Spontaneous Remission Of Childhood Acute Marrow Fibrosis And Megakaryoblastic Leukemia
Elhasid, R., Tohami, T., Moustafa-Hawash, N., Ben-Ezra, J., Izraeli, S., & Sayar, D. (2012). spontaneous remission of childhood acute marrow fibrosis and megakaryoblastic leukemia. Journal of pediatric hematology/oncology, 34(7), 565–568. https://doi.org/10.1097/MPH.0b013e318257a172
View Original Source →Abstract
Spontaneous remission in 2 children with myelofibrosis, one with megakaryocytic acute myeloblastic leukemia and t(1;22) (with recurrence later) and one with Down syndrome and GATA1 mutation (permanent), are described. One had sepsis and was treated with antibiotics and blood products, whereas the other received only blood products. Remission was spontaneous, without chemotherapy treatment. Possible explanations for these outcomes include immunologic response to sepsis by a leukemia-specific T-cell response or the release of various cytokines, such as tumor necrosis factor and interleukin-2, during infections. Natural killer and cytotoxic T cells transfused with blood products might have also triggered an immune response.
Case Details
Disease Location
Bone marrow
Personal Characteristics
2 year old male with down syndrome
Clinical Characteristics
Presented with pancytopenia cbc revealed hgb 9.1g/dl, wbc 7300, absolute neutrophil count 1300/ul, and platelet 85,000/ul marrow aspirate and biopsy showed 20% cellularity with advanced-stage of fibrosis but no leukemia evidence cytogenetic analysis revealed 47xy+21c a gata-exon 2 mutation was demonstrated by chromatography but no on DNA sequence he required rbc transfusion every 3-4 weeks due to low hgb ~7g/dl
Remission Characteristics
One year later, there was near normalization of blood count with hgb 10-11g/dl, absolute neutrophil 3.5-4.5k/ul, and platelet 75k-95k/ul marrow biopsy at this time revealed 90% cellularity of all cell lineages with normal reticulin no gata1 mutation could be shown a final marrow biopsy at 4.5 years old (2.5 years since diagnosis) showed 100% cellularity with 2 + reticution fibrosis and no evidence of acute leukemia
Treatment & Mechanisms
Proposed Remission Mechanisms
Proliferation of t cells in nonirradiated rbc tranfusion given to both patients might have elicited immune response and thereby graft-vs-leukemia effect cytokines, NK clels or allogeneic lymphocytes from blood tranfusion might have a role in this similar to those of graft-vs-leukemia effect described in allogeneic transplanted patients
Clinical Treatment
Rbc tranfusions
Non-Clinical Treatment
None reported