hairy cell leukemia
Introduction
Introduction to hair cell leukemia Hairy cell leukemia is a rare chronic clonal B cell proliferative disorder. The cytoplasm of pathological Bxibao is obviously bulging, like hairy, so it is named hair cell (HC). HC mainly infiltrates the bone marrow and spleen, clinically with splenomegaly, circulating blood cell reduction and peripheral blood and/or bone marrow with a large amount of HC as an important sign. basic knowledge The proportion of illness: 0.3% Susceptible people: no special people Mode of infection: non-infectious Complications: hypersplenism, splenomegaly, spleen rupture, fracture, nodular polyarteritis, myelofibrosis
Cause
Hair cell leukemia cause
Genetic factors (50%):
The cause of HCL is unknown. Human T-cell leukemia virus II (HTLV-II) has been proposed. Epstein-Barr virus infection is associated with HCL. The healthy people who are exposed to radiation or organic solvents have a high degree of controversy. It is recognized that there are many cases of HCL in the family, and some HCL patients have chromosomal abnormalities such as chromosome 12, 14q, 5, del (5q13), etc., so genetic factors may be related to the pathogenesis of HCL.
Prevention
Hair cell leukemia prevention
Do not touch X-rays and other harmful radiation too much. Personnel engaged in radiation work should do personal protection and strengthen preventive measures. Infants and pregnant women are more sensitive to radiation and vulnerable to injury. Women should avoid exposure to excessive radiation during pregnancy, otherwise the incidence of leukemia in the fetus is higher. Do not abuse drugs. Use caution when using chloramphenicol, cytotoxic anticancer drugs, immunosuppressive drugs, etc., and must be guided by a doctor. Do not use or abuse them for a long time.
Complication
Hair cell leukemia complications Complications, spleen hyperthyroidism, spleen and spleen rupture, nodular polyarteritis, myelofibrosis
1. Co-infection with HCL is highly susceptible to neutropenia and infection, which may be caused by tumor cell infiltration and splenomegaly, or by cell damage or mononuclear cell deficiency.
2. Splenic spleen, some patients with hypersplenism may be combined with spleen rupture.
3. Combined with bone damage, such as osteolytic manifestations, pathological fractures.
4. Combined with nodular polyarteritis, usually occurs after splenectomy.
5. Combined with myelofibrosis.
Symptom
Hairy cell leukemia symptoms Common symptoms Lymph node enlargement Low heat fatigue Liver enlargement Thrombocytopenia Whole blood cell reduction
Spleen enlargement can be seen in about 85% of patients, splenomegaly is more common, superficial lymph nodes are less swollen, occasionally mild hepatic enlargement, soft tissue infiltration, osteolytic bone damage, spleen rupture are reported. HCL patients are often accompanied by some manifestations of autoimmune diseases, such as symptoms of arthritis, joint pain, skin erythema, skin damage, low fever, etc. These symptoms are not related to tumor burden, often self-limiting, corticosteroid treatment is effective.
Examine
Examination of hairy cell leukemia
1. Pathology: spleen HC infiltration is limited to red pulp, white pulp is not tired, and atrophy. Although the number of blood mononuclear cells decreased in HCL, the number of tissue cells in the red pulp remained increased. In addition, a special "red cell lake" is seen, which is a pseudo sinus surrounded by HC, filled with red blood cells in the middle. Hepatic HC mainly infiltrates the portal area and hepatic sinus, and hepatoma-like pseudosinus formation is seen.
2. Imaging: A few X-ray examinations of patients with HCL showed osteolytic lesions, sometimes accompanied by signs of osteosclerosis around them, and patients often complained of bone pain. Pulmonary infection is a common complication of HCL, and chest X-ray or CT examination may have corresponding signs.
Diagnosis
Diagnosis and diagnosis of hairy cell leukemia
Diagnostic criteria
The easiest and most convenient diagnostic method is to detect hair cells. If the morphology is atypical or difficult to determine, TRAP staining should be performed. The positive ones can be basically diagnosed. The immunophenotype detects a certain number of CD11C, CD25 or CD103 positive cells. Diagnostic value, electron microscopy is also a meaningful diagnostic tool when conditions are available.
Differential diagnosis
1. Primary chronic myelofibrosis (IMF): Both are characterized by splenomegaly, "dry pumping" of the bone marrow and increased fibrous tissue. There is also a decrease in whole blood cells in the late stage of IMF, so it has similarities with HCL.
2. Hypersplenism: HCL has splenomegaly and complete blood cell reduction and other signs of hypersplenism, so it should be differentiated from primary and other secondary splenomegaly, hair cells (or) in the bone marrow as the main identification Point, atypical morphology should be distinguished by TRAP staining or immunophenotypic examination. Secondary splenomegaly has clinical and laboratory characteristics of primary disease, and it is usually not difficult to distinguish from HCL.
