Acute myeloid leukemia with mediastinal myeloid sarcoma refractory to acute myeloid leukemia therapy but responsive to l-asparaginase

We report the case of a 14-year-old female with acute myeloid leukemia (AML) and myeloid sarcomas (MS) in the anterior mediastinum and around numerous bones. Laboratory tests showed a white blood cell count of 4.0 × 10 9 /l with 7.0 % blasts. Computed tomography revealed a mediastinal mass and pleur...

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Veröffentlicht in:International journal of hematology 2012-07, Vol.96 (1), p.136-140
Hauptverfasser: Takahashi, Hiroyoshi, Koh, Katsuyoshi, Kato, Motohiro, Kishimoto, Hiroshi, Oguma, Eiji, Hanada, Ryoji
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container_end_page 140
container_issue 1
container_start_page 136
container_title International journal of hematology
container_volume 96
creator Takahashi, Hiroyoshi
Koh, Katsuyoshi
Kato, Motohiro
Kishimoto, Hiroshi
Oguma, Eiji
Hanada, Ryoji
description We report the case of a 14-year-old female with acute myeloid leukemia (AML) and myeloid sarcomas (MS) in the anterior mediastinum and around numerous bones. Laboratory tests showed a white blood cell count of 4.0 × 10 9 /l with 7.0 % blasts. Computed tomography revealed a mediastinal mass and pleural effusion; pleural effusion cytology was negative for malignant cells. In addition, disseminated intravascular coagulation (DIC) was present. Following DIC therapy, thoracoscopic and bone marrow biopsies were performed. Immunostaining and surface marker analysis revealed that the blast cells were positive for cytoplasmic myeloperoxidase, CD4, CD7, CD33, CD44, CD117, and HLA-DR, but negative for CD34 and CD56. Karyotype was normal. MS associated with AML was diagnosed. Multidrug chemotherapy for AML was completely ineffective, and MS continued to progress. Immunohistochemistry revealed that the blasts were negative for asparagine synthetase (AS); therefore, chemotherapy including l -asparaginase was initiated. After the first administration of l -asparaginase, the patient’s condition improved; however, she subsequently developed tumor lysis syndrome and sepsis, which eventually led to death. Aggressive MS in childhood is rare and refractory to existing AML chemotherapy. Chemotherapy including l -asparaginase may prove to be effective in such cases, especially those in which blast cells show negative AS expression.
doi_str_mv 10.1007/s12185-012-1111-0
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Laboratory tests showed a white blood cell count of 4.0 × 10 9 /l with 7.0 % blasts. Computed tomography revealed a mediastinal mass and pleural effusion; pleural effusion cytology was negative for malignant cells. In addition, disseminated intravascular coagulation (DIC) was present. Following DIC therapy, thoracoscopic and bone marrow biopsies were performed. Immunostaining and surface marker analysis revealed that the blast cells were positive for cytoplasmic myeloperoxidase, CD4, CD7, CD33, CD44, CD117, and HLA-DR, but negative for CD34 and CD56. Karyotype was normal. MS associated with AML was diagnosed. Multidrug chemotherapy for AML was completely ineffective, and MS continued to progress. Immunohistochemistry revealed that the blasts were negative for asparagine synthetase (AS); therefore, chemotherapy including l -asparaginase was initiated. After the first administration of l -asparaginase, the patient’s condition improved; however, she subsequently developed tumor lysis syndrome and sepsis, which eventually led to death. Aggressive MS in childhood is rare and refractory to existing AML chemotherapy. 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Laboratory tests showed a white blood cell count of 4.0 × 10 9 /l with 7.0 % blasts. Computed tomography revealed a mediastinal mass and pleural effusion; pleural effusion cytology was negative for malignant cells. In addition, disseminated intravascular coagulation (DIC) was present. Following DIC therapy, thoracoscopic and bone marrow biopsies were performed. Immunostaining and surface marker analysis revealed that the blast cells were positive for cytoplasmic myeloperoxidase, CD4, CD7, CD33, CD44, CD117, and HLA-DR, but negative for CD34 and CD56. Karyotype was normal. MS associated with AML was diagnosed. Multidrug chemotherapy for AML was completely ineffective, and MS continued to progress. Immunohistochemistry revealed that the blasts were negative for asparagine synthetase (AS); therefore, chemotherapy including l -asparaginase was initiated. After the first administration of l -asparaginase, the patient’s condition improved; however, she subsequently developed tumor lysis syndrome and sepsis, which eventually led to death. Aggressive MS in childhood is rare and refractory to existing AML chemotherapy. 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Laboratory tests showed a white blood cell count of 4.0 × 10 9 /l with 7.0 % blasts. Computed tomography revealed a mediastinal mass and pleural effusion; pleural effusion cytology was negative for malignant cells. In addition, disseminated intravascular coagulation (DIC) was present. Following DIC therapy, thoracoscopic and bone marrow biopsies were performed. Immunostaining and surface marker analysis revealed that the blast cells were positive for cytoplasmic myeloperoxidase, CD4, CD7, CD33, CD44, CD117, and HLA-DR, but negative for CD34 and CD56. Karyotype was normal. MS associated with AML was diagnosed. Multidrug chemotherapy for AML was completely ineffective, and MS continued to progress. Immunohistochemistry revealed that the blasts were negative for asparagine synthetase (AS); therefore, chemotherapy including l -asparaginase was initiated. After the first administration of l -asparaginase, the patient’s condition improved; however, she subsequently developed tumor lysis syndrome and sepsis, which eventually led to death. Aggressive MS in childhood is rare and refractory to existing AML chemotherapy. Chemotherapy including l -asparaginase may prove to be effective in such cases, especially those in which blast cells show negative AS expression.</abstract><cop>Japan</cop><pub>Springer Japan</pub><pmid>22644614</pmid><doi>10.1007/s12185-012-1111-0</doi><tpages>5</tpages></addata></record>
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subjects Adolescent
Antineoplastic Agents - administration & dosage
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
Asparaginase - administration & dosage
Biological and medical sciences
Case Report
Fatal Outcome
Female
Hematologic and hematopoietic diseases
Hematology
Humans
Leukemia, Myeloid, Acute - complications
Leukemia, Myeloid, Acute - drug therapy
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Mediastinal Neoplasms - complications
Mediastinal Neoplasms - diagnosis
Mediastinal Neoplasms - drug therapy
Medical sciences
Medicine
Medicine & Public Health
Oncology
Pneumology
Sarcoma, Myeloid - drug therapy
Treatment Outcome
Tumors of the respiratory system and mediastinum
title Acute myeloid leukemia with mediastinal myeloid sarcoma refractory to acute myeloid leukemia therapy but responsive to l-asparaginase
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