Aggressive natura killer cell leukemia presenting with hemophagocytic lymphohistiocytosis
Center for Children's Cancer & Blood Disorders, Sydney Children's Hospital, Randwick, New South Wales, Australia. Pediatric Blood & Cancer
(Impact Factor: 2.39).
03/2008; 50(3):654-7. DOI: 10.1002/pbc.21358
Aggressive natural killer cell leukemia (ANKL) is a very rare condition and when reported occurs almost exclusively in adults. We report a pediatric case of ANKL that presented with hemophagocytic syndrome, preceding the onset of leukemia by 12 weeks. Clinical and laboratory findings are discussed, along with morphology, immunophenotyping and cytogenetics, as well as the association with Epstein-Barr virus (EBV). This case is noteworthy for the expression of CD8 on the malignant cells, the cytogenetic findings that include abnormalities of chromosomes 6 and 7, as well as the age of the patient.
Figures in this publication
Available from: Sherif Rezk
- "This hypothesis is mainly based on the similar ethnic background (Asian), morphology, immunophenotype, and genotype (lack of T-cell receptor gene rearrangements) of both entities. Moreover, EBV is detected in over 75% of aggressive NK-cell leukemia cases and near 100% in nasal NK/T cell lymphoma  . "
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ABSTRACT: Extranodal NK/T-cell lymphoma, nasal type, is an aggressive EBV-associated lymphoma that mainly involves the nasal cavity but has also been reported to involve other extranodal sites without nasal involvement. In contrast to aggressive NK cell leukemia (a marrow-based aggressive leukemia of NK-cell origin); extensive bone marrow and blood involvement is extremely uncommon by nasal type NK/T lymphoma. We report a patient with extranodal NK/T-cell lymphoma, nasal type that developed extensive bone marrow involvement during the course of her disease with some overlapping features with aggressive NK-cell leukemia.
International journal of clinical and experimental pathology 01/2011; 4(7):713-7. · 1.89 Impact Factor
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ABSTRACT: We report the clinical course of a child with precursor B-cell acute lymphoblastic leukemia who developed fever, hepatosplenomegaly, and refractory thrombocytopenia after initiation of chemotherapy. Bone marrow aspiration on Day 8 of induction showed new onset of hemophagocytosis and he met clinical, laboratory, and histopathologic criteria for hemophagocytic lymphohistiocytosis. His symptoms improved significantly after an increased dosage of steroids.
Journal of Pediatric Hematology/Oncology 01/2009; 30(12):956-8. DOI:10.1097/MPH.0b013e3181864ab9 · 0.90 Impact Factor
Available from: Satya Prakash Yadav
Indian pediatrics 01/2011; 48(1):79-80. · 1.04 Impact Factor
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