OBJECTIVE: To study the clinical features and prognosis of children with acute megakaryocytic leukemia (AMKL) and the clinical effect of acute myeloid leukemia 03 (AML03) regimen for the treatment of pediatric AMKL. METHODS: The clinical data were collected from 47 children with AMKL who were diagnosed from May 2011 to December 2019. The treatment outcomes and prognostic factors were analyzed. The Kaplan-Meier method and the log-rank test were used for survival analysis. RESULTS: Among the 47 children with AMKL, 22 with non-Down syndrome-AMKL were treated by the AML03 regimen, with a median follow-up time of 11.4 months. For the 22 non-Down syndrome-AMKL patients, the remission rate of bone marrow cytology was 85% and the negative rate of minimal residual disease (MRD) was 79% after induction Ⅱ, with a 2-year overall survival (OS) rate of (50±13)% and a 2-year event-free survival (EFS) rate of (40±12)%. The group with positive immunophenotypic marker CD56 had significantly lower 2-year EFS and OS rates than the group with negative CD56 ( CONCLUSIONS Children with AMKL tend to have a low remission rate and a poor prognosis. Positive immunophenotypic marker CD56, bone marrow cytology during early treatment response, and MRD results are important factors influencing the prognosis. Allogeneic hematopoietic stem cell transplantation has no significant effect on the prognosis of AMKL.
Child ; Disease-Free Survival ; Hematopoietic Stem Cell Transplantation ; Humans ; Leukemia, Megakaryoblastic, Acute/therapy* ; Neoplasm, Residual ; Prognosis ; Remission Induction ; Survival Rate ; Treatment Outcome

No abstract available.
Bone Marrow/pathology ; *Bone Marrow Transplantation ; Child, Preschool ; Chromosomes, Human, Pair 11 ; Chromosomes, Human, Pair 2 ; Humans ; Infant ; Karyotyping ; Leukemia, Megakaryoblastic, Acute/genetics/*therapy ; Male ; Siblings ; Tissue Donors ; Translocation, Genetic/*genetics ; Transplantation, Homologous

No abstract available.
Aged ; Antineoplastic Combined Chemotherapy Protocols/adverse effects/*therapeutic use ; Blood Cells/pathology ; Bone Marrow Cells/pathology ; Carcinoma, Non-Small-Cell Lung/*drug therapy/radiotherapy ; Humans ; Karyotyping ; Leukemia, Megakaryoblastic, Acute/*diagnosis/etiology ; Lung Neoplasms/*drug therapy/radiotherapy ; Male

The association of hematological malignancies with a mediastinal germ cell tumor (GCT) is very rare. We report one case of a young adult male with primary mediastinal GCT who subsequently developed acute megakaryoblastic leukemia involving isochromosome (12p). A 25-yr-old man had been diagnosed with a mediastinal GCT and underwent surgical resection and adjuvant chemotherapy. At 1 week after the last cycle of chemotherapy, his peripheral blood showed leukocytosis with blasts. A bone marrow study confirmed the acute megakaryoblastic leukemia. A cytogenetic study revealed a complex karyotype with i(12p). Although additional chemotherapy was administered, the patient could not attain remission and died of septic shock. This case was definitely distinct from therapy-related secondary leukemia in terms of clinical, morphologic, and cytogenetic features. To our knowledge, this is the first case report of a patient with mediastinal GCT subsequently developing acute megakaryoblastic leukemia involving i(12p) in Korea.
Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Bleomycin/administration & dosage ; Bone Marrow/pathology ; *Chromosomes, Human, Pair 12 ; Cisplatin/administration & dosage ; Etoposide/administration & dosage ; Humans ; Isochromosomes ; Karyotyping ; Leukemia, Megakaryoblastic, Acute/drug therapy/etiology/*genetics ; Male ; Mediastinal Neoplasms/*diagnosis/drug therapy/surgery ; Neoplasms, Germ Cell and Embryonal/*diagnosis/drug therapy/surgery ; Neoplasms, Second Primary/drug therapy/etiology/*genetics ; Republic of Korea ; Shock, Septic/pathology

To investigate the leukemia relapse of AL patients after HLA haploidentical bone marrow transplantation (HLA HBMT), 2 relapsed leukemia patients received HLA HBMT were studied, peripheral blood simples and bone marrow smear were examined, morphologic change of bone marrow cells was observed, while the HLA genotype and chromosome karyotye were analyzed by PCR and routine G-banding methods, respectively. The results indicated that the two cases were diagnosed primarily as acute lymphocytic leukemia (common cell subtype) and acute megakaryocytic leukemia, in which chromosome abnormalities or activation of protooncogene in leukemic cells were observed. The complete hematopuietie reconstitution of donor origin was obtained in these 2 cases after HLA HBMT, but the leukemic cells in these 2 leukemia patients were confirmed to be donor origin after relapse, their blood groups and HLA genotype were found to be originated from donor. These 2 relapsed leukemia patients were diagnosed as acute lymphocytic leukemia (B cell subtype) and acute megakaryocytic leukemia. It is suggested that high-dose of immunosuppressive agents used in transplantation may contribute to leukemia relapse of donor origin in these patients. Abnormalities in hematopoietic microenvironment may be also involved in the leukemia development. Donor-cell leukemia after allogeneic hematopoietic stem cell transplantation can be an ideal model to investigate the related events in human leukemogenesis.
Cell Transformation, Neoplastic ; Child ; HLA Antigens ; immunology ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Histocompatibility ; Humans ; Infant ; Leukemia, Megakaryoblastic, Acute ; blood ; therapy ; Male ; Neoplasms, Second Primary ; etiology ; pathology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; blood ; therapy ; Recurrence ; Transplantation, Homologous

A case of recurrent rhinocerebral mucormycosis that has occurred during an induction chemotherapy for acute megakaryocytic leukemia in a 10 year-old boy is reported. He had suffered from high fever, proptosis, right eye ball pain and necrotic inflammation of hard palate during the chemotherapy of leukemia. CT scan of the paranasal sinus showed inflammatory change of right ethmoid and maxillary sinuses, and right orbital cystic mass which displaced medial rectus muscle. Pathologic examination of the inflammatory mass revealed mucormycosis with characteristic hyphae invading vessel walls. He was managed with 2 times of extensive debridement of necrotic tissue and currettage of cystic mass, and intravenous amphotericin-B for 80 days with apparent improvement. Seven months after discharge from the hospital, necrosis of posterior nasal septum and hard palate was noted for second time. It was managed again with 2 times of extensive debridement. Since this last operation he is on follow-up for 16 months uneventfully and is on therapy with low dose Ara-C in continuous remission.
Child ; Cytarabine ; Debridement ; Drug Therapy ; Exophthalmos ; Fever ; Follow-Up Studies ; Humans ; Hyphae ; Induction Chemotherapy ; Inflammation ; Leukemia ; Leukemia, Megakaryoblastic, Acute ; Male ; Maxillary Sinus ; Mucormycosis* ; Nasal Septum ; Necrosis ; Orbit ; Palate, Hard ; Tomography, X-Ray Computed