Presentation
Acute megakaryocytic leukemia (AML M7) is a rare type of acute myelogenous leukemia in adults, commonly presenting with myelofibrosis. [ncbi.nlm.nih.gov]
Entire Body System
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Anemia
There were three cases of megaloblastic anemia (cases 56, 407, and 79) ( Table 3 ). [doi.org]
A 52-year-old man had aregenerative anemia unresponsive to pyridoxine hydrochloride. Acute leukemia developed, and he died four months after diagnosis. [ncbi.nlm.nih.gov]
There was history of death of one sibling at the age of 11 months due to severe anemia and abdominal distension, evaluation was not done in that child. There was no history of transfusion dependent anemia in the family. [ijpmonline.org]
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Poor Feeding
The twins presented at 2 months of age with fever and poor feeding and subsequently developed progressive hepatosplenomegaly. [journals.lww.com]
DS-AML in newborn usually presents with nodular skin infiltrates (bluish, fibroma-like tumors, leukemia cutis), hepatosplenomegaly, lethargy, poor feeding, pallor, purpura/petechiae, respiratory distress. [4] Most Down's syndrome AML are of AML-M7 (DS-AMKL [jdrntruhs.org]
Skin
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Petechiae
DS-AML in newborn usually presents with nodular skin infiltrates (bluish, fibroma-like tumors, leukemia cutis), hepatosplenomegaly, lethargy, poor feeding, pallor, purpura/petechiae, respiratory distress. [4] Most Down's syndrome AML are of AML-M7 (DS-AMKL [jdrntruhs.org]
Skin Findings in the skin include the presence of petechiae ecchymosis due to thrombocytopenia and pallor due to anemia. It can result in leukocytoclastic vasculitis. [lecturio.com]
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Subcutaneous Nodule
On examination, he was having abdominal distension with subcutaneous nodules, hepatosplenomegaly with liver 8 cm below the costal margin and spleen 6 cm below the costal margin, respiratory distress, and icterus [Figure 4], [Figure 5]. [jdrntruhs.org]
Neurologic
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Lethargy
DS-AML in newborn usually presents with nodular skin infiltrates (bluish, fibroma-like tumors, leukemia cutis), hepatosplenomegaly, lethargy, poor feeding, pallor, purpura/petechiae, respiratory distress. [4] Most Down's syndrome AML are of AML-M7 (DS-AMKL [jdrntruhs.org]
Workup
His initial workup, and the course of management and outcome, is described in detail. [ncbi.nlm.nih.gov]
During his initial workup, he was found to have polymorph nuclear leukocytosis (total leukocyte count - 14,300/cmm) with thrombocytopenia (60,000/cmm) and marginally raised serum lactate dehydrogenase levels (234 IU/L) with his chest radiograph showing [marinemedicalsociety.in]
X-Ray
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X-Ray Abnormal
Although the radiographic spectrum of bony changes in leukemia have been well characterized, skeletal X-ray abnormalities in the setting of AML M7 in pediatric patients have been described in few reports that were associated with bone marrow fibrosis. [ncbi.nlm.nih.gov]
PET, SPECT
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Abnormal Bone Scan
A case of acute megakaryocytic leukemia in a 13-month-old infant was encountered in whom an abnormal bone scan was associated with abnormal I-131 MIBG uptake in the femoral bone marrow. [ncbi.nlm.nih.gov]
Serum
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Thrombocytosis
Acute megakaryoblastic leukemia Corresponding ICD-10-CM Codes (U.S. only) C94.2 Acute megakaryoblastic leukemia (effective October 01, 2015) Signs and Symptoms Anemia Asthenia Cytopenia Easy bruising or bleeding Fever Hepatosplenomegaly Thrombocytopenia Thrombocytosis [seer.cancer.gov]
chronic myelogenous leukemia, polycythemia vera, essential thrombocytosis, and primary myelofibrosis. In one review of adult-AMKL, 25% of 49 cases were considered as secondary to one of these MPN. [en.wikipedia.org]
Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis The criteria for MDS/MPN with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T; previously known as RARS-T) include thrombocytosis ( 450 10 9 /L) associated with refractory [doi.org]
The Philadelphia chromosome abnormality was detected in 3 patients (8%) without prior history or clinical features of chronic myeloid leukemia, such as splenomegaly, basophilia, or thrombocytosis. Table 2. [bloodjournal.org]
