Disease Presentation

Patients with acute lymphoblastic leukemia (ALL) present with signs and symptoms related to impaired hematopoiesis from progressively worsening bone marrow involvement. Anemia may result in fatigue, light-headedness, dyspnea, and pallor. Patients with thrombocytopenia may develop petechiae, purpura, and hemorrhage. Fevers and infections also commonly occur due to neutropenia. Approximately one-third of patients have been reported to have infections, hemorrhage, or constitutional symptoms at the time of diagnosis.1-3 Clinical characteristics for patients presenting with ALL are given in Table 12.1.

Leukocytosis is often observed at the time of initial disease presentation, and the German Multicenter Trial GMALL 03/87 and 04/89 reported that 51% of their adult patients had a white blood cell (WBC) count of more than 30 X 109/L.1 Investigators at MD Anderson Cancer Center noted that 26% of their adult ALL patients had a WBC count of more than 30 X 109/L and 39% had a WBC count between 5 and 30 X 109/L.12 The French Group on Therapy for Adult ALL LALA 87 Trial observed that 31% of adult B-cell ALL patients and 55% of T-cell ALL patients had an initial WBC count of

30 X 109/L or above.4 The GIMEMA 0496 study found that 32% of their adult pro-B-cell ALL patients had an initial WBC count of more than 50 X 109/L.13 Patients with T-cell ALL have been reported to have a WBC count greater than 100 X 109/L in 25% of adult patients and in 50-77% of children.5-7 In addition, 40-50% of children with B-cell ALL have been noted to have an initial WBC count of more than 20-25 X 109/L.814

Anemia occurs frequently when patients present with ALL. A hemoglobin of less than or equal to 8 g/dL has been noted in about 30% of adult pro-B-cell ALL patients,1 while a hemoglobin of less than 12 g/dL has been observed in 85% of B-cell ALL and 65% of T-cell ALL patients.4 Investigators at MD Anderson Cancer Center found that 69% of their adult ALL patients had an initial hemoglobin of less than 10 g/dL.12 Fifty-five percent of children with T-cell ALL treated at St Jude Children's Research Hospital were noted to have an initial hemoglobin of less than 11 g/dL.7 French investigators observed that 42% of their children and 60% of infants with ALL had presenting hemoglobin levels of less than 8 g/dL.9 More than 80% of children on Children's Cancer Group (CCG) trials were also found to be anemic at diagnosis, with a hemoglobin level of less than or equal to 10 g/dL.8

Thrombocytopenia is also commonly observed at the time of diagnosis. Kantarjian et al. noted that 74%

Table 12.1 Signs and symptoms at the time of ALL diagnosis

Reported (%)a

Hemorrhage Infection B symptoms

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