Acute lymphoblastic leukemia (acute lymphocytic leukemia) - malignant disease resulting from clonal proliferation of immature lymphoid cells. The disease usually occurs in children with a peak incidence between 2 and 4 years and the ratio of cases in boys and girls 1.2:1. The clinical picture is characterized by intoxication, anemic, osteo-articular, proliferative and hemorrhagic syndromes. Characterized by nonspecific complaints of fatigue, malaise, fatigue, loss of appetite. There are unmotivated rises in body temperature, pale skin, due to intoxication and anemia.
Arthralgia associated with osteoporosis and subperiosteal proliferation of tumor nature. Increasingly concerned about the pain in the lower extremities and spine. Proliferative syndrome is characterized by splenomegaly, an increase in peripheral (and sometimes mediastinal) lymph nodes, hemorrhagic syndrome develops in approximately 50% of cases, is characterized by petechiae, and hemorrhages.
Morphological classification of acute lymphoblastic leukemia according to the Fab-classification includes three groups:
L1, типичные mikrolimfoblasty (TdT +, CD19 +, CD10 +)? There is approximately 75% of ALL
L2 or T-ALL. Large lymphoblasts with distinct nucleoli of irregular shape (TdT +, CD7 +)? It occurs in 20% of cases
L3 or B-ALL. And for the children in the 75-80% of the typical type of L1.
Metastasis. Sometimes it develops due to the appearance neuroleukemia extramedullary lesions of the central nervous system. For diagnosis neuroleukemia conduct brain CT, MRI, EEG, CSF study, etc. Leukemic infiltration of the testes is less common than neuroleukemia and diagnosis based on biopsy data. Other options: Mikulicz syndrome (infiltration of the salivary glands), and infiltration of the kidneys, lungs and heart.
Diagnosis of acute lymphoblastic leukemia (lymphocytic leukemia)
Typically, studies begin with an analysis of peripheral blood. At the same time revealed the blasts almost always (98% of cases). For the blood smear is characterized by "the failure of leukemic" or leukemic hiatus "- available only blasts and mature cells without intermediate stages. For acute lymphocytic leukemia is characterized by normochromic anemia and thrombocytopenia. Other signs are less common: leukopenia (20%), leukocytosis (50%). On the basis of complaints and the blood picture can sometimes be a suspected acute lymphocytic leukemia. Accurate diagnosis is impossible without a study of red bone marrow. And here is important histological, cytochemical, and cytogenetic characteristics of bone marrow blasts. It is important to identify the Philadelphia chromosome (Ph-chromosome), which is formed by reciprocal translocation sites 9 and 22 chromosomes. For an accurate diagnosis and planning of therapy for ALL is necessary to conduct additional tests to determine the damage of other organs (their infilratsii blast): examination of an otolaryngologist, a neurologist, urologist, as well as carrying out medical imaging (CT, MRI, ultrasound of the abdomen).
The factors of poor prognosis include: age over 10 years, high leukocytosis, male gender, presence of Ph-chromosome leukemic CNS involvement, morphology of B-ALL. Treatment of acute lymphoblastic leukemia (lymphocytic leukemia)
Treatment of acute lymphoblastic leukemia due to the appearance and the continuous improvement of chemotherapy can now obtain a complete clinical and hematological remission, and in most cases to achieve a complete cure. Different treatment protocols use the same principles:
The principle of a wide range of effects. We use different mechanism of action of chemotherapy.
The principle of adequate dosages. Lower dosages increases the risk of relapse, increased dosages increases the risk of complications.
The principle of phasing and continuity in the appointment of chemotherapy.
The principle of adequacy of prevention extramedullary lesions (ensuring the penetration of chemotherapy into the CNS and testes, CNS radiotherapy, intrathecal medications, etc.).
The basic principle of treatment is that it is continuous from start to completion of treatment. If a relapse of the disease, the most adverse considered early relapse, bone marrow relapse and recurrence of L2. Relapses greatly reduce the chances of full recovery, as accurately identify the presence and location of the pool of residual blasts is quite difficult. Treatment of relapse usually is to use a few short blocks of high-dose chemotherapy. Most effective when it adopted the protocol BFM.
Further improvement of treatment protocols in developed countries can hope for better results and reduce the number of cases of possible recurrence.