Search

We report the case of a young black male with a spinal intradural granulocytic sarcoma proved by needle aspiration. The tumor was evaluated by myelography, computed tomography, and magnetic resonance imaging (MRI). Other than its rarity, the “dripping candle wax” appearance on MR T1-weighted images and the lack of enhancement with Gd-DTPA makes this case unique. Progressive changes of the tumor following chemo- and radiotherapy were successfully demonstrated by MR.

The unusual presentation of acute myeloblastic leukemia as a scapular granulocytic sarcoma in an infant without systemic manifestations is reported for the first time. Granulocytic sarcoma as a presentation of leukemia should be considered in the differential diagnosis of scapular masses during childhood. Surgery is limited to obtain sufficient tissue for histopathologic diagnosis.

Serum from 21 patients with lymphoblastic leukemia, five with myeloblastic leukemia and 30 age matched control subjects tested for thymic hormone activity in an assay that measures the induction of T cell surface antigen. This activity was subnormal in serum from 10 of 16 patients with untreated lymphoblastic leukemia (p less than 0.001) but was within the normal range when the leukemia was in remission. Low inductive activity was associated with an inhibitor of T cell induction which was less than 30,000 daltons in molecular size and interfered with induction by purified thymopoietin plus a high concentration of ubiquitin or by normal serum alone.

Summary Thymic specific deoxyribonucleotidyl transferase (terminal transferase) was identified and partially purified from peripheral blood leukocytes of a patient with chronic myeloblastic leukemia (CML). The enzyme polymerizes deoxyribonucleoside triphosphates in the presence of an oligodeoxynucleotide alone as initiator, independent of template and the incorporation of one radiolabeled deoxyribonucleotide is inhibited by the presence of other unlabeled deoxynucleotides. The purified enzyme prefers oligo(dA) among various primers tested and favors Mn ++ for activity. This finding and the previous report in human acute lymphocytic leukemic cells (4) suggest that some human leukemias may be thymic cell derived. Alternatively, the results may be explained by the presence of thymic gland derived cells among the leukemic cells or if in certain leukemias which may have nothing to do with thymus cells, terminal transferase activity is increased.

Chromosomal studies of relatives of an infant with myeloblastic leukemia and clinical and cytogenetic findings consistent with partial D1 trisomy showed excessive breakage, chromatid exchange and endoreduplication, and fragment formation in 3 generations of the pedigree, which suggests a genetic factor acting in the manner of a “breakage gene” and constituting a possible link between leukemogenesis, congenital anomalies, and chromosomal aberrations.