large granular lymphocytic leukemia
Introduction
Introduction to large granular lymphocyte leukemia Large granular lymphoblastic leukemia (LGLL), initiated in 1977, is a chronic neutropenic clinical syndrome with peripheral blood granulocyte lymphopenia. Officially named around 1985. Its cytogenetics is a clonal abnormality that determines the nature of its malignant tumor. Commonly used names include: T lymphocyte proliferative diseases, lymphoproliferative diseases with granulosa lymphocytes, and T chronic lymphocytic leukemia. The FAB collaborative group classified it as chronic T lymphocytic leukemia. Large granular lymphocytes (LGL) account for 10% to 15% of normal peripheral blood mononuclear cells, including CD3-(NK cells) and CD3 (T cells). Therefore, the REAL classification divides LGLL into T-LGLL and NK-LGLL. basic knowledge The proportion of illness: 0.0003% Susceptible people: no specific people Mode of infection: non-infectious Complications: myelofibrosis
Cause
The cause of large granular lymphocytic leukemia
(1) Causes of the disease
The cause of T-LGLL is unknown and may be related to HTLV1/II-like retrovirus, which is associated with EB virus infection.
(two) pathogenesis
The clonal proliferation of LGL requires several steps: antigen response, the addition of specific lymphokines (such as IL-12, IL-15, etc.), abnormal regulation of Fas/Fas ligand apoptosis regulation pathway, and the pathogenesis may be related to HTLV I/ The HTLV-I virus has been isolated from the II-like retrovirus; the pol, px gene region of the HTLV-II virus has been cloned and sequenced, and the EB virus infection may be related to the occurrence of NK-LGLL. LGLL has an EB virus infection rate of more than 50%; EB virus RNA or nuclear antigen can be detected by different methods.
Prevention
Large granular lymphocytic leukemia prevention
Enhance physical fitness, strengthen exercise, and prevent viral infections.
Complication
Large granular lymphocytic leukemia complications Complications, myelofibrosis
Repeated infection with fever, hepatosplenomegaly, and myelofibrosis is the most common complication of LGLL.
Symptom
Symptoms of large granular lymphocytic leukemia Common symptoms Persistent fever, night sweats, liver enlargement, lymph node enlargement, repeated infection, granulocytes, hepatosplenomegaly, rheumatoid arthritis
T-LGLL accounts for about 85% of LGLL, which is common in young patients, and one-third of patients can be asymptomatic at the time of presentation; initial symptoms include repeated bacterial infections (often associated with neutropenia), fatigue, 20% to 30% Patients may have night sweats, weight loss, main signs: 20% to 50% of patients have splenomegaly, liver swelling accounted for 20%, lymph nodes, lung infiltration is rare.
NK-LGLL progresses rapidly, with a young age of onset, with a median age of 39 years. The initial symptoms are mainly fever, night sweats, weight loss and other symptoms of B-cell disease and hepatosplenomegaly. Most patients have bone marrow infiltration, sometimes accompanied by Myelofibrosis, some patients may have gastrointestinal involvement, and rheumatoid arthritis is rare.
1, clinical manifestations of repeated infections, mild swelling of the spleen, no skin damage.
2, blood cell counts increased moderately, neutrophils decreased significantly, the number of lymphocytes was >5 × 109 / L, of which LGL accounted for 50% to 90%, lasting 3 months.
3, bone marrow can be seen that erythroid cell proliferation is low, myeloid cells or thermal barrier LGL is interstitial infiltration.
4. The immunophenotypes are CD3, CD8, CD16, TCR, GD4-, CD5-, CD7-, CD25-, CD56-.
Examine
Examination of large granular lymphocytic leukemia
1. Peripheral blood: may have anemia, thrombocytopenia, neutropenia, absolute value often <0.5 × 109 / L, lymphocyte absolute value increased, LGL significantly increased.
2. Bone marrow: showing myeloid cell maturation disorder, LGL infiltration, and plasma cells can be increased.
3. Acid phosphatase (ACP) staining: strong positive, non-specific esterase (ANAE) staining weakly positive or negative.
4. Rheumatoid factor: 60% positive, 80% anti-nuclear antibody positive, 41% anti-neutrophil antibody and anti-platelet antibody, often monoclonal high-globulinemia, cellular immunodeficiency, NK cell reduction, activity reduce.
5. The Coombs test is positive, and T-LGLL accounts for about 50%.
6. Bone marrow biopsy: B lymph node nodules and diffusely distributed LGL are often found, granulocyte maturation arrest and PRCA are also seen.
7. Lymph node and spleen biopsy: mainly found leukemia infiltration of red pulp and spleen nodules, increased plasma cells, and significant germinal center.
8. According to clinical manifestations, symptoms and signs, X-ray, B-ultrasound, and electrocardiogram were selected.
Diagnosis
Diagnosis and diagnosis of large granular lymphocyte leukemia
Patients with chronic or recurrent neutropenia or PRCA, rheumatoid factor-positive with increased LGL should consider the possibility of T-LGLL, cytomegalovirus and HIV infection can lead to mild LGL cell growth, and some patients with CD3- LGL cells increase, but the clinical manifestations of NK-LGLL are chronic, and the cytogenetic study can prove that LGL is polyclonal.
