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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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Acute panmyelosis with myelofibrosis

Acute panmyelosis with myelofibrosis (also known as acute myelofibrosis, acute myelosclerosis, and acute myelodysplasia with myelofibrosis) is an acute panmyeloid proliferation associated with fibrosis of the bone marrow. This disorder is very rare and occurs in all age groups.[57] The disorder may occur de novo or after treatment with alkylating-agent chemotherapy and/or radiation (Refer to the section on Acute myeloid leukemias and myelodysplastic syndromes, therapy related of this summary for more information). Clinical features include constitutional symptoms such as weakness and fatigue. (Refer to the PDQ summary on Fatigue for more information.)

Morphologic and cytochemical features include the following:

  • Marked pancytopenia.
  • Anisocytosis.
  • Dysplastic changes in myeloid cells.
  • Hypercellular bone marrow (biopsy).
  • Variable degrees of hyperplasia of erythroid precursors, granulocytes, and megakaryocytes in the bone marrow.
  • Increased number of small- to large-sized megakaryocytes with dysplastic features in the bone marrow.
  • Marked increase in reticulin fibers in the bone marrow.

Immunophenotypically, blasts may express one or more myeloid-associated antigens (CD13, CD33, CD117, and MPO). Some cells may express erythroid or megakaryocytic antigens. The major differential diagnosis includes acute megakaryoblastic leukemia, acute leukemias with associated marrow fibrosis, metastatic tumor with a desmoplasmic reaction, and chronic idiopathic myelofibrosis.[57] (Refer to the PDQ summary on Chronic Myeloproliferative Disorders Treatment for more information.)

No specific chromosomal abnormalities are associated with acute panmyelosis with myelofibrosis. This AML is reported to respond poorly to chemotherapy and to be associated with a short survival.[57]

Myeloid sarcoma

Myeloid sarcoma (also known as extramedullary myeloid tumor, granulocytic sarcoma, and chloroma) is a tumor mass that consists of myeloblasts or immature myeloid cells, occurring in an extramedullary site;[57] development in 2% to 8% of patients with AML has been reported.[79] Clinical features include occurrence common in subperiosteal bone structures of the skull, paranasal sinuses, sternum, ribs, vertebrae, and pelvis; lymph nodes, skin, mediastinum, small intestine, and the epidural space; and occurrence de novo or concomitant with AML or a myeloproliferative disorder.[57,79]

Morphologic and cytochemical features include the following:

  • Granulocytic sarcoma composed of myeloblasts, neutrophils, and neutrophil precursors with three subtypes based on degree of maturation (i.e., blastic, immature, and differentiated).
  • Monoblastic sarcoma preceding or occurring simultaneously with acute monoblastic leukemia.
  • Tumors with trilineage hematopoiesis occurring with transformation of chronic myeloproliferative disorders.
  • Myeloblasts and neutrophils positive for MPO.
  • Neutrophils positive for naphthol ASD chloroacetate esterase.

Immunophenotyping with antibodies to MPO, lysozyme, and chloroacetate are critical to the diagnosis of these lesions.[57] The myeloblasts in granulocytic sarcomas express myeloid-associated antigens (CD13, CD33, CD117, and MPO). The monoblasts in monoblastic sarcomas express acute monoblastic leukemia antigens (CD14, CD116, and CD11c) and usually react with antibodies to lysozyme and CD68. The main differential diagnosis includes non-Hodgkin lymphoma of the lymphoblastic type, Burkitt lymphoma, large-cell lymphoma, and small round cell tumors, especially in children (e.g., neuroblastoma, rhabdomyosarcoma, Ewing/primitive neuroectodermal tumors, and medulloblastoma).

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WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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