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*The [[immunophenotype]] was detected by [[flow cytometry]] and [[immunohistochemistry]] assay.<ref name="pmid17605859">{{cite journal |author=Lei Q, Liu Y, Tang SQ |title=[Childhood acute megakaryoblastic leukemia] |language=Chinese |journal=Zhongguo Shi Yan Xue Ye Xue Za Zhi |volume=15 |issue=3 |pages=528-32 |year=2007 |pmid=17605859 |doi=}}</ref> | *The [[immunophenotype]] was detected by [[flow cytometry]] and [[immunohistochemistry]] assay.<ref name="pmid17605859">{{cite journal |author=Lei Q, Liu Y, Tang SQ |title=[Childhood acute megakaryoblastic leukemia] |language=Chinese |journal=Zhongguo Shi Yan Xue Ye Xue Za Zhi |volume=15 |issue=3 |pages=528-32 |year=2007 |pmid=17605859 |doi=}}</ref> | ||
*In blood and bone marrow smears megakaryoblasts are usually medium sized to large cells with a high nuclear- cytoplasmic ratio. Nuclear [[chromatin]] is dense and homogeneous. There is scanty, variable basophilic cytoplasm which may be vacuolated. An irregular cytoplasmic border is often noted in some of the megakaryoblasts and occasionally projections resembling budding atypical platelets are present. The megakaryoblasts lack [[myeloperoxidase]] activity and stain negatively with Sudan black B (SBB). | *In blood and bone marrow smears megakaryoblasts are usually medium sized to large cells with a high nuclear- cytoplasmic ratio. Nuclear [[chromatin]] is dense and homogeneous. There is scanty, variable basophilic cytoplasm which may be vacuolated. An irregular cytoplasmic border is often noted in some of the [[megakaryoblasts]] and occasionally projections resembling budding atypical platelets are present. The megakaryoblasts lack [[myeloperoxidase]] activity and stain negatively with [[Sudan black B]] (SBB). | ||
*They are alpha [[naphthyl butyrate esterase]] negative and manifest variable alpha naphthyl acetate esterase activity usually in scattered clumps or granules in the cytoplasm. [[PAS]] staining also varies from negative to focal or granular positivity, to strongly positive staining. | *They are alpha [[naphthyl butyrate esterase]] negative and manifest variable alpha naphthyl acetate esterase activity usually in scattered clumps or granules in the cytoplasm. [[PAS]] staining also varies from negative to focal or granular positivity, to strongly positive staining. | ||
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*More precise identification by immunophenotyping or with [[electron microscopy]] (EM). [[Immunophenotyping]] using MoAb to megakaryocyte restricted antigen (CD41 and CD61) may be diagnostic.<ref name="pmid12239137">{{cite journal |author=Vardiman JW, Harris NL, Brunning RD |title=The World Health Organization (WHO) classification of the myeloid neoplasms |journal=[[Blood]] |volume=100 |issue=7 |pages=2292-302 |year=2002 |pmid=12239137 |doi=10.1182/blood-2002-04-1199 |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=12239137}}</ref> | *More precise identification by immunophenotyping or with [[electron microscopy]] (EM). [[Immunophenotyping]] using MoAb to megakaryocyte restricted antigen (CD41 and CD61) may be diagnostic.<ref name="pmid12239137">{{cite journal |author=Vardiman JW, Harris NL, Brunning RD |title=The World Health Organization (WHO) classification of the myeloid neoplasms |journal=[[Blood]] |volume=100 |issue=7 |pages=2292-302 |year=2002 |pmid=12239137 |doi=10.1182/blood-2002-04-1199 |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=12239137}}</ref> | ||
=== Karyotype analysis: === | ===Karyotype analysis:=== | ||
* It | *It is essential owing to the fact that AMKL has complex [[karyotype]] with peculiar presentation (t(1;22)(p13;q13).<ref name="DöhnerEstey2010">{{cite journal|last1=Döhner|first1=Hartmut|last2=Estey|first2=Elihu H.|last3=Amadori|first3=Sergio|last4=Appelbaum|first4=Frederick R.|last5=Büchner|first5=Thomas|last6=Burnett|first6=Alan K.|last7=Dombret|first7=Hervé|last8=Fenaux|first8=Pierre|last9=Grimwade|first9=David|last10=Larson|first10=Richard A.|last11=Lo-Coco|first11=Francesco|last12=Naoe|first12=Tomoki|last13=Niederwieser|first13=Dietger|last14=Ossenkoppele|first14=Gert J.|last15=Sanz|first15=Miguel A.|last16=Sierra|first16=Jorge|last17=Tallman|first17=Martin S.|last18=Löwenberg|first18=Bob|last19=Bloomfield|first19=Clara D.|title=Diagnosis and management of acute myeloid leukemia in adults: recommendations from an international expert panel, on behalf of the European LeukemiaNet|journal=Blood|volume=115|issue=3|year=2010|pages=453–474|issn=0006-4971|doi=10.1182/blood-2009-07-235358}}</ref><ref name="De MarchiAraki2019">{{cite journal|last1=De Marchi|first1=Federico|last2=Araki|first2=Marito|last3=Komatsu|first3=Norio|title=Molecular features, prognosis, and novel treatment options for pediatric acute megakaryoblastic leukemia|journal=Expert Review of Hematology|volume=12|issue=5|year=2019|pages=285–293|issn=1747-4086|doi=10.1080/17474086.2019.1609351}}</ref> | ||
===Radiographic features:=== | ===Radiographic features:=== | ||
*Using Chest X-ray ('''CXR'''), '''skeletal survey''', '''CT scan''', and '''bone scan''' are: | *Using [[Chest X-ray]] ('''CXR'''), '''[[skeletal survey]]''', '''[[CT scan]]''', and '''[[bone scan]]''' are: | ||
