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Peñuela R, Hernandez I, Fernandes-Pineda M, Cortina L, Zapata D, Urrego O, Herrera J, Saenz I, Orduz R, Mejia F, Moreno L, Velazco M. Spontaneous remission without treatment of acute myelomonocytic leukemia associated with COVID-19 infection. Hematol Transfus Cell Ther 2024; 46:506-510. [PMID: 38199950 PMCID: PMC11451367 DOI: 10.1016/j.htct.2023.11.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2023] [Revised: 10/16/2023] [Accepted: 11/09/2023] [Indexed: 01/12/2024] Open
Affiliation(s)
| | | | | | - Lázaro Cortina
- Hemato-Oncology Clinic Sebastian de Belarcazar, Cali, Colombia
| | - Diana Zapata
- Hemato-Oncology Clinic Sebastian de Belarcazar, Cali, Colombia
| | - Olga Urrego
- Imbanaco Clinical Bone Marrow Transplant Unit, Cali, Colombia
| | - Juan Herrera
- Imbanaco Clinical Bone Marrow Transplant Unit, Cali, Colombia
| | - Isabel Saenz
- Libre University, CQB clinical-pathological laboratory, Cali, Colombia
| | - Recio Orduz
- INPAC research group, Colsanitas Clinic, Bogota Colombia
| | - Fabián Mejia
- INPAC research group, Colsanitas Clinic, Bogota Colombia
| | - Liliana Moreno
- INPAC research group, Colsanitas Clinic, Bogota Colombia
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Sun XY, Yang XD, Yang XQ, Ju B, Xiu NN, Xu J, Zhao XC. Antibiotic and glucocorticoid-induced recapitulated hematological remission in acute myeloid leukemia: A case report and review of literature. World J Clin Cases 2022; 10:7890-7898. [PMID: 36158489 PMCID: PMC9372864 DOI: 10.12998/wjcc.v10.i22.7890] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/10/2021] [Revised: 10/29/2021] [Accepted: 06/26/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Leukemic hematopoietic cells acquire enhanced self-renewal capacity and impaired differentiation. The emergence of symptomatic leukemia also requires the acquisition of a clonal proliferative advantage. Untreated leukemia patients usually experience an aggressive process. However, spontaneous remission occasionally occurs in patients with acute myeloid leukemia (AML), most frequently after recovery from a febrile episode, and this is generally attributed to the triggering of antineoplastic immunity. There may be another explanation for the spontaneous remission as implicated in this paper.
CASE SUMMARY A 63-year-old Chinese man presented with high fever, abdominal pain and urticaria-like skin lesions. He was diagnosed with AML-M4 with t(8;21) (q22;q22)/RUNX1-RUNX1T1 based on morphological, immunological, cytogenetic and molecular analyses. He had a complex chromosome rea-rrangement of 48,XY,t(8;21)(q22;q22),+13,+13[9]/49,idem,+mar[9]/49,idem,+8[2]. He also had a mutated tyrosine kinase domain in fms-like tyrosine kinase 3 gene. He was treated with antibiotics and glucocorticoids for gastrointestinal infection and urticaria-like skin lesions. The infection and skin lesions were quickly resolved. Unexpectedly, he achieved hematological remission along with resolution of the febrile episode, gastrointestinal symptoms and skin lesions. Notably, after relapse, repeating these treatments resulted in a return to hematological remission. Unfortunately, he demonstrated strong resistance to antibiotic and glucocorticoid treatment after the second relapse and died of sepsis from bacterial infection with multidrug resistance. The main clinical feature of this patient was that symptomatic AML emerged with flaring of the gut inflammatory disorder and it subsided after resolution of the inflammation. Learning from the present case raises the possibility that in a subgroup of AML patients, the proliferative advantage of leukemia cells may critically require the presence of inflammatory stresses.
CONCLUSION Inflammatory stresses, most likely arising from gastrointestinal infection, may sustain the growth and survival advantage of leukemic cells.
