Terminal Acute Leukemia Research Articles

Polyploidy in Myelodysplastic Syndrome: A Case Report

Structural chromosomal abnormalities are common in myelodysplastic syndromes (MDSs), and complex abnormalities are known to confer a poor prognosis. Polyploidy is rare. We report a patient with MDS in whom 8/15 cells were 77,XYY; 2/15 were 83,XYY, and 5/15 were diploid (46,XY). He experienced rapid evolution of disease, transforming to terminal acute leukemia.

Subacute and chronic myelomonocytic leukemia in children (juvenile CML). Clinical and hematologic observations, and identification of prognostic factors.

The clinical and hematologic characteristics of 38 children with subacute and chronic myelomonocytic leukemia (S & CMMOL) are described, and the prognostic significance of these characteristics as recorded at diagnosis is reported. The common and distinctive feature of these children was the excessive proliferation of cells of neutrophilic and monocytic series. The disease predominated in younger children, 95% were younger than 4 years, and boys were more affected than girls (22/16). The onset of the disease was heralded most often by acute or subacute symptoms. Splenomegaly was the most common physical finding at diagnosis. Leukocytosis was usually under 100 X 10(9)/l. Monocytosis and granulocytosis were often associated with normoblastosis, and, in some cases, with moderate blastosis (less than or equal to 30%). Severe anemia and marked thrombocytopenia were found in about one third of patients, increased fetal hemoglobin levels in 53%, and increased gamma-globulin levels in 50% of cases. The Philadelphia chromosome was absent in all blood and marrow cell karyotypes. Thirty-three of 38 patients were treated with moderate or intensive chemotherapy, and in all cases treatment never resulted in a complete remission. Terminal acute leukemia occurred in 11 cases. Of the 38 patients, 29 have died (median survival time, 16 months). Initial characteristics predicting a short survival (log-rank test) included: older age (greater than or equal to 2 years) (P less than 0.001), hepatomegaly (P less than 0.05), bleeding (P less than 0.001), thrombocytopenia (P less than 0.01), high counts of blasts and normoblasts in peripheral blood (P less than 0.01, P less than 0.01). Sex, infections, cutaneous manifestations, lymphadenopathy, degree of splenomegaly, hemoglobin levels, fetal hemoglobin, leukocyte counts, percent of blasts in bone marrow, and serum gamma-globulin levels were of no prognostic value. When survival was plotted on a semilogarithmic scale, a change in death rate was evident at the second year of survival suggesting that there may be two subgroups of patients with myelomonocytic picture, one with very rapid, and another with a slower rate of mortality. A stepwise discriminant-function analysis was performed in an attempt to distinguish between those children who lived less than or equal to 2 years and those who lived longer. A linear combination of variables which best discriminated between these two subgroups was found. Nearly all patients could be classified as a short-survivor or long-survivor on the basis of age and platelet, blast, and normoblast counts in peripheral blood.(ABSTRACT TRUNCATED AT 400 WORDS)

Chronic Lymphocytic Leukemia Terminating in Acute Leukemia

The occurrence of acute leukemia in patients with chronic lymphocytic leukemia is being reported with increasing frequency.^1-31Although the majority of these cases have been described as "acute blastic terminal phase" or as straightforward lymphoblastic in nature, a substantial number of patients' terminal acute leukemia has been described as myeloblastic, monocytic, and myelomonocytic, which raises the question of a separate origin to this terminal acute leukemia. A review and analysis of the literature are the substance of this report. <h3>LITERATURE REVIEW</h3> In 1949, Lawrence et al¹described 100 patients with chronic lymphocytic leukemia treated with radioactive phosphorus (³²P). Fifty-eight of the patients also received local radiation to spleen or lymph nodes either prior to or subsequent to the³²P therapy. Two of the 100 patients developed "acute leukemia with increased numbers of blast cells in the blood smear" (type not stated). The following year, another report

Abnormalities of chromosome 1 in myeloproliferative disorders

Three patients with myeloproliferative disorders showed a similar chromosome abnormality, accompanied by other abnormalities that were different in each case. Marrow cells from all three patients were trisomic either for the entire chromosome 1 or for its long arm. Patient 1 had a brief period of anemia and thrombocytopenia which preceded a terminal acute leukemia; Patient 2 had polycythemia vera (P.V.) that terminated in acute leukemia; and Patient 3 has P.V. The detection of an abnormal karyotype in Patients 1 and 2 was an important factor in establishing the diagnosis of acute leukemia. Preliminary evidence supports the suggestion that some chromosomal changes are nonrandom in myeloproliferative diseases. Nonrandom abnormalities involving the same chromosome have been observed in several human neoplastic disorders.

Congenital Agranulocytosis: Prolonged Survival and Terminal Acute Leukemia

Abstract Three unrelated, Caucasian patients with a disorder resembling infantile genetic agranulocytosis have been studied. There was no history of consanguinity. Parents and siblings were normal. Onset occurred before 3 weeks of age and persisted throughout life. All had severe neutropenia, myeloid arrest pattern of the bone marrow, eosinophilia, monocytosis and hyperglobulinemia. Other congenital anomalies were not present. Their clinical courses were characterized by recurrent, severe bacterial infections particularly of the skin, mouth and lungs but without bacteremia. The infections responded to, but were not prevented by antibiotics, which were administered almost continuously. Fungal infections did not occur and viral infections were not severe. Splenectomy and corticosteroids were without apparent benefit. Relative, though transient, neutrophilia occurred during a few episodes of infection. Chromosome analysis was normal in two of the patients. In one, maturation of neutrophilic leukocytes was not observed in cultures of bone marrow to which normal serum or cysteine was added. One boy is living at 14 10/12 years of age, and one died at 13¾ years of disseminated infection. A unique occurrence was the development of terminal acute leukemia (monocytic) in the third patient, a girl at the age of 14¼ years.