LEUKEMIA

  Delong Liu, MD, PhD

Assistant Professor of Medicine
Director and Attending Physician
Mini-Allogeneic Bone Marrow Transplantation
Division of Oncology
New York Medical College

For detailed therapy information, please visit www.zaaci.org

What is Leukemia?

Leukemia is a form of “blood cancer”. It is the result of uncontrolled growth of blood cells, i.e., red cells (RBC), white cells (WBC), and platelets. Normally, the three populations of above-mentioned blood cells are mainly made in the bone marrow of  sternum, and pelvic bones.  There are precursor cells, so-called “stem cells”, in the bone marrow for RBC, WBC, and platelets. When genetic changes occur in the DNA sequences in the precursor cells, uncontrolled growth may take place, resulting in the leukemia.

 

Different Types of Leukemia

  1. Acute Leukemia

a.   Acute myeloid leukemia (AML): This is the most common type of acute leukemia in adults. There are seven different subtypes, M1 through M7

b.   Acute lymphoid leukemia (ALL): This type is most common in children. There are three subtypes:  L1, L2, and L3. Each type can be a T- or B- cell in origin.

c.   Acute undifferentiated or biphenotypic leukemia: the leukemia cells in this class can not be assigned to the above two types.

  1. Chronic Leukemia

a.   Chronic myelogenous leukemia (CML): there are three different phases,  including chronic phase, accelerated phase, and blast crisis phase.

b.   Chronic lymphocytic leukemia (CLL): there are different stages of CLL, depending upon lymph node involvement, anemia, low platelets, and spleen involvement.

Diagnosis of Leukemia

Patients with leukemia frequently present with anemia, low platelets counts,  bleeding,  and infection. From time to time, patients may be found to have spleen or lymph node enlargement on physical examination. Not infrequently, patients may have persistent bleeding after minor surgery, such as tooth extraction. A clear diagnosis is based upon peripheral blood smear, bone marrow evaluation, flow cytometry study, and cytogenetic study.  These should be done for every patient. The flow cytometry study is routinely done to assist classification of  the leukemia. The cytogenetic study is particularly important for predicting prognosis. AML with the cytogenetic changes, t(8;21), t(15,17), and inv(16),  are considered to have good prognosis. Deletion of chromosome 5, 7, or addition of chromosome 8 are considered to be poor prognostic factors, whereas normal or other type of cytogenetic changes are intermediate. ALL with t(4;11) or t(9;22) have poor prognosis.

 

Therapy for acute leukemia

In general, there are different phases of treatment for acute leukemia, including  induction, consolidation, maintenance.

a.       AML: most commonly used chemotherapy drugs are cytarabine and idarubicine, and “7+3” is still the standard way of giving the drugs outside of a clinical study. A  repeat of bone marrow evaluation is routinely done around day 14 to assess the effect of the chemotherapy. Another bone marrow evaluation is usually done at day 28 to document whether the leukemia is in complete remission, in other words, whether the leukemia has been controlled to the extent that no evidence of leukemia can be identified by routinely used clinical studies. Consolidation chemotherapy is usually given a week or two after patient has recovered from chemotherapy. Maintenance chemotherapy is not routinely used for AML. Two new advances in the therapy of AML-M3 (acute promyelocytic leukemia) initially originated from China. All trans-retinoic acid and arsenic trioxide have been proven to increase rate of complete remission with significantly less toxicity and fewer toxic death in the therapy of M3.

b.      ALL: The therapy of ALL is considerably more complicated and less standardized. There is still no uniformed accepted “standard therapy” for ALL. Vincristine, prednisone, cyclophosphomide, daunorubicine, and asparaginase, are commonly used for induction. High dose cytarabine and mitoxantrone are being studied for induction therapy. Maintenance therapy for at least two years is usually given. Administration of chemotherapy to the central nervous system through the spinal fluid (intrathecal chemotherapy) is routinely done. Methotrexate and cytarabine are the commonly used drugs. Prophylactic radiation therapy is not done any more due to unproven benefit and long-term complications. Lymphoblastic lymphoma is considered to be another form of ALL, and treated the same way. Mature B-cell ALL (Burkitt’s ALL, L3) is treated differently from other type of ALL, usually involving the use of high dose methotrexate. Children with ALL do much better than adults, due to the different biological behavior and characteristics of their leukemia cells.

Bone Marrow Transplantation (BMT) for Leukemia

There are Allogeneic BMT and autologous BMT. Allogeneic means from a different person, whereas autologous means from self. Allogeneic BMT is much more valuable. There are six different types of HLA ( human leukocyte antigen) antigens usually tested, the  so-called “HLA typing”.  If two individuals have all six HLA matched, it is considered to be HLA identical. Siblings (brothers and sisters) have a 25% chance to be HLA-identical. In the Caucasian population, due to the large database from HLA bank, there is roughly a 70% chance for any Caucasian patient, particularly the European descendent,  to have a HLA-identical donor. For young patients with CML, allogeneic BMT is the preferred treatment, and roughly 75% of those patients may be cured. Interferon therapy should be stopped at least 3 months before BMT. Allogeneic BMT is usually not advisable for patients who are older than 55 years old due to the significantly higher possibility of treatment-related death. Transplantation of growth factor-mobilized peripheral blood stem cells may become more and more popular due to the ease of procurement and rapid engraftment. However, long-term sequelae are still unclear. Mini-allogeneic BMT with milder form of treatment is under intensive clinical study and may be used for therapy of patients who are older than 55 years old and /or have other significant diseases that preclude them from conventional BMT.