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Adult Acute Myeloid Leukemia Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Classification of Adult Acute Myeloid Leukemia

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No unique chromosomal abnormalities are associated with myeloid sarcoma.[57,79] AML with maturation and t(8; 21)(q22; q22) and AMML Eo with in (16)(p13; q22) or t(16;16)(p13; q22) may be observed and monoblastic sarcoma may be associated with translocations involving 11q23.[57] The presence of myeloid sarcoma in patients with the otherwise good-risk t(8; 21) AML may be associated with a lower complete remission rate and decreased remission duration.[80] Myeloid sarcoma occurring in the setting of MDS or MPD is equivalent to blast transformation. In the case of AML, the prognosis is that of the underlying leukemia.[57] Although the initial presentation of myeloid sarcoma may appear to be isolated, several reports indicate that isolated myeloid sarcoma is a partial manifestation of a systemic disease and should be treated with intensive chemotherapy.[79,81,82]

Acute Leukemias of Ambiguous Lineage

Acute leukemias of ambiguous lineage (also known as acute leukemias of undetermined lineage, mixed phenotype acute leukemias, mixed lineage acute leukemias, and hybrid acute leukemias) are types of acute leukemia in which the morphologic, cytochemical, and immunophenotypic features of the blast population do not allow classification in myeloid or lymphoid categories; or the types have morphologic and/or immunophenotypic features of both myeloid and lymphoid cells or both B and T lineages (i.e., acute bilineal leukemia and acute biphenotypic leukemia).[83,84,85,86,87] These rare leukemias account for less than 4% of all cases of acute leukemia and occur in all age groups but are more frequent in adults.[83] Clinical features include symptoms and complications caused by cytopenias, i.e., fatigue, infections, and bleeding disorders. (Refer to the PDQ summary on Fatigue for more information.)

Morphologic and immunophenotypic features of these acute leukemias include the following:[83,84,86,87]

  • Undifferentiated acute leukemia in which the leukemic cells lack any differentiating characteristics and lack markers for a given lineage.
  • Bilineal acute leukemia in which a dual population of blasts exhibits morphologic features and markers of two distinct lineages, i.e., myeloid and lymphoid or B and T.
  • Biphenotypic acute leukemia in which the blasts exhibit the morphological features of only one lineage but express markers of more than one lineage.

The differential diagnosis includes myeloid antigen-positive ALL or lymphoid-positive AML (from which biphenotypic acute leukemia should be distinguished) and minimally differentiated AML (from which undifferentiated acute leukemia must be distinguished).

Cytogenetic abnormalities are observed in a high percentage of bilineal and biphenotypic leukemias.[84,85,88,89] Approximately 33% of cases have the Philadelphia chromosome, and some cases are associated with t(4; 11)(q21; q23) or other 11q23 abnormalities. In general, the prognosis appears to be unfavorable, particularly in adults; the occurrence of the translocation t(4; 11) or the Philadelphia chromosome are especially unfavorable prognostic indicators.[83,85,90]

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