Idiopathic acquired sideroblastic anemia terminating in acute myelosclerosis

Abstract

Acute myelosclerosis is an unusual myeloproliferative syndrome characterized by pancytopenia, absence of massive hepatosplenomegaly, and an absence of tear‐drop poikilocytes. The marrow is usually fibrotic with atypical megakaryocytic proliferation and trilinear dyspoiesis. Median survival is approximately six months from onset of symptoms. The authors report a case of acute myelosclerosis that evolved in a patient two and one‐half years after the onset of idiopathic acquired sideroblastic anemia. A review of the other previously reported case of this unusual transformation and comments on the pathogenesis of the transformation from idiopathic acquired sideroblastic anemia to acute myelofibrosis are included.

Emergence of JAK2-mutant primary myelofibrosis in myelodysplastic syndrome: rare case report, literature review, and implications for clonal progression

“…However, in our case, the appearance of fibrosis coincided with the loss of MDS-associated RS and del(20q). Furthermore, there have been descriptions of MDS patients transitioning to acute panmyelosis with myelofibrosis (APMF) [8][9][10]. However, APMF is characterized by an abrupt onset with fever and bone pain, with no to minimal splenomegaly, more numerous megakaryocytes and leukemic level (≥20%) blasts [1].…”

Myelofibrosis in primary myelodysplastic syndromes: a clinico‐morphological study of 10 cases

“…Under the appellation acute myelodysplasia. This taxonomic confusion is compounded further by descriptions of acquired idiopathic sideroblastic anaemia terminating in acute myelosclerosis (Yeung & Trowbridge, 1977: Butler et al 1982: Bested et al 1984 and idiopathic myelofibrosis (Lukowicz et al, 1982). These cases were characterized by an acute onset, absence of organomegaly and fulminating course with death from cytopenia occurring within a few months.…”

“…The relation between myelodysplasia and acute megakaryoblastic leukaemia is complicated by the recognition that primary MDS may terminate in megakaryoblastic transformation (Balducci et al, 1978, J. M. Eiennett, personal communication) and that trilineage dysplasia may accompany de novo acute megakaryoblastic leutkaemia (Brito-Babapulle et al, 1987). This taxonomic confusion is compounded further by descriptions of acquired idiopathic sideroblastic anaemia terminating in acute myelosclerosis (Yeung & Trowbridge, 1977: Butler et al 1982: Bested et al 1984 and idiopathic myelofibrosis (Lukowicz et al, 1982).…”

Idiopathic acquired sideroblastic anaemia transforming to acute myelosclerosis.

“…While patients with this disease, usually elderly, often die of unrelated causes, this disease often undergoes transformation to acute leukaemia.2-7 Transformation to acute myelofibrosis, however, is rare. [8][9][10] We report a patient with idiopathic acquired sideroblastic anaemia who developed acute myelofibrosis." In addition to the characteristic ring sideroblasts and erythroid hyperplasia, this patient's bone marrow showed atypical megakaryocytic proliferation before progression to acute myelofibrosis.…”