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Affiliation(s)
- Xiao-Yun Sun
- Department of Hematology, The Central Hospital of Qingdao West Coast New Area, Qingdao 266555, Shandong Province, China
| | - Xiao-Dong Yang
- Department of Hematology, The Central Hospital of Qingdao West Coast New Area, Qingdao 266555, Shandong Province, China
| | - Xiao-Qiu Yang
- Department of Pharmacology, The Central Hospital of Qingdao West Coast New Area, Qingdao 266555, Shandong Province, China
| | - Bo Ju
- Department of Hematology, The Central Hospital of Qingdao West Coast New Area, Qingdao 266555, Shandong Province, China
| | - Nuan-Nuan Xiu
- Department of Hematology, The Central Hospital of Qingdao West Coast New Area, Qingdao 266555, Shandong Province, China
| | - Jia Xu
- Department of Hematology, The Central Hospital of Qingdao West Coast New Area, Qingdao 266555, Shandong Province, China
| | - Xi-Chen Zhao
- Department of Hematology, The Central Hospital of Qingdao West Coast New Area, Qingdao 266555, Shandong Province, China
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Spontaneous Complete Remission of Acute Myeloid Leukemia in the Absence of Disease-Modifying Therapy following Severe Pulmonary Involvement by Coronavirus Infectious Disease-19. Case Rep Hematol 2022; 2022:2603607. [PMID: 35070460 PMCID: PMC8777391 DOI: 10.1155/2022/2603607] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2021] [Accepted: 12/08/2021] [Indexed: 11/24/2022] Open
Abstract
Coronavirus infectious disease-19 (COVID-19) usually alters the innate and adaptive immune setting by excessive production of proinflammatory cytokines, leading to a deviation in the natural course of simultaneous malignant disease. In the absence of disease-modifying therapy, complete remission of acute myeloid leukemia (AML) is an extraordinary event caused mainly by an immune-related mechanism secondary to a severe infectious process. We present a 57-year-old woman with a new diagnosis of AML associated with a 11q23/KMT2A abnormality who had achieved temporary spontaneous remission in the absence of disease-modifying therapy following the severe pulmonary infection with coronavirus lasting for six months. We review the literature and explain the potential impact of stimulated immune responses by COVID-19 on induction of remission in a patient with AML that could provide an excellent opportunity for new immune-based therapies to evolve for the hematologic malignancies. Despite the high ability of the immune process to destroy the malignant cells, the remission of duration is usually short. Therefore, it seems that continuing treatment after SR of AML by a consolidation regimen or bone marrow transplantation, based on a risk-adapted treatment approach, may reduce the recurrence risk.
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Fan WJ, Xu TT, Sang LN, Guo JJ, Li YF, Pei ZX, Jiang ZX. [A case of spontaneous remission of acute myeloid leukemia with MLL-AF9 rearrangement and abnormal liver function]. ZHONGHUA XUE YE XUE ZA ZHI = ZHONGHUA XUEYEXUE ZAZHI 2021; 42:851-857. [PMID: 34788926 PMCID: PMC8607016 DOI: 10.3760/cma.j.issn.0253-2727.2021.10.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/03/2021] [Indexed: 11/13/2022]
Abstract
Objective: To explore the clinical features and possible pathogenesis of spontaneous remission of acute myeloid leukemia (AML) . Methods: We retrospectively analyzed the clinical data of a patient with spontaneous remission of AML, MLL-AF9 rearrangement, and abnormal liver function in the First Affiliated Hospital of Zhengzhou University, and the relevant pieces of literature were summarized. Results: The patient experienced lung infection, fever, and liver dysfunction and was treated with anti-infection and blood transfusion. After complete response (CR) , the patient remained in CR with mild, indirect bilirubin elevation at 35 months of follow-up. Additionally, 56 cases of adult AML (non-acute promyelocytic leukemia) were reported in the literature from 1990 to June 2021. The cases were checked by bone marrow aspiration, and our patients were summarized and analyzed. Furthermore, 57 patients, including 37 males and 20 females, with a median age of 51 (20-83) years and a median remission time of five months; 52 patients achieved complete remission. In addition, there were five cases with long-term remission and a chromosomal record, with no recurrence so far, three with normal karyotype and two with t (9;11) (q21;q23) . Conclusion: The spontaneous remission of leukemia is rare and may be related to immunosuppression and genes.
