High risk acute lymphocytic leukemia: A study of 141 cases with initial white blood cell counts over 100,000/cu mm

Abstract

The cases of one hundred and forty-one patients (85 males, 56 females) treated for hyperleucocytic acute lymphocytic leukemia (H-ALL) were reviewed. In all cases the initial white blood cell count was over 100,000/cu mm. One hundred patients (71%) attained complete remission (CR). The median duration of CR was six months and the median survival was nine months for all patients and 11 months for those who attained CR. Age, initial hemoglobin, and the height of initial white blood cell count over 100,000 had no significant prognostic value. Relapses occurred earlier in patients with a mediastinal mass. The results depended on the treatment used. With modern treatment, including more intensive chemotherapy and central nervous system prophylaxis, CR rate increased from 65% to 81% and median duration of CR improved from four months to ten months. The most important prognostic difference was related to the sex: CR rate was higher (78.5% vs. 66%) and median duration of CR and hematological remission was longer for females (nine months vs. six months and ten months vs. 6.5 months, respectively). This difference only appeared with modern treatments, however: before 1972 the median duration of CR was four months for both sexes, and after 1972, it was eight months for males and 17 months for females. This difference could be explained by the site of the first relapse, which was testicular in only 2% of cases before 1972 and 27% (47% of the males who relapsed) after 1972.

Childhood acute lymphoblastic leukemia with hyperleukocytosis at presentation

“…Among pediatric ALL patients, hyperleukocytosis has been found to occur in 6.1-18% of cases, in which the leukocyte count was >100×10 9 /L and in 5-8.4% of cases, in which the leukocyte count was >200×10 9 /L [1, 2, 10, 11]. The incidence of hyperleukocytosis in our study (19.2%) was similar to those reported from previous studies.…”

“…One of the immediate therapeutic problems in the management of patients with hyperleukocytosis is related to acute metabolic complications secondary to tumor lysis syndrome [1, 2, 11]. The rapid destruction of leukemic blasts because of chemotherapy may produce massive release of intracellular potassium and phosphate, which in tum results in acute renal dysfunction.…”

“…Dutcher et al [18] reported that adult acute non-lymphocytic leukemia and hyperleukocytosis was associated with a lower CR rate; these patients required more cycles of induction chemotherapy, and had significantly shorter disease-free survival and OS. Harousseau et al [2] studied the CR rates of 141 ALL patients with hyperleukocytosis aged between 3 months and 66 years. They found that initial leukocyte count was not associated with the CR rate, but CR duration tended to be shorter when initial leukocyte counts were high.…”

“…The initial leukocyte count in these cases is a particularly strong predictor of complete remission (CR) induction and event free survival (EFS). Hyperleukocytosis caused by ALL is associated with early morbidity and mortality due to the hyperviscosity created by the high leukocyte count [1, 2, 3, 4, 5]. Pediatric ALL patients who initially present with hyperleukocytosis are at risk of early complications that lead to a mortality rate as high as 20% during remission induction therapy [3, 5].…”

Testicular relapse in adult acute lymphocytic leukemia: A case report and literature review

“…In the pediatric population testicular relapse is more common in children who, at the time of initial diagnosis, have a high WBC [2,3,7,8,11,14,21,23], splenomegaly and/or hepatomegaly [2,14,21], markedly enlarged lymph nodes [9,21], a mediastinal mass [2,21], and lymphoblasts of T-cell lineage [3]. It seems that these patients who develop a testicular relapse have a disease more like that of lymphoma [2].…”