The prognosis of chronic lymphocytic leukemia is extremely variable depending on the patient. Prognostic classifications have been developed to tailor the treatment to the changing characteristics of the disease. Those most frequently used prognostic classification used are Rai_9 in the United States and Binet10 in Europe.
| Rai Classification | |||
| Stages | Prognosis
|
Criteria | Median survival(months) |
| Stage 0 | Good Prognosis | Lymphocytosis (high lymphocyte count) and no enlargement of the lymph nodes, spleen, or liver; red blood cell and platelet counts are almost normal | > 120 |
| Stage I |
Intermediate prognosis |
Lymphocytosis associated with enlarged lymph nodes, while spleen and liver are normal and the red blood cell and platelet counts are almost normal. | > 100 |
| Stage II | Lymphocytosis associated with enlarged spleen and possibly an enlarged liver, with or without enlarged lymph nodes; the red blood cell and platelet counts are almost normal. | 70 | |
| Stage III |
Bad Prognosis |
Lymphocytosis associated with anemia, with or without enlarged lymph nodes, spleen, or liver. Platelet counts are almost normal. | 24 |
| Stage IV | Lymphocytosis associated with thrombocytopenia (low blood platelets), with or without anemia, enlarged lymph nodes, spleen, or liver. | ||
| Binet Staging(BinetC lassification ) | ||||
| Stages | Prognosis
|
Criteria | Distribution of LLCin% | Median survival(months) |
| Stage A | Good prognosis | Lymphocytosis, hemoglobin concentration> 100 g / land platelet count> 100 g / l, with less than three lymph nodes |
63 % | > 120 |
| Stage B | Intermediary prognosis | Lymphocytosis, hemoglobin concentration> 100 g / land platelet count> 100 g / l, with more than 3 lymph nodes |
30 % | 70 |
| Stage C | Bad prognosis | Lymphocytosis, hemoglobin<100 g / L or platelet count<100 G / l regardless of the number oflymph nodes or Lymphoidtissueaffected |
7 % | 40 |
Complications of Chronic Lymphocytic Leukemia
Complications can occur during or after Chronic Lymphocytic Leukemia treatment. For instance, hyperuricemia (increased uric acid in the blood) is not uncommon in patients suffering the disease.
Richter’s syndrome occurs in 10% of cases, characterized by a complication of B cell chronic lymphocytic leukemia (CLL) and hairy cell leukemia in which the leukemia changes into a fast-growing diffuse large B cell lymphoma. This complication can be fatal without proper and urgent care.
Bone marrow failure may also occur, leading to insufficient production of blood cells. The decrease in production of red blood cells (erythrocytes) leads to anemia, reduction of platelet production will lead to thrombocytopenia and bleeding disorders, and lack of neutrophils (granulocytes) promotes bacterial infections.
Humoral immunity deficit can occur and it is largely due to hypogammaglobulinemia, immune deficiency disease. It must be confirmed by specific blood test: Immunoglobulins (IgA, IgG, IgM). Infections are more common and tend to affect the head and neck and lung area.
Treatment of Chronic Lymphocytic Leukemia
The treatment of Chronic Lymphocytic Leukemia depends on the stage of the disease, as it is the case of almost cancer. According to the Binet classification, there is no indication today to treat patients with stage A CLL which corresponds to 65 % of patients; it is however necessary to treat patients with stage B or C. Etiological treatment based on oral or injectable chemotherapy. In cases of poor prognosis, it may be offered to the patient intensive treatment in the form of intensive chemotherapy followed by bone marrow transplantation: autologous (donor is the patient himself) or allogeneic (donor being a member of his siblings). Complications are specifically treated depending on their characteristic.
Therapeutic Modalities
Chemotherapy – chemotherapy drugs used in the intensive treatment of Chronic Lymphocytic Leukemia include chlorambucil (used alone), fludarabine (used alone), as well as monthly courses of CHOP chemotherapy which is a combination of four agents:
- C : Cytoxan® (cyclophosphamide)
- H : Adriamycin® (hydroxy doxorubicin)
- O : vincristine (Oncovin®)
- P : Prednisone
Targeted Therapy – Leukemic cells being CD20 +, an anti- CD20 (monoclonal antibody specifically recognizing CD20) can be used during the treatment to stop the proliferation of the cancer cells. Rituximab ( MabThera®) is thus part of the therapy.
Treatment of Autoimmune Complications
Autoimmune complications are treated with corticosteroids continuously. Second-line injection in intravenous immunoglobulin allows transient improvement. Radiotherapyof the spleen or surgical removal (splenectomy) of the latter is useful as a last resort.
Treatment of Infectious Complications
- Infectious complications of bacterial origin are treated with appropriate antibiotics.
•Repeatedinfections related to humoral immunity characterized by hypogammaglobulinemia can be prevented by the use of intravenous immunoglobulin versatile. - If CD4 lymphopenia, reflecting a deficit of cellular immunity, a preventive treatment byBactrim(sulfamethoxazoleand trimethoprim) is indicated.