Abstract
Myeloid sarcoma (MS) as a rare localized solid tumor mass consists of myeloblasts or immature myeloid cells was found in an extramedullary site of a 4 year male child who was suffering with fever, abdominal distension since 4 months and swelling of legs from 1month . On examining pallor, bilateral cervical and inguinal lymphadenopathy, bilateral pitting pedal edema, Hepatosplenomegaly & ascites found. On investigation anemia with leukocytosis, thrombocytopenia was found. USG abdomen showed multiple non necrotic lymphnodes. Bone marrow aspiration suggestive of JMML, lymphnode biopsy showed myeloid sarcoma and was confirmed by immune histochemistry (IHC) markers LCA, KP-1(CD68), myeloperoxidase are positive.Immunophenotyping is useful in differentiating myeloid sarcoma from lymphoma and leukaemia. Child was treated with 3/7 regimen of cytoarabinoside, daunorubicin.
Highlights
In world health organization (WHO) 2007 classification of lymphoid and hematopoietic neoplasms has described myeloid sarcoma (MS) as a rare localized solid tumor mass consisting of myeloblasts or immature myeloid cells in an extramedullary site [1]
MS may occur as denovo or concurrently with acute myeloid leukemia (AML), myeloproliferative disorder (MPD) or myelodysplastic syndrome (MDS) or relapse of AML [3] or after allogenic stem cell transplantation [4]
Bone Marrow Aspiration (BMA) shows M:E ratio 1.7:1, increased megaloblastoid erythropoeisis, myelopoesis with predominant cells includes blast cells 12%, promyelocytes 8%, myelocytes 17%, metamyeloctes 5%, band forms & neutrophils 16% lymphocytes 5, erythroid cells 37%, megakaryocytes reduced with dysmegakaryosis with multiple form of nucleoli suggestive of juvenile myelomonocytic leukemia (JMML)
Summary
In world health organization (WHO) 2007 classification of lymphoid and hematopoietic neoplasms has described myeloid sarcoma (MS) as a rare localized solid tumor mass consisting of myeloblasts or immature myeloid cells in an extramedullary site [1]. MS may occur as denovo or concurrently with acute myeloid leukemia (AML), myeloproliferative disorder (MPD) or myelodysplastic syndrome (MDS) or relapse of AML [3] or after allogenic stem cell transplantation [4]. Ultrasound abdomen shows multiple oval shaped isoechoic non necrotic lymphnodes are noted in periportal, peripancreatic, mesenteric, parailiac regions largest measuring 9-11 mm size. Bone Marrow Aspiration (BMA) shows M:E ratio 1.7:1 , increased megaloblastoid erythropoeisis, myelopoesis with predominant cells includes blast cells 12%, promyelocytes 8%, myelocytes 17%, metamyeloctes 5%, band forms & neutrophils 16% lymphocytes 5, erythroid cells 37%, megakaryocytes reduced with dysmegakaryosis with multiple form of nucleoli suggestive of juvenile myelomonocytic leukemia (JMML). Inguinal lymphnode biopsy showed there are few presereved follicles with expansion of inter follicular & parafollicular area, infilterated by medium to large sized cells with irregular nuclei , prominent nucleoli. Final diagnosis of extramedullary lymphnode manifestion of monocytic type of myeloid sarcoma was made
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