View Article PubMed Google Scholar Balatzenko G, Guenova M, Zechev J, Toshkov S: Acute megakaryoblastic leukaemia with extreme thrombocytosis and p190 (bcr/abl) rearrangement. Ann Hematol. 2004, 83: 381-385. 10.1007/s00277-003-0783-1. [jhoonline.biomedcentral.com]
Treatment
We conclude that these genetic aberrations may be used for risk group stratification of pediatric AMKL and for treatment tailoring. [ncbi.nlm.nih.gov]
Patients were treated with different treatment protocols according to their respective study group. [doi.org]
Adult-AMKL has remained poorly responsive to the treatment regiments used in DS-AMKL and non-DS-AMKL. These treatments have given complete remission rates of 43-50%. [en.wikipedia.org]
Prognosis
In conclusion, childhood acute megakaryoblastic leukemia is a rare and easily misdiagnosed disease with poor prognosis. [ncbi.nlm.nih.gov]
These cases carry a poor prognosis. Another third of cases is found in Down syndrome. These cases have a reasonably fair prognosis. The last third of cases may be heterogeneous, and carry a poor prognosis. [checkorphan.org]
A patient’s prognosis depends on the cause of the illness. One third, or 33 percent, of children who are found to have a mutation of the T cells have a poor prognosis, One third of patients with Down’s syndrome have a better prognosis. [knowcancer.com]
Etiology
Though the etiology of this complication remains unclear, our experience suggests that early institution of chemotherapy along with supportive care is the best treatment for control of hypercalcemia. [ncbi.nlm.nih.gov]
Hitzler, Origin of Leukemia in Children with Down Syndrome, Etiology of Acute Leukemias in Children, 10.1007/978-3-319-05798-9_5, (109-131), (2016). Sa A. Wang and Robert P. [doi.org]
The etiology is different compared to treatment-related leukemia. [marinemedicalsociety.in]
Manifestation code identifies conditions for which it is important to record both the etiology and the symptom of the disease. [books.google.it]
Epidemiology
October 01, 2015) Signs and Symptoms Anemia Asthenia Cytopenia Easy bruising or bleeding Fever Hepatosplenomegaly Thrombocytopenia Thrombocytosis Diagnostic Exams Immunophenotyping Lumbar puncture Peripheral blood smear Progression and Transformation None Epidemiology [seer.cancer.gov]
Characteristics Epidemiology AMKL is diagnosed in 7–10% of infants and children with AML without Down syndrome (DS). [link.springer.com]
Epidemiology The disease is rare and, due to difficulty in diagnosis, its exact incidence is not known. [atlasgeneticsoncology.org]
Down's syndrome and leukemia: epidemiology, genetics, cytogenetics and mechanisms of leukemogenesis. Cancer Genet Cytogenet. 1987 ; 28 : 55 -76. Robison LL. Down syndrome and leukemia. Leukemia. 1992 ; 6 (suppl 1): 5 -7. [doi.org]
Cancer Epidemiology, Biomarkers, and Prevention. 2005;14(2):467-75. D’Souza G, Kreimer AR, Viscidi R, et al. Case-control study of human papillomavirus and oropharyngeal cancer. New England Journal of Medicine. 2007;356(19):1944-56. [news.cancerconnect.com]
Pathophysiology
It brings together the pathophysiology of disease with detailed clinical guidance on diagnosis and management for the full range of childhood cancers, including aspects important in optimal supportive care. [books.google.com]
A better understanding of the pathophysiology of M7 AML and new therapeutic strategies are needed for patients with M7 AML. Footnotes Reprints: Guillermo Garcia-Manero, Department of Leukemia, University of Texas M.D. [bloodjournal.org]
Acute basophilic leukemia Acute panmyelosis with myelofibrosis Myeloid sarcoma Myeloid proliferations related to Down syndrome Transient abnormal myelopoiesis Myeloid leukemia associated with Down syndrome Blastic plasmacytoid dendritic cell neoplasm Pathophysiology [lecturio.com]
Prevention
In boys Gardasil is approved for prevention of genital warts. In girls it is approved for prevention of genital warts as well as cancer or precancer of the cervix, vulva, and vagina. [news.cancerconnect.com]
Related Journals of Megakaryocytic Leukemia Journal of Leukemia, Advances in Cancer Prevention, Cancer Diagnosis, Cancer Science & Therapy, Archives in Cancer Research, Leukemia, Nature Reviews Cancer, International Journal of Cancer, Cancer Research, [omicsonline.org]
Prevention - Acute megakaryoblastic leukemia Not supplied. [checkorphan.org]
Get optimal test results with guidance on error detection, correction, and prevention as well as cost-effective test selection. [books.google.com]