Prolymphocytic leukemia

Introduction to young lymphocytic leukemia Prolymphocyticleukemia (PLL) is a relatively rare type of lymphocytic leukemia. The incidence is mainly middle-aged and old, generally over 50 years old, more common in men, belonging to a peripheral lymphocyte tumor originating from B or T cells. basic knowledge The proportion of illness: 0.005% Susceptible people: no special people Mode of infection: non-infectious Complications: anemia, central nervous system leukemia

Cause of juvenile lymphocytic leukemia

Cause:

Although there are several related factors, the cause is unknown, and the disease is gender-susceptible.

Pathogenesis

Cytogenetics, about 60% of patients have 14q abnormalities, the rate of trisomy abnormalities on chromosome 12 is lower than that of CLL patients (patients with this abnormality may be developed by CLL), 6q- and the weight of chromosomes 1 and 12 Occasionally visible; t(6:12)(q15;q14) abnormalities are also reported, t(2;13)(q35;q14) is a common chromosomal change in children with rhabdomyosarcoma, also seen in B-PLL To.

Cell genomics, 80% of PLL is the origin of malignant B cells. These cells often have monoclonal immunoglobulin gene rearrangements and express B cell surface antigens similar to CLL cells. Unlike B-CLL, PLL often expresses higher concentrations. Surface immunoglobulin, mostly IgM (with or without IgD); can not form rosettes with mouse red blood cells, B cells of half of PLL patients express CD5 surface antigen, monoclonal antibody SN8 can bind PLL B cells with CLL or HCL The B cells are differentiated. 20% of the PLLs are malignant T cells, which often express CD2 antigen; they can form rosettes with sheep red blood cells. In these cases, 75% of patients express CD4, 20% express CD8 antigen; about 15% of cases of leukemia The cells simultaneously expressed CD4 and CD8, indicating that the cells originated from the original T cells, T-PLL often expressed CD7 antigen, but not CD1, HLA-DR or TdT, T-PLL cells were positive for -specific esterase, and the enzyme type It is a large particle type.

Young lymphocytic leukemia prevention

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease. Due to the intricate condition of leukemia, the treatment of leukemia is very difficult, not a one-size-fits-all treatment, and there are many factors that can affect the condition of leukemia. Therefore, leukemia must require a comprehensive treatment plan. There are various causes of leukemia, which makes the condition change ever, making the treatment of leukemia extremely difficult. Therefore, comprehensive leukemia treatment is needed, which not only pays attention to physical treatment, but also psychologically and diet. Pay attention to it. Reasonable dietary habits: Maintain a light diet. Eat more fresh vegetables and aquatic products.

Young lymphocytic leukemia complications Complications anemia central nervous system leukemia

Similar to other leukemias, it can be combined with infection, fever, hemorrhage, anemia, hepatosplenomegaly, skin damage, liver and kidney dysfunction, cardiopulmonary dysfunction, central nervous system leukemia and other complications, severe cases can be life-threatening.

Symptoms of young lymphocytic leukemia Common symptoms Lymph node enlargement Ascites Oral ulcer Weight loss Low heat bleeding tendency Pleural effusion

The course of the disease can be acute, subacute and chronic, mostly chronic, initial symptoms include fatigue, weakness, weight loss, anorexia, often low fever and recurrent oral ulcers, a small number of patients have bone pain and acquired bleeding tendency Spleen enlargement is a feature of this disease. It can have spleen, mild to moderate enlargement of the liver, little or no lymphadenopathy in B-PLL, and T-PLL lymphadenopathy is common. T-PLL patients can still There are related manifestations of trunk, facial and arm skin involvement, usually appearing at an early stage, often manifested as diffuse invasive erythema on the face and ear, no desquamation, non-itch erythroderma; in some cases, skin infiltration resembles a honeycomb It is ineffective for antibiotic treatment. Very few cases may have central nervous system leukemia, leukemia pleural effusion or ascites, and some patients may have cardiopulmonary complications.

Examination of young lymphocytic leukemia

Blood picture

Almost all patients have positive cell pigmentation anemia, more than half of patients have thrombocytopenia; white blood cells are significantly increased, often greater than 100 × 109 / L, the proportion of young lymphocytes is greater than 50%, compared with mature lymphocytes its morphological characteristics The cell body is slightly larger, the cytoplasm is rich, the nuclear/plasma ratio is slightly lower, the nuclear chromatin concentration is in the form of massive or thick, unevenly arranged, densely distributed along the periphery of the nuclear membrane, and the nuclear and nucleoli are not synchronized. That is, the nucleolus is obvious and the nucleoplasm is relatively mature. The young lymphocytes of T-PLL patients have a high nuclear/plasma ratio, strong basophilic cytoplasm, no granules, and often have protrusions; the nucleus is elliptical or irregular, and can be folded and twisted. The nuclear chromatin is dense and the nucleolus is obvious. It is usually one. About 19% of the T-PLL cells are small, and the nucleoli are not obvious under the light microscope. The nucleolus is visible under the electron microscope. This group is a small cell variant T-PLL. .

2. Bone marrow

Hyperplasia was markedly active, mainly lymphocytes. The morphology of young lymphocytes was consistent with peripheral blood. Dry bone marrow extraction was rare. Biopsy showed diffuse or mixed infiltration of leukemia cells.

Phase contrast microscope: 1 to 2 nucleus of young lymphocytes, some cells have coarse particles in the cytoplasm, sometimes clustered on the cytoplasmic side, and some cell cytoplasm can be seen with protruding small villi.

