• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제27권5호
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• pp.464-467
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• 2001

Aplastic anemia is a hematopoietic disorder characterized by marked reduction or absence of erythoid, granulocytic, and megakariocytic cells in the bone marrow with resultant pancytopenia. To control of infection & bleeding secondary to leukopenia and thrombocytopenia, the inflammatory lesions in oral & maxillofacial area should be removed. Material & Method: The extractions were performed on 21 patients with severe aplastic anemia. The initial, pre-operative and postoperative CBCs were checked up. Amount and kind of transfused platelet in each patient and increment of platelet level were recorded. The complications were documented. Result : A mean of 2.9 teeth were extracted from each patient(ranging between 1 and 13). Furthermore, surgical extractions including ostectomy and odontectomy of the third molar were performed on 11 patients. The preoperative WBC levels presented between $600/{\mu}L$ and $5000/{\mu}L$(mean $2376/{\mu}L$). The WBC values decreased by an average of $145/{\mu}L$ per patient after extractions had been performed. The teeth of 16 patients were extracted under 10.0g/dL, and the mean change in postoperative hemoglobin levels in comparison with preoperative hemoglobin levels was -0.06 per patient. The initial platelet levels were between 1000/(L and $81,000/{\mu}L$(mean $20,174/{\mu}L$). In five patients, extractions were performed with platelet levels less than $50,000/{\mu}L$. Conclusion : The results suggest that more active and preventive treatments in the oral and maxillofacial area are possible and are necessary to remove the infectious foci on the patients with severe aplastic anemia. We report the results of our experiences and literature reviews in treatment of the patients with severe aplastic anemia in our department.

• Natural Product Sciences
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• 제25권1호
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• pp.59-63
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• 2019

Hematopoiesis has a pivotal role in the maintenance of body homeostasis. Ironically, several hematological disorder caused by chemicals, drugs, and other environmental factors lead to severe bone marrow failure. Current treatments like stem cell transplantation and immunosuppression remain ineffective to ameliorate this diseases. Therefore, a newtreatment to overcome this entity is necessary, one of them by promoting the usage of medicinal plants. Thus, this study aimed to evaluate the hematopoiesis potency of S. javanica berries and leaves extracts in chloramphenicol (CMP)-induced aplastic anemia mice model. In this present study, several types of blood progenitor cell such as $TER-119^+VLA-4^+$ erythrocytes lineage, $Gr-1^+$ granulocytes, and $B220^+$ B-cell progenitor cells were evaluated by flow cytometry analysis. Accordingly, we revealed that S. javanica berries and leaves extracts significantly promoted $TER-119^+VLA-4^+$ erythrocytes lineage and $Gr-1^+$ granulocytes after exposed by CMP. Thus, these results suggested that S. javanica berries and leaves extracts might have hematopoiesis activity in CMP-induced aplastic anemia mice model.

• Clinical and Experimental Pediatrics
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• 제50권6호
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• pp.519-523
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• 2007

Aplastic anemia is a rare disease, which is characterized by pancytopenia and hypocellular bone marrow without infiltration of abnormal cells or fibrosis. The incidence in Asia is higher than in the West and new cases are diagnosed at a rate of 5.1 per million pediatric populations per year in Korea. The pathophysiology is understood roughly by defective hematopoiesis, impaired bone marrow micro-environment and immune mechanism. Treatments are performed on basis of pathogenesis and selected depending on the severity. Immunosuppressive therapy with antilymphocyte or antithymocyte globulin and cyclosporine is effective in the majority of patients but has some problems including relapse or clonal evolution. Recently, there have been clinical trials of immunosuppression with hematopoietic growth factors or other drugs. Allogeneic hematopoietic stem cell transplantation (HSCT) is curative in children with severe aplastic anemia. The overall survival in HSCT from HLA-identical sibling is higher than alternative donor, including HLA matched unrelated donor or cord blood. We have to consider quality of life after HSCT because of high survival rate. However, chronic graft versus host disease and graft failure are important factors that affect the quality of life and overall survival. We need further investigation to make new regimens aimed at overcoming these risk factors and perform clinical trials.