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Affiliation(s)
- W J Fan
- Department of Hematology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450000, China
| | - T T Xu
- Department of Blood Transfusion, Henan Provincial People's Hospital, Zhengzhou 450000, China
| | - L N Sang
- Department of Hematology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450000, China
| | - J J Guo
- Department of Hematology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450000, China
| | - Y F Li
- Department of Hematology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450000, China
| | - Z X Pei
- Jiaozuo First People's Hospital, Jiaozuo 454002, China
| | - Z X Jiang
- Department of Hematology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450000, China
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Patients with spontaneous remission of high-risk MDS and AML show persistent preleukemic clonal hematopoiesis. Blood Adv 2020; 3:2696-2699. [PMID: 31515231 DOI: 10.1182/bloodadvances.2019000265] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2019] [Accepted: 07/30/2019] [Indexed: 12/31/2022] Open
Abstract
Key Points
We report longitudinal mutational analyses of 2 patients with high-risk MDS and AML experiencing spontaneous disease remissions. Both patients had persistent clonal hematopoiesis during remission, harboring all but 1 of the mutations from the initial diagnostic sample.
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Spontaneous Complete Remission in a Patient with Acute Myeloid Leukemia and Severe Sepsis. Case Rep Hematol 2017; 2017:9593750. [PMID: 28811942 PMCID: PMC5546120 DOI: 10.1155/2017/9593750] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2017] [Revised: 06/08/2017] [Accepted: 06/21/2017] [Indexed: 12/21/2022] Open
Abstract
Without treatment, acute myeloid leukemia (AML) is almost always fatal. Spontaneous remission of AML is a rare phenomenon and usually with a short duration. The exact mechanisms are unknown. However, its association with infection and blood transfusions has been described. We report a 53-year-old male who presented with severe sepsis and who was diagnosed with AML (M4). He has experienced complete spontaneous remission with relatively long duration. To the best of our knowledge, it is the first case of spontaneous remission described in Iran.
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Abstract
Spontaneous remission (SR) of acute myeloid leukemia (AML) is rare. We collected all 46 reported cases of AML with SR. Fever occurred in 91.3% of cases before remission, which was largely due to pneumonia (54.5%) and bacteremia (24.2%). Pneumonia and bacteremia were significantly more common among those who achieved complete remission (CR) compared to those who achieved only a partial remission (p = 0.032). Although 88.6% of remissions were CR, the median duration of remission was only 5 months. Eight cases did not relapse during the follow-up period. The mechanism of SR in AML likely involves the stimulatory effect of systemic febrile infection on the immune system. Immediate treatment of infections and fever may contribute to the rarity of SR in AML. The results of this review improve our understanding of the important role of the immune system in countermanding AML and may provide new ideas for immunotherapy.
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Affiliation(s)
- Armin Rashidi
- Division of Oncology, Washington University School of Medicine , St. Louis, MO , USA
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8
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Spontaneous hematological remission of acute myeloid leukemia. Contemp Oncol (Pozn) 2014; 18:67-9. [PMID: 24876824 PMCID: PMC4037986 DOI: 10.5114/wo.2013.38915] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2013] [Revised: 10/18/2013] [Accepted: 11/08/2013] [Indexed: 12/04/2022] Open
Abstract
Spontaneous remission (SR) of acute myeloid leukemia (AML) in adults is observed very rarely. To date, about 100 cases have been presented in the literature. To our best knowledge, we describe the first adult Polish patient suffering from acute myelomonocytic leukemia (48, XY, +13, +21/46, XY), in whom after supportive therapy, including non-irradiated, non-leukocyte depleted red cell transfusions and low-dose corticosteroid, we observed resolution of the disease without cytogenetic remission. We suggest a potential transfusion-associated graft versus-host-diseases (TA-GVHD) and graft-versus leukemia (GVL) reaction which might lead to spontaneous hematological remission. However, we did not observe clinical symptoms of such reactions apart from a short episode of non-infectious diarrhea. Additionally, steroids were administered but their role in inducing SR, in our opinion, seems less probable. This 77-year-old man remained in SR for 7 months, when repeated analysis showed AML recurrence. He died due to septic shock 2.5 months later. Additionally, we present a review of the literature.