Ultrastructure: Young lymphocytes have villi-like small protrusions, 0.07-2.5m long, most cells have large nucleoli, nuclear round, cytoplasm rich, Golgi is underdeveloped.

Cytochemistry: 80% of patients had positive PAS, ACP-positive, TRAP, POX, SB were negative, and non-specific esterase was positive in T-PLL patients.

3. Cytogenetics

60% of B-PLLs have 14q, 76% of T-PLLs have chromosome 14 breaks, breakpoints are q11 and q32, inv(14)(q11;q32) is a common karyotype, and 53% of T-PLLs have Chromosome 3 on chromosome 8.

4. Immunophenotype

T-PLL: CDla-, YdT-, CD2, CD3, CD5, CD7, CD25-/, CD38 /-; CD4, CD8- accounted for 65%, CD4, CD8 accounted for 2l%, CD4-, CD8- 13%.

B-PLL: CD5-/, CD10-/, CD11c-, CD25-, CD23-/, CD24, B-1y7-, FMC7, slg ( or +).

5. Biochemical examination

Serum calcium is normal.

According to clinical manifestations, symptoms and signs, X-ray, CT, MRI, B-ultrasound, electrocardiogram and other tests were selected.

Diagnosis and differentiation of young lymphocytic leukemia

Diagnostic criteria

Comprehensive domestic and foreign literature, the diagnostic criteria of PLL are as follows.

1. More common in patients over 50 years old, the spleen is obvious, and the course of disease is progressive.

2. Peripheral blood leukocytes increased significantly, and young lymphocytes were >55%.

3. Young lymphocytes are characterized by large cell bodies, basophilic quality, dense nuclear chromatin, clear nucleoli, and low nuclear ratio.

4. Immunophenotype B-PLL: high expression of SIg, CD19, CD79b, CD7, CD22 positive, CD5, CD23 negative, T-PLL: CD2, CD3, CD4, CD5, CD7 positive.

5. Can exclude CLL.

Diagnostic evaluation:

The spleen of the elderly is obviously swollen, the course of disease is progressive, the number of white blood cells and lymphocytes are obviously elevated, and there are nucleoli, which can be basically diagnosed as PLL. For example, nucleoli atypical, feasible Fulgen staining, therefore, clinical manifestations And blood morphological examination is the basic basis for diagnosis. In order to distinguish between B-PLL and T-PLL, clinical manifestations can provide clues to determine the dependence on immunophenotypic detection.

Differential diagnosis

1.CLL Both are mostly elderly, with splenomegaly and peripheral blood lymphocytes significantly increased, the main identification points:

Most of the 1CLL patients progress slowly, the course of disease can last for several years, even more than 10 years, and the PLL condition is progressive, the treatment response is poor, and the survival period is only about 1 year.

The white blood cell increase of 2PLL is much higher than that of CLL, and the 3/4 PLL white blood cell is >100×109/L.

The lymphocytes of 3PLL have clear nucleoli, while the lymphocytes of CLL are mature small lymphocytes with no nucleoli.

4 Different immunophenotypes, whether T-PLL needs to be identified with less than 1% of T-CLL in CLL is still controversial. Most authors believe that T-CLL is actually a variant of T-PLL, so there is no discriminating value.

In addition, 10% of B-CLL leukemia cells are mixed, that is, mainly small lymphocytes, but about 10% of lymphocytes are naive, may have nucleoli, sometimes up to 50%, called CLL /PLL, 80% CLL/PLL, its naive lymphocyte ratio is stable, clinical manifestations, immunophenotype, duration and survival are not different from typical CLL. Some people call it CLL variant, in addition, 20% CLL/ PLL can be converted into a typical PLL during the course of disease. The average life span after transformation is only 9 months. It belongs to CLL variant CLL/PLL. According to clinical manifestations and immunophenotype, it can be distinguished from PLL, converted CLL/PLL, according to transformation. Pre-existing CLL clinical and immunophenotypic characteristics are not difficult to identify with PLL.

2. Hairy cell leukemia (HCL) HCL has the characteristics of splenomegaly and peripheral blood lymphocytosis, so it needs to be identified with PLL:

The peripheral blood of 1HCL patients is mostly due to the decrease of whole blood cells, while the white blood cells of PLL patients must increase significantly.

The lymphocytes of 2HCL are hair cells (HC), and most of the lymphocytes of PLL have nucleoli, which are significantly different in morphology.

3HCL was positive for HC-tartaric acid phosphatase staining (TRAP) and PLL was negative.

4 immunophenotypes are different.

5 electron microscopy: HCL showed significant hairy bulges and lamellar layers, ribosomal complexes, and PLL had obvious nucleoli.

3. Acute lymphoblastic leukemia (ALL) There are a large number of naive lymphocytes in the blood and bone marrow of ALL patients, and sometimes the spleen is also obviously enlarged, and it needs to be differentiated from PLL:

1ALL is more common in children and adolescents, and PLL is mainly seen in the elderly.

2ALL is often associated with superficial lymphadenopathy, while PLL is rare and is only found in some T-PLLs.

3 Morphologically, the primitive of ALL, the chromatin of naive lymphocytes is thinner than PLL, and the nucleolus is not as clear as the PLL.

4 immunophenotype, B-ALL expressed earlier antigens, such as CD20, CDl9, CD10, while B-PLL expressed more mature antigens, such as SIg.

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