acid-phosphatase and Primary-Myelofibrosis

We present a case of acute megakaryoblastic leukemia identified by electron microscopy and platelet-specific antibodies. The histological examination of bone marrow showed distinct myelofibrosis. In accordance with recent communications, low-dose cytosine arabinoside treatment (20 mg twice daily s.c. over 21 days) was initiated. The subsequent bone marrow examination showed a severe hypoplasia with persistent blasts. Amsacrine and VP-16 were given without success. Finally the patient died of septicemia without proof of pathogen uninfluenced by antibiotic and antiseptic therapy 6 weeks after diagnosis. Our case report confirms the poor prognosis of acute megakaryoblastic leukemia.

Topics: Acid Phosphatase; Aged; Amsacrine; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow; Cytarabine; Etoposide; Humans; Leukemia, Megakaryoblastic, Acute; Male; Neoplasm Proteins; Neoplastic Stem Cells; Primary Myelofibrosis

Topics: Acid Phosphatase; Blood Platelet Disorders; Bone and Bones; Bone Diseases; Breast Neoplasms; Electrophoresis, Polyacrylamide Gel; Erythrocytes; Female; Hematologic Diseases; Humans; Isoenzymes; Leukemia; Leukocytes; Lipidoses; Male; Primary Myelofibrosis; Prostate; Prostatic Neoplasms; Spleen; Thromboembolism

Topics: Acid Phosphatase; Bone Marrow; Humans; Leukemia, Hairy Cell; Male; Middle Aged; Primary Myelofibrosis

Two girls, each less than 2 yr of age, developed acute megakaryoblastic leukemia (malignant myelosclerosis). Both presented with anemia, severe thrombocytopenia, and a low percentage of blasts in their peripheral blood. Their marrow showed marked reticulin fibrosis with an increase in blasts and immature megakaryocytes. The blasts stained negatively for myeloperoxidase and Sudan Black B, but showed acid phosphatase (ACP) and alpha-naphthyl acetate esterase (ANAE) activity inhibitable by sodium fluoride. They were identified as megakaryoblasts by the platelet peroxidase reaction. Cytogenetic studies showed multiple chromosomal abnormalities in both cases. Chemotherapy with vincristine, prednisone, and L-asparaginase was without effect, while daunorubicin and cytosine arabinoside induced a complete remission in one case. The second case responded to a combination of cytosine arabinoside, daunorubicin, and 6-thioguanine. This article documents that acute megakaryoblastic leukemia occurs in early childhood and describes its clinical, pathologic, and cytogenetic features. Previous reports of childhood "myelofibrosis" are reviewed, and their possible relationship with acute megakaryoblastic leukemia is discussed.

Topics: Acid Phosphatase; Acute Disease; Esterases; Female; Histocytochemistry; Humans; Infant; Leukemia; Megakaryocytes; Microscopy, Electron; Primary Myelofibrosis

Acute megakaryoblastic leukemia or acute "malignant" myelosclerosis is an acute and rapidly progressive myeloproliferative syndrome characterized by minimal or absent splenomegaly, pancytopenia, diffuse marrow fibrosis, and circulating blasts of megakaryocytic origin. The disease must be differentiated from other hematologic malignancies especially myelofibrosis with myeloid metaplasia. The radiographic changes of osteosclerosis in our patient have not been previously reported in the literature.

Topics: Acid Phosphatase; Acute Disease; Hemoglobinometry; Humans; Leukemia, Myeloid, Acute; Male; Middle Aged; Osteosclerosis; Primary Myelofibrosis; Radiography

Topics: Acid Phosphatase; Bone Marrow; Humans; Isoenzymes; Leukemia, Hairy Cell; Primary Myelofibrosis

Topics: Acid Phosphatase; Acute Disease; Adult; Cell Differentiation; Esterases; Histocytochemistry; Humans; Leukemia; Male; Naphthaleneacetic Acids; Osteosclerosis; Primary Myelofibrosis; Staining and Labeling

Topics: Acid Phosphatase; Blood; Blood Chemical Analysis; Blood Coagulation Disorders; Blood Platelet Disorders; Blood Platelets; Hodgkin Disease; Humans; Leukemia; Leukemia, Myeloid; Liver Cirrhosis; Plasma; Platelet Count; Potassium; Primary Myelofibrosis; Thrombocythemia, Essential