A boy is described with clinical and laboratory manifestations which were indistinguishable from von Willebrand’s disease. However, the relatively recent onset of symptoms, the negative family history, and the normal coagulation studies in both parents and six siblings led to the belief that the bleeding syndrome was acquired rather than inherited. The patient subsequently developed systemic lupus erythematosus following a smallpox vaccination. The findings of von Willebrand’s syndrome disappeared following corticosteroid therapy and did not return after cessation of therapy.
An indirect immunofluorescence assay was used to quantitate TdT- containing (TdT+) cells in the mononuclear leukocyte fraction of peripheral blood from normal subjects and patients with acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LL). In normal children (10) and adults (10), 0.036% +/- 0.014% (mean +/- SD) and 0.030% +/- 0.015% TdT+ cells were found. In peripheral bloods from 10 children receiving chemotherapy for tumors other than ALL or LL, 0.040% +/- 0.039% TdT+ cells were found. Serial determinations were performed on 15 patients with ALL or LL who were in clinical remission. Eight of these patients remained in continuous remission and always had fewer than 0.11% TdT+ cells in their peripheral blood. Three patients who developed systemic relapse were found to have progressively rising numbers of TdT+ cells in their peripheral blood prior to clinical evidence of relapse. All 3 of these patients had greater than 0.1% TdT+ cells in their peripheral blood from 3 to 8 wk prior to clinical relapse. In 3 other patients, localized extramedullary relapse developed, but no trend was found on serial TdT determinations. Thus, the indirect immunofluorescence assay for TdT detects a small population of cells in normal peripheral blood. In patients with ALL, progressive increases above this normal level were associated with subsequent bone marrow relapse.
A case of Hageman factor deficiency in a 13-year-old white female is presented and the laboratory procedures necessary for identification of this abnormality are outlined. No bleeding was observed at the time of tonsillectomy and adenoidectomy, confirming the benign nature of the disorder. Decreased Hageman factor activity was found in the plasma of other family members, being in accord with the autosomal recessive nature of the characteristic responsible for this factor.
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