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Zeng Q, Yuan Y, Li P, Chen T. Spontaneous remission in patients with acute myeloid leukemia with t(8;21) or cutaneous myeloid sarcoma: two case reports and a review of the literature. Intern Med 2013; 52:1227-33. [PMID: 23728561 DOI: 10.2169/internalmedicine.52.9505] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
Spontaneous remission (SR) in patients with acute myeloid leukemia (AML) is rare. We herein present two such cases. The first case was of AML-M2 accompanied by a bone marrow cytogenetic analysis revealing 46, XY, t(8;21)(q22,q22). The second case was of isolated cutaneous myeloid sarcoma (MS) that progressed to AML within seven months. Both of the patients had symptoms of infection and anemia and were therefore treated with antibiotics and transfusions. The SR lasted for two months and one month, respectively. Currently, the mechanisms underlying SR remain ambiguous. Possible underlying mechanisms with a review of the related literature are discussed.
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MESH Headings
- Adult
- Chromosomes, Human, Pair 21/genetics
- Chromosomes, Human, Pair 8/genetics
- Female
- Humans
- Leukemia, Myeloid, Acute/diagnosis
- Leukemia, Myeloid, Acute/genetics
- Male
- Remission, Spontaneous
- Sarcoma, Myeloid/diagnosis
- Sarcoma, Myeloid/genetics
- Skin Neoplasms/diagnosis
- Skin Neoplasms/genetics
- Translocation, Genetic/genetics
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Affiliation(s)
- Qingchao Zeng
- Department of Hematology, Huashan Hospital, Fudan University, China
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Hatlen MA, Wang L, Nimer SD. AML1-ETO driven acute leukemia: insights into pathogenesis and potential therapeutic approaches. Front Med 2012; 6:248-62. [PMID: 22875638 DOI: 10.1007/s11684-012-0206-6] [Citation(s) in RCA: 59] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2012] [Accepted: 04/16/2012] [Indexed: 11/30/2022]
Abstract
The AML1-ETO fusion transcription factor is generated by the t(8;21) translocation, which is present in approximately 4%-12% of adult and 12%-30% of pediatric acute myeloid leukemia (AML) patients. Both human and mouse models of AML have demonstrated that AML1-ETO is insufficient for leukemogenesis in the absence of secondary events. In this review, we discuss the pathogenetic insights that have been gained from identifying the various events that can cooperate with AML1-ETO to induce AML in vivo. We also discuss potential therapeutic strategies for t(8;21) positive AML that involve targeting the fusion protein itself, the proteins that bind to it, or the genes that it regulates. Recently published studies suggest that a targeted therapy for t(8;21) positive AML is feasible and may be coming sometime soon.
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Affiliation(s)
- Megan A Hatlen
- Molecular Pharmacology and Chemistry Program, Sloan-Kettering Institute, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA
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11
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Abstract
Somatic rearrangements of transcription factors are common abnormalities in the acute leukemias. With rare exception, however, the resultant protein products have remained largely intractable as pharmacologic targets. One example is AML1-ETO, the most common translocation reported in acute myeloid leukemia (AML). To identify AML1-ETO modulators, we screened a small molecule library using a chemical genomic approach. Gene expression signatures were used as surrogates for the expression versus loss of the translocation in AML1-ETO-expressing cells. The top classes of compounds that scored in this screen were corticosteroids and dihydrofolate reductase (DHFR) inhibitors. In addition to modulating the AML1-ETO signature, both classes induced evidence of differentiation, dramatically inhibited cell viability, and ultimately induced apoptosis via on-target activity. Furthermore, AML1-ETO-expressing cell lines were exquisitely sensitive to the effects of corticosteroids on cellular viability compared with nonexpressers. The corticosteroids diminished AML1-ETO protein in AML cells in a proteasome- and glucocorticoid receptor-dependent manner. Moreover, these molecule classes demonstrated synergy in combination with standard AML chemotherapy agents and activity in an orthotopic model of AML1-ETO-positive AML. This work suggests a role for DHFR inhibitors and corticosteroids in treating patients with AML1-ETO-positive disease.
