Overview.
Lymphangioblastic leukemia, also known as lymphosarcoma cell leukemia, is a term that is no longer used due to new histologic and immunologic studies. In the early stage of malignant lymphoma, the lesions are confined to the lymphoid tissues. In the late stage, the lymphoma cells spread widely to the bone marrow and appear in the peripheral blood, accompanied by a decrease in normal blood cells, and the leukemia that appears at this time is known as lymphosarcomatous leukemia, or also known as lymphosarcomatous leukemia stage.
Etiology
The disease is transformed by the progression of lymphoma. The etiology of lymphoma has not been fully elucidated so far, and its development involves various aspects such as heredity, infection by viruses and other pathogens, physical and chemical factors such as radiation and chemicals, and immune status.
Symptoms
It often presents with progressive enlargement of lymph nodes, hepatosplenomegaly, and occasionally giant spleen. Mediastinal lymph node enlargement may be complicated by superior vena cava compression. Further aggravation of anemia, bleeding of skin and mucous membranes, fever, bone and joints, nervous system, skin may be involved, pericardial effusion, pleural effusion, ascites and other manifestations may occur.
Examination
1. Blood picture
Lymphoma cells with abnormal morphology can be seen in blood film under light microscope. Red blood cells and platelets are reduced; the total number of white blood cells is increased, which is (15~40)×109/L.
2. Bone marrow
The presence of tumor cells identical to those in the peripheral lymphoid tissue. Lymphomatoid leukemia resembles acute lymphoblastic leukemia, but the cells have pseudopod-like protruding anomalies, the nuclei are lacunar or twisted, and the nucleoli are often single and eccentric.
3. Immunologic test
T or B lymphocyte surface markers, respectively.
4. Genetic tests
For example, myc gene test and bcl-2 gene test.
Diagnosis
Diagnosis can be made on the basis of the history of lymphoma, the results of blood and bone marrow tests, and the clinical manifestations.
Differential diagnosis
It is mainly differentiated from acute lymphoblastic leukemia. It is currently believed that lymphoblastic lymphoma and acute lymphoblastic leukemia are different names for the same disease, while lymphoblastic lymphoma can be distinguished from other types of lymphoblastic leukemia by immunophenotyping and chromosome and gene examination.
Treatment
1. General treatment
Sterilization and isolation measures should be given during chemotherapy, oral care, and drugs that affect platelet function, such as aspirin, should be avoided.
2. Induction of remission
Induction of remission is to minimize the leukemia cells with chemotherapeutic drugs after the diagnosis of the disease to achieve complete remission.
3. Consolidation and Intensive Treatment
After complete remission, there are still residual leukemia cells of varying degrees, which need to be further consolidated and intensified with chemotherapeutic drugs.
4. Hematopoietic stem cell transplantation
Allogeneic hematopoietic stem cell transplantation is recommended for adult patients with suitable donors, especially those with high risk factors.
5. Targeted drug therapy
New drugs such as rituximab (Merova), BTK inhibitors, lenalidomide, etc. can be used in the treatment of this disease.
6. Cell therapy
For example, CAR-T cell therapy has been successfully used in the treatment of this disease.
Prognosis
This disease is a manifestation of advanced dissemination of lymphoma and usually has a poor prognosis, but some cases have a better prognosis, such as Burkitt’s lymphoma.
Prevention
Avoid some pathogenic factors, such as ionizing radiation, cytotoxic drugs, chemicals such as phosphorus, fluorine, benzene and carbon tetrachloride.
Nursing care
Strengthen nutrition, moderate exercise, avoid crowded places. Improve the resistance of the body.