rates of graft failure, GVHD, and infectious complications will facilitate the emergence of haploidentical HSCT as a front-line therapy for treating acquired SAA in children and adolescents who have no suitably matched donors.

• Clinical and Experimental Pediatrics
• /
• v.58 no.7
• /
• pp.267-269
• /
• 2015

Antithymocyte globulin (ATG) is used as an immunosuppressive treatment (IST) to deplete clonal suppressor T cells in patients with severe aplastic anemia (SAA). The depletion of suppressor T cells by ATG may affect the activation of B cells, which results in an increased risk for autoimmune conditions. A 12-year-old boy was diagnosed with idiopathic SAA. As he did not have an human leukocyte antigen-matched sibling, he was treated with rabbit ATG (3.5 mg/kg/day for 5 days) and cyclosporine. Five months later, he became transfusion independent. However, 23 months after IST, he complained of mild hand tremors, sweating, weight loss, palpitations, and goiter. Results of thyroid function tests revealed hyperthyroidism (free thyroxine, 3.42 ng/dL; thyroid stimulating hormone [TSH], <0.01 nIU/mL; triiodothyronine, 3.99 ng/mL). Results of tests for autoantibodies were positive for the antimicrosome antibody and TSH-binding inhibitory immunoglobulin, but negative for the antithyroglobulin antibody and antinuclear antibody. He was treated with methimazole, and his symptoms improved. The patient has been disease free for 39 months after IST and 9 months after methimazole treatment. This case report suggests that although rare, rabbit ATG may have implications in the pathogenesis of autoimmune hyperthyroidism. Our findings suggest that thyroid function tests should be incorporated in the routine follow-up of SAA patients treated with ATG.

• Journal of The Korean Dental Society of Anesthesiology
• /
• v.13 no.4
• /
• pp.203-207
• /
• 2013

Aplastic anemia (AA) is a serious hematologic disease characterized by hypocellular bone marrow and deficient production of erythrocytes, granulocytes and platelets. Serious complications such as uncontrolled bleeding and bacteremia can occur. A case of severe AA are presented with dental considerations. A 4-year-old boy had been referred from Seoul National University Hospital for dental examination before the hematopoietic stem cell transplantation (HSCT). Treatments were planned under general anesthesia, due to the poor compliance. Following medical consult, dental treatments were performed after platelet transfusion and antibiotic prophylaxis. Postoperatively, neither significant bleeding nor complictation was observed. On the time of the treatment planning. the anesthesiologist and dentist should perform a complete hematological assessment. It is imperative not only platelet counts but also other leukocyte counts are under safe boundaries. It is mandatory to follow strict aseptic precautios for all anesthetic and surgical maneuvers. In severe thrombocytopenic patients, platelet transfusion should be considered. Also, it is recommended to establish a good oral hygiene.