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Ocular extramedullary myeloid cell tumour in children: an Indian study
S Gujral1, S Bhattarai, A Mohan
1Unit of Laboratory Oncology, Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
Insights
Diagnosing extramedullary myeloid cell tumors (EMT) in children requires careful examination. Prompt diagnosis of acute myeloid leukemia (AML) and related conditions is crucial, necessitating a full hemogram and blood smear review.
Area of Science:
- Pediatric Hematology
- Oncology
- Diagnostic Pathology
Background:
- Extramedullary myeloid cell tumors (EMT) are rare in children, often presenting diagnostic challenges.
- These tumors can be associated with acute myeloid leukemia (AML) and related hematologic disorders.
- Accurate diagnosis is critical for appropriate treatment and patient outcomes.
Purpose of the Study:
- To investigate the laboratory characteristics of pediatric EMT.
- To identify diagnostic challenges and pitfalls in identifying EMT.
- To recommend optimal diagnostic work-up for suspected EMT.
Main Methods:
- Retrospective analysis of 32 children diagnosed with EMT.
- Review of peripheral blood smear and bone marrow examination findings.
- Analysis of cases with missed or incorrect diagnoses.
Main Results:
- EMT constituted 41% of pediatric AML cases studied.
- Diagnoses included AML (29 cases) and refractory anemia with excess blasts in transformation (RAEB-t; 3 cases).
- Diagnostic errors occurred in cases lacking peripheral blood smear examination.
Conclusions:
- Diagnostic work-up for proptosis must include a full hemogram and meticulous peripheral blood smear examination.
- Bone marrow examination is essential when indicated.
- RAEB-t with EMT should be classified as AML.
Abstract:
Thirty-two children with extramedullary myeloid cell tumour (EMT) who constituted 41 per cent of children with acute myeloid leukaemia (AML) were studied to ascertain their laboratory characteristics and potential problems in diagnosis. The diagnosis, established by peripheral blood smear and/or bone marrow examination, was AML (n = 29) and refractory anaemia with excess blasts in transformation (RAEB-t; n = 3). The six referred patients in whom the diagnosis had been missed, and two cases wrongly reported as histiocytosis on aspiration cytology, were those in whom a peripheral blood smear had not been examined. It is concluded that diagnostic work-up of proptosis must include a full haemogram, meticulous peripheral blood smear examination, repeated if necessary, and bone marrow examination where relevant. RAEB-t cases with extramedullary myeloid cell tumour should be classified as acute myeloid leukaemia.