spontaneous remission of childhood acute marrow fibrosis and megakaryoblastic leukemia

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

9 months male

Clinical Characteristics

Presented with anemia, thrombocytopenia, hepatosplenomegaly and high fever total blood count revealed hgb 4.9g/dl, leukocytes 10x10^9/l, and platelet count 38x10^9/l abdominal ultrasound showed splenomegaly, liver with multiple nodules, and retroperitoneal lymphadenopathy blood cultures showed growth of staphylococcus aureus which he was treated with cefamezine stool was positive for rotavirus chest xray revealed right upper lobe pneumonia bronchoalveolar lavage was pcr positive for aspergillus which he received voriconazole marrow aspirate yielded "dry tap" and marrow biopsy demonstrated severe myelofibrosis with reticulin grade 3 and immature megakaryocytes that stained strongly for anti-factor 8 antibody he received several blood and platelet transfusions another marrow aspirate was performed 4 weeks later due to persistent thrombocytopenia and it revealed hypoplasia with 12% micromegakaryoblasts karyotype analysis revealed 2/20 cells with t(1;22)(p12;q13) megakaryoblastic acute myeloid leukemia was diagnosed after sr, disease recurred 4 months later (8 months since initial diagnosis) with thrombocytopenia and reappearance of t(1;22) as demonstrated by right-pcr and karyotype analysis

Remission Characteristics

2 weeks after diagnosis, total blood count revealed platelet count 95x10^9/l repeated marrow aspirate revealed normal cellularity and absence of blast cells 3 weeks later, platelet count reached 197x10^9/l and remained in normal limits since then marrow evaluations were repeated 4 months after first admission and demonstrated normal cellularity without myelofibrosis on biopsy, and no blasts as well as normal karyotype with no evidence of t(1;22) translocation on aspirate the right-pcr was negative 4 months after time of diagnosis no hepatic nodules were present on abdominal ultrasound after recurrence, complete remission was achieved by chemotherapy

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

Cefamezine voraconazole blood and platelet tranfusions chemo

Non-Clinical Treatment

None reported