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1/3. Acute erythremic myelosis (true erythroleukaemia): a variant of AML FAB-M6.

    AIMS: Classic erythroleukaemia (acute myeloid leukaemia M6, or M6 AML) is defined as an excess of myeloblasts in an erythroid predominant background. Leukaemia variants in which the primitive blast cells are demonstrably erythroid are extremely rare and poorly characterised. Variably referred to as "true erythroleukaemia" or "acute erythremic myelosis", they are often included within the M6 AML category even though they do not meet strict criteria for this type of AML. methods: Two cases of acute erythroid neoplasia are presented with clinical, morphological, immunophenotypic, and cytogenetic analysis. RESULTS: Both patients presented with profound anaemia, one in a setting of long standing myelodysplasia. bone marrow examination revealed a predominant population of highly dysplastic erythroid cells in both cases. In one case, the liver was infiltrated by neoplastic erythroid cells. Both patients died within four months of diagnosis. CONCLUSIONS: This report illustrates that cases of acute leukaemia occur in which the dominant neoplastic cell is a primitive erythroid cell without an accompanying increase in myeloblasts. This does not preclude the neoplastic clone originating in a multipotent haemopoietic stem cell, as suggested by cases arising in patients with myelodysplasia. Acute erythremic myelosis should be recognised as a distinct variant of M6 AML.
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ranking = 1
keywords = anaemia
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2/3. Fanconi's anaemia developing erythroleukaemia.

    A 15-year-old boy with Fanconi's anaemia (FA) for 10 years developed acute erythroleukaemia. During the leukaemic phase, granulopoietic stem cells (CFUc) were absent from his bone marrow and blood, but proliferation and differentiation of bone marrow cells could be seen in semipermeable diffusion chambers in vivo, and globin synthesis of erythroblasts had become imbalanced. Chromosomal lesions of peripheral blood lymphocytes differed in the leukaemic phase from those in the pancytopenic phase. These data indicate that erythro-, myelo-, thrombo-, and lymphocytic cell lines all were involved in both the leukaemic and the pancytopenic process. It is suggested that terminal myeloproliferative disease developed as part of the natural history of FA.
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ranking = 5
keywords = anaemia
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3/3. A case of systemic lupus erythematosus with sideroblastic anaemia terminating in erythroleukaemia.

    A case of systemic lupus erythematosus is described in which for 10 years the only significant findings were erythema multiforme and vasculitis. Gross hepatosplenomegaly with persistent pancytopenia developed, and bone marrow examination revealed the presence of sideroblasts. The patient's condition deteriorated, and subsequently she developed a severe bleeding tendency, terminating in erythroleukaemia.
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ranking = 4
keywords = anaemia
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