chronic lymphocytic leukemia
Introduction
Introduction to chronic lymphocytic leukemia CLL is a clonal malignant disease of B lymphocytes (uncommon T lymphocytes), which is often painless, accompanied by the progressive accumulation of slowly progressing mature small lymphocytes, the immune function of this cell. Incomplete, and low response to antigenic stimuli. Immune insufficiency is associated with inappropriate antibody formation in abnormal B cells. These antibodies have an inhibitory effect on the immune function of the body. The progression of CLL can lead to bone marrow failure and direct tissue infiltration. basic knowledge The proportion of illness: 0.003% Susceptible people: no specific population Mode of infection: non-infectious Complications: soft tissue sarcoma
Cause
Causes of chronic lymphocytic leukemia
Genetic factors (25%):
It has been reported that B-cell type CLL occurs in many people in the same family. The first-generation children of CLL have three times more risk of developing CLL or other malignant lymphoproliferative diseases than the average person, and most of them are young, suggesting genetic factors. It plays an important role in the pathogenesis of familial CLL.
Gene mutation (25%):
The p53 gene is an important tumor suppressor gene located at the 17p13.1 site and encodes a 53-kD nucleic acid phosphoprotein. The mutation or defect may be the cause of the disease in nearly half of the tumor patients. Short arm loss on chromosome 17 is only seen in 10% to 15% of CLL patients. In addition, 10% to 15% of CLL patients have p53 gene mutations, patients with p53 gene mutations are mostly progressive, with high proliferation rate of leukemia cells, short survival time, clinical characteristics of resistance to first-line treatment drugs, seen in half of Richter Syndrome and B cell lymphocytic leukemia suggest that p53 gene mutation may be acquired in the course of certain CLL patients.
Cytokine (10%):
CLL cells have the ability to secrete a variety of cytokines, such as TNF-, TGF- (transfer growth factor), IL-7 (interleukin-7), IL-5, IL-2, etc. These factors have direct or indirect stimulation of CLL leukemia Cell proliferation or prevention of apoptosis of CLL cells, and inhibition of normal lymphocytes and bone marrow hematopoietic cell proliferation, and thus cytokines are associated with CLL patients' disease and disease progression.
Other factors (10%):
Some scholars have suggested that retroviruses and ionizing radiation can cause this type of leukemia.
Prevention
Chronic lymphocytic leukemia prevention
It is generally necessary to keep the patient's daily life, to maintain a good mood, to treat the disease correctly, and to establish confidence in the fight against the disease. Otherwise, the illness of the seven emotions may make the condition worse. Diet should be light, eat spicy and hot products.
Complication
Chronic lymphocytic leukemia complications Complications, soft tissue sarcoma, lung cancer
First, due to the decline of immune function, often easy to concurrent infection, is one of the main reasons for the patient's disease deterioration and death, the most common is bacterial infection, followed by viral infection, fungal infection is less common.
Second, secondary to the second type of tumor, about 9% to 20% of patients can appear, the most common are soft tissue sarcoma, lung cancer and so on.
Symptom
Symptoms of chronic lymphocytic leukemia Common symptoms Night sweats, bloating, pleural effusion, low heat, nodular bleeding, dyspepsia, hepatomegaly, cervical lymphadenopathy, hepatosplenomegaly
Typical B-cell slow-onset is slow, early often asymptomatic, can be found by chance during physical examination or routine blood tests, and others are found by lymph nodes or hepatosplenomegaly.
(A) lymphadenopathy: the most common (70%), can be systemic, mild to moderate swelling, occasionally obvious swelling, no tenderness, touch with a rubbery feeling, no adhesion to the skin, common in the neck Department, underarm and groin, tonsil, lacrimal gland, salivary glands are timely, can produce Mikulicz syndrome.
(B) hepatosplenomegaly: common splenomegaly (40%), mild to moderate swelling, late can reach the pelvic cavity, occasionally spleen infarction or spleen rupture, hepatomegaly (10%) is not as good as the spleen, when Significant swelling with liver damage, often suggesting advanced cases.
(3) Infiltration of other organs 50% of patients have skin manifestations, more common in slower granules, specific such as nodules, erythroderma, etc., also non-specific such as pruritus, stomach and small intestine infiltration are common, visible reduction , bloating, indigestion, black feces, diarrhea, etc., pulmonary infiltration mainly diffuse nodules, miliary infiltration (40%) and pleural effusion (15%), pleural effusion, chylothorax due to lymphatic obstruction Common skeletal lesions are decalcification and bone sparse (5%), rare bone dissolution, pathological examination of more than 60% of patients with bilateral leukemia infiltration, but the general lesions are mild, about 20% of patients with proteinuria and microscopic hematuria, Nervous system lesions have speckle-like brain infiltration, and even nodular brain tumor formation, meninges can also occur, the seventh pair of cranial nerves, hypothalamus, pituitary and peripheral neuropathy, intracranial pressure can be increased.
(D) immunodeficiency manifestations: the incidence of banded or herpes simplex is high, patients are prone to purulent infections such as pneumonia, but also accompanied by a second malignant tumor, especially skin and colon tumors, accompanied by diffuse tissue cells Lymphoma, known as Richter syndrome, has an incidence of about 3.3%. It can also be associated with rheumatoid arthritis and myasthenia gravis.