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Marisavljevic D, Markovic O, Zivkovic R. An unusual case of smoldering AML with prolonged indolent clinical course and spontaneous remission in the terminal phase. Med Oncol 2009; 26:476-9. [DOI: 10.1007/s12032-008-9153-0] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/22/2008] [Accepted: 12/11/2008] [Indexed: 11/24/2022]
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13
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Jain N, Hubbard J, Vega F, Vidal G, Garcia-Manero G, Borthakur G. Spontaneous Remission of Acute Myeloid Leukemia: Report of Three Cases and Review of the Literature. ACTA ACUST UNITED AC 2008. [DOI: 10.3816/clk.2008.n.008] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
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Al-Tawfiq JA, Al-Khatti AA. Spontaneous remission of acute monocytic leukemia after infection with Clostridium septicum. Int J Lab Hematol 2007; 29:386-9. [PMID: 17824921 DOI: 10.1111/j.1365-2257.2006.00846.x] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
Spontaneous remissions of acute myeloid leukemia (AML) have been reported in association with infection. Here, we report a case of spontaneous remission of AML in a 47-year-old Saudi Arabian male patient who presented with a few weeks history of recurrent abdominal pain, vomiting and fever. He was diagnosed with acute monocytic leukemia (AML, FAB M5b) and a perforated bowel. He also had Clostridium septicum bacteremia and thus chemotherapy was deferred. He received supportive therapy and intravenous antibiotics. Six weeks later, he achieved spontaneous and complete remission lasting for about 4 months. The remission and relapse were documented by bone marrow examination. Similarly, previous reports of spontaneous remission of AML were short lived and were followed by relapse and progression.
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Affiliation(s)
- J A Al-Tawfiq
- Internal Medicine Services Division, Dhahran Health Center, Saudi Aramco Medical Services Organization Saudi Aramco, Dhahran, Saudi Arabia.
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Ozyurek E, Alioglu B, Coskun M, Ozbek N. Successful use of short-course high-dose methylprednisolone in a child with acute myeloblastic leukemia (FAB M2) and myeloid tumor. Leuk Lymphoma 2006; 47:923-5. [PMID: 16753881 DOI: 10.1080/10428190600813259] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
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Ozyurek E, Alioglu B, Coskun M, Ozbek N. Successful use of short-course high-dose methylprednisolone in a child with acute myeloblastic leukemia (FAB M2) and myeloid tumor. Leuk Lymphoma 2006. [DOI: 10.1080/14753630500400217] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
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Hiçsönmez G. The effect of steroid on myeloid leukemic cells: The potential of short-course high-dose methylprednisolone treatment in inducing differentiation, apoptosis and in stimulating myelopoiesis. Leuk Res 2006; 30:60-8. [PMID: 15979702 DOI: 10.1016/j.leukres.2005.05.015] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2005] [Revised: 05/09/2005] [Accepted: 05/17/2005] [Indexed: 11/21/2022]
Abstract
Several in vitro studies have shown that dexamethasone (Dex) and prednisolone can induce differentiation of some mouse and human myeloid leukemic cells to macrophages and granulocytes. Based on in vitro experiments, we have shown that short-course (3-7 days) high-dose methylprednisolone (HDMP) (20-30 mg/kg/day) treatment can induce differentiation of myeloid leukemic cells in vivo in children with different subtypes of acute myeloblastic leukemia (AML) (AML-M1, -M2, -M3, -M4, -M7). We have also shown that induction of apoptosis of myeloid leukemic cells with or without differentiation is possible by short-course HDMP treatment. In addition, short-course HDMP treatment has been shown to be effective in accelerating leukocyte recovery, possibly stimulating normal CD34-positive hematopoietic progenitor cells. Addition of HDMP to mild cytotoxic chemotherapy (low-dose cytosine arabinoside (LD-Ara-c), weekly mitoxantrone and Ara-c or 6-thioguanine) increased the remission rate (87-89%) and improved the outcome of AML children. We believe that the results of our 17-year clinical experience will provide important benefits to AML patients.