*Osteolytic lesions in the ribs, diffuse metaphysical lucency, periostitis with periosteal reaction, and pathologic fractures involving long bones, such as femoral bones, and increase uptake in the involved bones.<ref name="AthaleKaste2002">{{cite journal|last1=Athale|first1=Uma H.|last2=Kaste|first2=Sue C.|last3=Razzouk|first3=Bassem I.|last4=Rubnitz|first4=Jeffrey E.|last5=Ribeiro|first5=Raul C.|title=Skeletal Manifestations of Pediatric Acute Megakaryoblastic Leukemia|journal=Journal of Pediatric Hematology/Oncology|volume=24|issue=7|year=2002|pages=561–565|issn=1077-4114|doi=10.1097/00043426-200210000-00014}}</ref> | *Osteolytic lesions in the ribs, diffuse [[metaphysical lucency]], [[periostitis]] with periosteal reaction, and pathologic fractures involving long bones, such as femoral bones, and increase uptake in the involved bones.<ref name="AthaleKaste2002">{{cite journal|last1=Athale|first1=Uma H.|last2=Kaste|first2=Sue C.|last3=Razzouk|first3=Bassem I.|last4=Rubnitz|first4=Jeffrey E.|last5=Ribeiro|first5=Raul C.|title=Skeletal Manifestations of Pediatric Acute Megakaryoblastic Leukemia|journal=Journal of Pediatric Hematology/Oncology|volume=24|issue=7|year=2002|pages=561–565|issn=1077-4114|doi=10.1097/00043426-200210000-00014}}</ref> | ||
==References== | ==References== | ||
Latest revision as of 03:53, 19 April 2021
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Other Diagnostic Studies
- In adults, the features include
- Pancytopenia with low blast counts in the blood
- Myelofibrosis
- An absence of lymphadenopathy and hepatosplenomegaly
- Poor response to chemotherapy,and short clinical course.
- In children, the same clinical presentation but with variable course especially in very young children; both leukocytosis and organomegaly may be present in children with M7. Complete remission and long term survival are more common in children than adults.
- The morphology of cells was observed by means of Bone marrow smear
- The immunophenotype was detected by flow cytometry and immunohistochemistry assay.[1]
- In blood and bone marrow smears megakaryoblasts are usually medium sized to large cells with a high nuclear- cytoplasmic ratio. Nuclear chromatin is dense and homogeneous. There is scanty, variable basophilic cytoplasm which may be vacuolated. An irregular cytoplasmic border is often noted in some of the megakaryoblasts and occasionally projections resembling budding atypical platelets are present. The megakaryoblasts lack myeloperoxidase activity and stain negatively with Sudan black B (SBB).
- They are alpha naphthyl butyrate esterase negative and manifest variable alpha naphthyl acetate esterase activity usually in scattered clumps or granules in the cytoplasm. PAS staining also varies from negative to focal or granular positivity, to strongly positive staining.
- A marrow aspirate is difficult to obtain in many cases because of variable degree of myelofibrosis.
- More precise identification by immunophenotyping or with electron microscopy (EM). Immunophenotyping using MoAb to megakaryocyte restricted antigen (CD41 and CD61) may be diagnostic.[2]
Karyotype analysis:
- It is essential owing to the fact that AMKL has complex karyotype with peculiar presentation (t(1;22)(p13;q13).[3][4]
Radiographic features:
- Using Chest X-ray (CXR), skeletal survey, CT scan, and bone scan are:
- Osteolytic lesions in the ribs, diffuse metaphysical lucency, periostitis with periosteal reaction, and pathologic fractures involving long bones, such as femoral bones, and increase uptake in the involved bones.[5]
References
- ↑ Lei Q, Liu Y, Tang SQ (2007). "[Childhood acute megakaryoblastic leukemia]". Zhongguo Shi Yan Xue Ye Xue Za Zhi (in Chinese). 15 (3): 528–32. PMID 17605859.
- ↑ Vardiman JW, Harris NL, Brunning RD (2002). "The World Health Organization (WHO) classification of the myeloid neoplasms". Blood. 100 (7): 2292–302. doi:10.1182/blood-2002-04-1199. PMID 12239137.
- ↑ Döhner, Hartmut; Estey, Elihu H.; Amadori, Sergio; Appelbaum, Frederick R.; Büchner, Thomas; Burnett, Alan K.; Dombret, Hervé; Fenaux, Pierre; Grimwade, David; Larson, Richard A.; Lo-Coco, Francesco; Naoe, Tomoki; Niederwieser, Dietger; Ossenkoppele, Gert J.; Sanz, Miguel A.; Sierra, Jorge; Tallman, Martin S.; Löwenberg, Bob; Bloomfield, Clara D. (2010). "Diagnosis and management of acute myeloid leukemia in adults: recommendations from an international expert panel, on behalf of the European LeukemiaNet". Blood. 115 (3): 453–474. doi:10.1182/blood-2009-07-235358. ISSN 0006-4971.
- ↑ De Marchi, Federico; Araki, Marito; Komatsu, Norio (2019). "Molecular features, prognosis, and novel treatment options for pediatric acute megakaryoblastic leukemia". Expert Review of Hematology. 12 (5): 285–293. doi:10.1080/17474086.2019.1609351. ISSN 1747-4086.
- ↑ Athale, Uma H.; Kaste, Sue C.; Razzouk, Bassem I.; Rubnitz, Jeffrey E.; Ribeiro, Raul C. (2002). "Skeletal Manifestations of Pediatric Acute Megakaryoblastic Leukemia". Journal of Pediatric Hematology/Oncology. 24 (7): 561–565. doi:10.1097/00043426-200210000-00014. ISSN 1077-4114.