Destructive bone involvement is a rarely recognized complication of hairy-cell leukemia (leukemic reticuloendotheliosis). We report a case in which localized hip pain and lytic bone lesions were the presenting manifestations. A second case of bone involvement in a patient with previously diagnosed h
Enzymes of purine metabolism in hairy cell leukemia
✍ Scribed by Dr. Anthony D. Ho; Günter Dietz; Irmtraut Trede; Reinhard Schwartz; A. Victor Hoffbrand; Werner Hunstein
- Publisher
- John Wiley and Sons
- Year
- 1986
- Tongue
- English
- Weight
- 464 KB
- Volume
- 58
- Category
- Article
- ISSN
- 0008-543X
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✦ Synopsis
Differences in activities of the purine degradative enzymes, adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), and 5'-nucleotidase (5'NT), have been observed among different classes of lymphoid malignancies. Recent studies have shown that hairy cell leukemia (HCL) may respond to treatment with the ADA inhibitor, 2-deoxycoformycin. This study demonstrates that the cells of HCL have significantly lower levels of ADA and 5"T (P always <0.01) when compared to levels in normal B-or T-lymphocytes, but have higher levels of P N P (P < 0.001 for both comparisons). Recent studies have shown that when treated with 12-0-tetradecanoyl-phorbol-13-acetate (TPA), cells of B-cell chronic lymphatic leukemia (B-CLL) acquire phenotypic characters of HCL. The authors have therefore also investigated the changes in enzyme pattern of B-CLL after incubation with TPA B-CLL cells are characterized by low levels of ADA, PNP, and 5'-NT, but TPA caused a marked increase in P N P activity (P < 0.001, t test for paired samples), a pattern similar to HCL. The results from biochemical studies are thus in accordance with the hypothesis that HCL cells are more mature than B-CLL cells. The special enzyme profile of HCL suggests that a P N P inhibitor might also be effective in the treatment of this disease.
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