• 대한치과교정학회지
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• 제52권5호
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• pp.362-371
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• 2022

Orthognathic surgery is the primary treatment option for severe skeletal discrepancy. However, orthodontic camouflage should be considered as an alternative treatment option, considering the risks of surgery. A 19.5-yearold man presented with a severe prognathic mandible with a Class III molar relationship and an anterior crossbite. Orthognathic surgery could be considered because of his severe skeletal discrepancy and mandibular prognathism. However, the anesthetist for orthognathic surgery did not recommend surgery under general anesthesia because of risk factors associated with the patient's aplastic anemia, including bleeding and infections. Thus, a camouflage treatment to promote backward rotation of the mandible via orthodontic extrusion of the posterior teeth was planned. An anterior bite plate, intermaxillary elastics, and fixed orthodontic appliances were used to extrude the posterior teeth and to align the dentition. After 17 months of nonsurgical orthodontic treatment, normal occlusion was achieved, and the facial profile was dramatically improved. This case report describes the dentoskeletal and soft-tissue effects of mandibular rotation and its long-term stability.

• Journal of Periodontal and Implant Science
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• 제28권1호
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• pp.187-191
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• 1998

Aplastic anemia is a disease characterized by general lack of bone marrow activity; It may affect not only the red blood cells but also the white blood cells and platelets, resulting in pancytopenia. Spontaneous gingival hemorrhage is present in some cases and it is related to the blood platelet deficiency. This case report presents the periodontal treatment of a patient with aplastic anemia. A 43-year-old female was referred for continuous gingival bleeding after periodontal treatment. Periodontal findings revealed generalized gingival imflammation, oozing of blood from gingival crevice, and it was diagnosed as adult periodontitis. Root planing and extraction of the upper left third molar with poor prognosis were put into operation after elevation of the platelet count with platelet transfusion. The extraction socket was sutured with 3-0 silk. Bleeding continued even after digital compression at the upper right second premolar, second molar, and left canine areas, which presented severe inflammation. Although platelets were transfused repeatedly, platelet count did not stay elevated since survival rate of the transfused platelets were low due to alloimmunization. Thrombin gauze packing was not effective. Bleeding ceased 3 days after treatment with transfusion of donor platelets. 20 days after the treatment, the gingiva was generally healthy except upper right second premolar and lateral incisor areas. The result of periodontal treatment was good, but bleeding control after treatment was troublesome. In the periodontal treatment of patient with aplastic anemia, elevation of the platelet count with platelet transfusion seems to be the best method for hemorrhage control.

• Journal of Yeungnam Medical Science
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• 제36권2호
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• pp.148-151
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• 2019

The dose of CD34+ cells is known to influence the outcome of allogeneic peripheral blood stem cell (PBSC) and/or T-cell-depleted transplantation. A previous study proposed that $2{\times}10^6\;CD34+\;cells/kg$ is the ideal minimum dose for allogeneic transplantation, although lower doses did not preclude successful therapy. In the case we present here, CD34+ cells were collected from a matched sibling donor on the day of allogeneic hematopoietic stem cell transplantation; however, the number of cells was not sufficient for transplantation. Consequently, PBSCs were collected three additional times and were infused along with cord blood cells from the donor that were cryopreserved at birth. The cumulative dose of total nuclear cells and CD34+ cells was $15.9{\times}10^8\;cells/kg$ and $0.95{\times}10^6\;cells/kg$, respectively. White blood cells from this patient were engrafted on day 12. In summary, we report successful engraftment after infusion of multiple low doses of CD34+ cells in a patient with severe aplastic anemia.

• Journal of Medicine and Life Science
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• 제17권3호
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• pp.103-106
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• 2020

Bone marrow failure, such as aplastic or myelophthisic anemia, can occur due to an underlying lymphoid malignancy and cause life-threatening events. A 58-year-old man diagnosed with angioimmunoblastic T-cell lymphoma had recently visited the emergency department because of an altered level of consciousness caused by acute severe anemia. The laboratory findings were strongly suggestive of bone marrow failure syndrome. Bone marrow examination was immediately performed and, subsequently, dexamethasone was initiated to control the underlying lymphoma. Intravenous immunoglobulin was also administered in combination due to combined immune hemolytic anemia and thrombocytopenia. Bone marrow examination revealed a packed marrow with marked fibrosis and lymphoma involvement. A diagnosis of secondary myelofibrosis related to the underlying lymphoma was made, and sequential combination chemotherapy was introduced despite the presence of severe anemia and thrombocytopenia. After combination chemotherapy, his hematologic profile and underlying lymphoma improved. Better understanding of various hematologic manifestations and knowledge of the rare condition of lymphoma are essential for appropriate diagnostic approaches and treatment.

• Radiation Oncology Journal
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• 제30권4호
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• pp.165-172
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• 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
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• 제58권6호
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• pp.199-205
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• 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
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• 제58권7호
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• pp.267-269
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• 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.