The clinical features of T-cell slow-leaching are rapid onset, hepatosplenomegaly, moderate lymphocytosis, frequent invasion of the central nervous system, deep gonads and dermis, poor response to treatment, and short survival time.
Symptoms and signs often appear later than blood changes. Slow-motion is a disease of middle-aged people. About 90% of patients are over 50 years old, with an average age of 65 years. Many patients have occasionally found lymphocytosis. The earliest symptoms are often fatigue, fatigue, shortness of breath during physical activity, superficial lymph nodes, especially lymph nodes in the neck, often causing the patient's attention first, in the late piles, up to 2 ~ 3cm in diameter, no tenderness, Hard, removable, mesenteric or retroperitoneal lymph nodes can cause abdominal or urinary system symptoms, spleen to moderate swelling, liver can also be swollen, but not as slow as significant, later appetite loss, weight loss, low fever, night sweats , anemia and other symptoms, about 10% or more patients can develop autoimmune hemolytic anemia, at this time anemia is often more serious, and may appear jaundice, late may have skin purpura and bleeding tendency, susceptible to infection, especially respiratory infections, this It is related to the reduction of normal immunoglobulin production, which may be the direct cause of death. In addition, the gastrointestinal tract and skeletal system may have different degrees of damage, some diseases. Skin itching, skin occasionally leukemic infiltration, the performance of nodules or thickening of the skin is purple or reddish brown, whole body skin with redness, tonsils, salivary gland or lacrimal gland may be swollen. Growing in small clusters or not growing.
Examine
Examination of chronic lymphocytic leukemia
(1) Blood picture: positive cell anemia, white blood cell count>10×109/L, classification: lymphocyte>50%, absolute value>5.0×109/L; mature lymphocytes, visible immature lymphocytes and abnormal Lymphocytes, platelets are normal or reduced.
(2) Bone marrow: hyperplasia is active to extreme activity, with mature lymphocytes proliferating significantly, accounting for more than 40%, original, naive lymphocytes <10%, erythroid, granules are relatively reduced, megakaryocytes are normal or reduced.
(3) The reduction of blood immunoglobulin; or the increase of individual immunoglobulin, mostly IgM type, positive detection of light chain or light chain.
Diagnosis
Diagnosis and diagnosis of chronic lymphocytic leukemia
Diagnostic criteria
1. Chronic phase
(1) Clinical manifestations: asymptomatic or low fever, fatigue, sweating, weight loss and other symptoms.
(2) Blood: WBC>10×109/L, classified into neutral, young and rod-shaped granulocytes, primordial cells (original + early) 10%, eosinophils and basophils, may have A small number of nucleated cells, neutrophil alkaline phosphatase scores decreased.
(3) Bone marrow: hyperplasia is extremely active, mainly granulocyte hyperplasia, mainly middle, young and rod-shaped granulocytes, primordial cells <10%.
(4) Positive for ph chromosome and/or bcr/abl fusion gene.
(5) CFU-GM culture, colony and clustering increased significantly compared with normal.
2. Acceleration period: It has the following two or more items, and other reasons may be considered for this period:
(1) Unexplained fever, anemia, bleeding tendency, and/or bone pain.
(2) Progressive swelling of the spleen.
(3) Progressive increase or decrease of platelets caused by non-drugs.
(4) Myeloblasts (type I+II) are >10% in blood or bone marrow, but <20%.
(5) Peripheral blood basophils >20%.
(6) Significant collagen fibrosis in the bone marrow.
(7) Other chromosomal abnormalities other than ph appear.
(8) No response to commonly used therapeutic drugs.
(9) CFU-GM has defects in proliferation and differentiation, and clustering increases.
Note: 2+3 should be excluded from spleen, and 2+6 should be excluded from secondary MF.
3, blast period: one of the following can be diagnosed for this period
(1) The granulocytes in the bone marrow (I+II type) or the original leaching + young leaching >20%, or the original single + young single in the peripheral blood or bone marrow >20%.
(2) Primary particles + promyelocytes in peripheral blood >50%.
(3) Extramedullary granulocyte infiltration.
In this period of clinical symptoms, the signs are worse than the accelerated phase, CFU-GM culture.
Differential diagnosis
First, lymphoma
The structure of lymph node in the late stage of slow leaching disappears, which is indistinguishable from small lymphocytic lymphoma. At present, the general concept is that the two are actually two aspects of a disease. When the tumor cells invade the blood and bone marrow, they are slow-leaching and only invade the lymph nodes. Small lymphocytic lymphoma is not affected by blood and bone marrow, and lymphoma cell leukemia refers to small lymphocytic lymphoma (differentiated lymphocytic lymphoma, low-grade non-Hodgkin's lymphoma) Bone marrow invasion of type lymphoma.
Second, young lymphocytic leukemia
The disease course is slower and shorter, the clinical spleen, no or mild lymph node enlargement, the total number of white blood cells is significantly increased, the young lymphocytes predominate, the cells are larger than the slow-leaching cells, the nucleoli are obvious, and the response to chemotherapy is poor. Wait differently than slow shower.
Third, hairy cell leukemia
There is a reduction of whole blood cells, splenomegaly, lymph node enlargement is not common, bone marrow often appears dry pumping, tumor cells are larger than slow-leaching cells, cytoplasm is abundant, and hair filamentous protrusions on the surface can be distinguished from slow-leaching.
