Prolymphocytic leukemia. Clinical, histopathological, and cytochemical observations

Abstract

The clinical, histopathological, and cytochemical features of eight patients with prolymphocytic leukemia, a rare variant of chronic lymphocytic leukemia, were reviewed. Six of the patients had clinical evidence of "massive" splenomegaly at the time of diagnosis, and in four of these this clinical impression was confirmed by splenic weights in excess of 2000 g. No patient had significant lymph node enlargement. The initial leukocyte count was elevated in seven patients and was greater than 100 X 10(9)/1 in four of them. The absolute prolymphocyte count ranged from 16.3 to 378.1 X 10(9)/1 and was greater than 100 X 10(9)/1 in four patients. Splenectomy in four patients had no lasting effect on the peripheral leukocyte count. In the four patients in whom the disease was shown by surface marker or immunocytochemical studies to be of B-cell origin, the histopathologic features were distinctive and were characterized by a pattern of infiltration which was nodular and diffuse in both the splenic red pulp and the bone marrow, whereas involvement of the lymph nodes was pseudonodular. In one patient in whom the prolymphocytes had cytochemical characteristics suggestive of T-cells, the distribution of the abnormal cellular proliferation in the lymph nodes was paracortical and the infiltrations of the spleen and the bone marrow were diffuse.

Prolymphocytic leukemia: Histologic findings in atypical cases

“…Prolymphocytic leukemia can usually be distinguished from other lymphoproliferative disorders in leukemic phase by the presence of characteristic prolymphocytes in the peripheral blood and the absence of significant lymphadenopathy on physical examination ' (Galton et al, 1974;Bearman et al, 1978;Come et al, 1980). Galton described 15 patients with prolymphocytic leukemia and made special mention of the absence of significant lymphadenopathy (Galton et al, 1974).…”

“…Galton described 15 patients with prolymphocytic leukemia and made special mention of the absence of significant lymphadenopathy (Galton et al, 1974). Bearman later described 6 additional patients with prolymphocytic leukemia, none of whom had marked lymphadenopathy (Bearman et al, 1978). Subsequent articles on this disease have also made note of this aspect of the physical examination (Kjeldsberg et al, 1980;Catovsky et al, 1973).…”

“…Victims of the disease are usually elderly males. A survey of 41 patients described in the literature, 61 per cent of whom were males, revealed a mean age of 67 years (Hollister and Coleman, 1982;Galton et al, 1974;Reddy et al, 1981;Oscier et al, 1981;Bearman et al, 1978). Anemia of moderate degree is usually present along with striking leukocytosis.…”

“…The mean white blood cell count at presentation in the patients noted above was 275 x 10 9 / L. Treatment of prolymphocytic leukemia has not had much impact on the clinical course; the response is usually poor and survival after diagnosis is usually brief. There may be brief improvement with drugs used in treatment of chronic lymphocytic leukemia, chlorambucil and prednisone (Hollister and Coleman, 1982;Bearman et al, 1978). Improvement has been reported with splenectomy or splenic irradiation (Hollister and Coleman, 1982;Oscier et al, 1981), and in a few cases, the response was sustained over many months.…”

“…Certain findings on physical examination are characteristic of the disease; patients usually have marked splenomegaly, but in contrast to other lymphoproliferative disorders, significant lymphadenopathy has been curiously absent (Galton et al, 1974;Bearman et al, 1978;Come et al, 1980;Kjedlsberg et al, 1980). We describe two cases of prolymphocytic leukemia in which lymphadenopathy was one of the most prominent features of the disease.…”