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In patients with juvenile chronic myelogenous leukemia, the peripheral-blood granulocyte–macrophage progenitors proliferate in vitro in the absence of exogenous granulocyte–macrophage colony-stimulating factor (GM-CSF), even when the cells are plated at very low densities.2 In these patients, the growth is due to a striking hypersensitivity of granulocyte–macrophage colony-forming units (CFU-GM) to GM-CSF alone3 and not to granulocyte colony-stimulating factor (G-CSF), as mistakenly stated in the Case Records.1
Spontaneous growth of CFU-GM depends on the presence of a small amount of GM-CSF produced by monocytes or macrophages, since the depletion of adherent cells abrogates this abnormal growth pattern. Thus, a simple clonogenic assay of the fraction of mononuclear cells derived from peripheral blood could have provided sufficient data, together with the clinical and laboratory findings, which included increased serum muramidase (lysozyme) levels, to establish the diagnosis of juvenile chronic myelogenous leukemia; this assay could also have been used to monitor remission.
Franco Locatelli, M.D.
Patrizia Comoli, M.D.
Vittorio Rosti, M.D.
University of Pavia
27100 Pavia, Italy
References
To the Editor: Locatelli et al. question the use of liver biopsy to make a diagnosis of juvenile chronic myelogenous leukemia in our patient. Since we are fully aware that such a diagnostic approach is unusual, we thank Dr. Locatelli and his colleagues for the opportunity to make clear that in this particular case, such a biopsy was both appropriate and necessary.
In the weeks preceding the liver biopsy, immunophenotyping of bone marrow aspirate as well as peripheral blood was nondiagnostic for any leukemic process, as were the findings on examination of two bone marrow aspirates and two bone marrow biopsy specimens. As noted in the case presentation, an aggressive workup continued. The liver biopsy was considered only when, over a period of a few days, serious respiratory distress developed as a result of rapidly progressing hepatosplenomegaly. A liver biopsy and a further bone marrow aspiration were performed at virtually the same time in order to establish the diagnosis of juvenile chronic myelogenous leukemia and exclude the possibility of primary liver disease. During this period the leukocyte count had risen from approximately 25,000 per cubic millimeter to 50,000 to 73,000 per cubic millimeter. When the biopsy indicated juvenile chronic myelogenous leukemia, systemic chemotherapy with daunorubicin and cytarabin was promptly instituted, with a rapid reduction in the burden of proliferating myeloid cells and alleviation of the respiratory compromise.
A clonogenic assay of mononuclear cells derived from peripheral blood can indeed confirm a diagnosis of juvenile chronic myelogenous leukemia. Such an assay, however, at best takes one to two weeks, especially if one seeks to measure sensitivity to GM-CSF in addition to spontaneous proliferation. The rapid progression of leukocytosis and hepatosplenomegaly in our patient rendered a clonogenic assay useless for diagnostic purposes.
Eric F. Grabowski, M.D., Sc.D.
David H. Ebb, M.D.
William S. Ferguson, M.D.
Massachusetts General Hospital
Boston, MA 02114
Robert I. Parker, M.D.
State University of New York at Stony Brook
Stony Brook, NY 11794-8111
References
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