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Affiliation(s)
- Gönül Hiçsönmez
- Department of pediatric Hematology, Ihsan Dogramaci Children's Hospital, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey.
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Fozza C, Bellizzi S, Bonfigli S, Campus PM, Dore F, Longinotti M. Cytogenetic and hematological spontaneous remission in a case of acute myelogenous leukemia. Eur J Haematol 2004; 73:219-22. [PMID: 15287921 DOI: 10.1111/j.1600-0609.2004.00281.x] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Several cases of spontaneous remission (SR) interrupting the invariably progressive course of untreated acute myeloblastic leukemia (AML) have been reported so far. We shall add to this series the hematological and cytogenetic SR occurring in a 72-yr-old man affected by AML following myelodysplastic syndrome. At diagnosis cytogenetic analysis showed the 48, xy, del (6) (p22-pter), +13, +14 karyotype. Owing to a lobar pneumonia, the chemotherapy was deferred and a broad spectrum antibiotic therapy was established. Supportive care included red cells and platelet transfusions and low-dose corticosteroid. Two months later, after the pneumonia had completely disappeared, a complete remission, lasting about 5 months, was documented on bone marrow morphological and cytogenetical examination, although some degree of myeloid dysplasia persisted. Possible mechanisms of the various SRs described during the course of AML are discussed with a review of the literature.
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Affiliation(s)
- C Fozza
- Institute of Hematology, University of Sassari, Italy.
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Hiçsönmez G, Cetin M, Tuncer AM, Yenicesu I, Aslan D, Ozyürek E, Unal S. Children with acute myeloblastic leukemia presenting with extramedullary infiltration: the effects of high-dose steroid treatment. Leuk Res 2004; 28:25-34. [PMID: 14630077 DOI: 10.1016/s0145-2126(03)00159-0] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
To evaluate whether children with acute myeloblastic leukemia (AML) presenting with extramedullary infiltration (EMI) have different clinical, morphologic features and prognosis from children without EMI, a 127 consecutive previously untreated children with AML were entered in this study. Fifty-one children (40%) had EMI at diagnosis and 27% of these showed multiple site involvement. Twenty-seven of 127 children (21%) presented myeloid tumors. No age related differences in the incidence of EMI was noted. However, analysis of clinical and biological features at diagnosis showed that WBC count > or =50 x 10(9) l(-1), hepatosplenomegaly >5 cm, FAB AML-M4 and AML-M5 subtypes and CD13, CD14 expression of bone marrow (BM) leukemic cells (>20%) were more frequent in children with EMI. Two consecutive treatment protocols were used. In both protocols remission was achieved with combined high-dose methylprednisolone (HDMP) as a differentiating and apoptosis inducing agent with mild cytotoxic chemotherapy (low-dose cytosine arabinoside (LD Ara-C), weekly mitoxantrone and Ara-C or 6-thioguanine). Administration of short-course (4-7 days) HDMP (20-30 mg/kg per day) alone resulted in a remarkable decrease in peripheral blood, BM blasts and in the size of EMI in responding patients. In both protocols, remission rate in patients with EMI was 71 and 80%, which was lower than that of the patients without EMI (87 and 89%). This may be attributed to the higher frequency of unfavorable features in children with EMI. However, in patients who presented with myeloblastoma and treated with a more intensive post-remission therapy (AML-94), the 4-year disease-free survival (DFS) and event-free survival (EFS) rates were not found to be significantly different from children who had no EMI (P>0.05). Whereas, the outcome of children who presented with gingival infiltration did not improve. In further studies, the prognostic significance of different localisation of EMI and the effect of addition of HDMP to cytotoxic chemotherapy should be explored in larger series.
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Affiliation(s)
- Gönül Hiçsönmez
- Department of Pediatric Hematology, Ihsan Doğramaci Children's Hospital, Hacettepe University, Ankara 06100, Turkey.
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