Release date: 2007-09-06
Diagnosis and treatment of invasive NK cell leukemia Invasive NK cell leukemia (ANKL) is a large granular lymphocytic leukemia (LGLL), mainly found in young adults, with a median age of onset of 39 years. The disease has been confirmed to be associated with EB virus infection. The tumor cells are monoclonal and highly malignant. The disease progresses rapidly, the median survival time is less than 2 months, and even the cases that have not been treated and died have a very poor prognosis. Since Fernandez first discovered and named ANKL in 1986, there have been such cases reported. In 1997, WHO classified it as a malignant proliferative disease of mature NK cells. In 2001, it officially listed it as a unique clinical subtype of mature T/NK cell tumors.
The vast majority of ANKL patients have obvious B symptoms at the onset, accompanied by superficial lymphadenopathy, some patients with skin nodules as the first performance, skin biopsy showed large granular lymphocytes (LGL) infiltration. More than 80% of patients have liver and spleen invasion, jaundice, abnormal liver function, palpation of liver and splenomegaly, and even spleen and giant liver. Gastrointestinal tract, central nervous system and reproductive organs are easily involved, which may be related to the easy infiltration of extramedullary tissue by CD56+ tumor cells. Some patients with ANKL may develop hemophagocytic syndrome, which may be related to the release of large amounts of interferon-gamma (IFN-γ) from tumor cells.
The onset of ANKL is fulminant, which can lead to multiple organ failure in the short term. It is mainly caused by liver failure, and gradually affects other organs, causing kidney, heart and lung failure, and eventually leading to death. Some ANKL also caused three blood cells to decline due to disease progression, abnormal blood coagulation, leading to intracranial hemorrhage and death.
At the beginning of the onset, about half of the patients may have abnormally elevated white blood cells, mainly lymphocytes. However, neutropenia, thrombocytopenia and anemia gradually appear during the development of the disease. The leukemia cells are LGL, the nucleus is irregular, the chromatin is deeply stained, the nucleolus is not obvious or obvious, the cytoplasm is rich, and the azurophilic granules of varying thickness can be seen. Previously, the diagnostic criteria for LGLL was LGL count >2×109/L, but recently, 25% to 30% of newly diagnosed patients had a peripheral blood neutrophil count of <0.5×109/L. Most patients have coagulopathy at the onset of the disease, and concurrent with diffuse intravascular coagulation (DIC). In the later stages of the disease, almost all patients have complete cytopenia and liver failure. Because the disease is associated with EBV infection, serum EBV-antibody is positive.
In the bone marrow smear, extensive or focal infiltration of leukemia cells can be seen, which is characterized by large granular lymphocytes, which are larger than normal lymphocytes, irregular in morphology, irregular in nuclei, and large sputum in the cytoplasm of most cells. Green particles with reactive hemophagocytosis and myeloid cell maturation disorders. Bone marrow biopsy showed abnormal cells clustered or flaky, common apoptotic bodies and bone marrow necrosis.
The classical immunophenotypes of leukemia cells are CD13-, CD33-, CD7+, CD5-, CD2+, CD56+, CD16+/-, and CD3 negative on the cell surface, but CD3ε chain expression is observed in the cytoplasm, and TCR gene rearrangement is negative. Recent literature reports that the ANKL cellular immunophenotype is also CD25+, CD122+, CD132+, which are the α, β and γ chains of IL-2 receptor, respectively. NK cells have a range of killer immunoglobulin receptors (KIRs), each of which selectively expresses one or more KIRs. Patients with NK cell proliferation after EBV infection may have deviations from the normal KIRs characteristics of NK cells, but the balance can be restored after successful treatment, while patients with T lymphocyte proliferation after EBV infection do not deviate from the above characteristics, so this is the diagnosis and treatment. Provide the basis.
At present, the accepted diagnostic criteria for this disease are: 1 often with fever, swollen liver, spleen and lymph nodes, involving peripheral blood and bone marrow; 2 neutropenia, thrombocytopenia and anemia, increased lymphocyte ratio, visible large granularity Lymphocytes; 3 bone marrow smear and biopsy showed more large infiltration of large particles; 4 consistent with NK cell immunophenotype; 5EBV-antibody positive; 6 no specific cytogenetic changes, more common chromosomal abnormalities are del ( 6) (q21q25); 7 exclude other large granular lymphoproliferative diseases.
ANKL belongs to one of the LGL leukemias and must be identified with other subtypes of LGL, as shown in Table 1.
Many of the characteristics of ANKL are similar to those of extranodal NK/T-cell lymphoma. For example, they are mainly found in Asian populations, and the same immunophenotype is associated with EBV infection. However, the age of extranodal NK/T-cell lymphoma is relatively large, involving the nasopharynx, and can also invade the upper and upper digestive tract. The skin and testicles can also be involved, but peripheral blood and bone marrow are rare. In addition, extranodal NK / T cell lymphoma is sensitive to radiotherapy / chemotherapy, and the prognosis is relatively good.
At present, there is no effective treatment for ANKL, and the combination of chemotherapy and hematopoietic stem cell transplantation is now used. Invasive NK cell leukemia should be treated with a strong chemotherapy regimen for acute lymphoblastic leukemia. After remission, continue with high-dose chemotherapy or allogeneic hematopoietic stem cell transplantation; if it is refractory disease or progression, change the treatment plan or use empiric therapy. Studies have shown that CD56+ acute leukemia has poor prognosis regardless of how to actively treat it. Some people think that it may be related to the expression of multidrug resistance gene and P glycoprotein in both normal and malignant NK cells. In addition, the disease is prone to tumor lysis syndrome during combined chemotherapy, causing hyperuricemia, hyperkalemia, hypocalcemia and acute renal failure, aggravating the condition and directly leading to death.
In short, ANKL is a highly invasive malignant disease, the disease progresses rapidly, and patients often die due to multiple organ failure. There is currently no effective treatment. Only early diagnosis and prevention of further development of the disease can win time for patient survival. ——Midi Medical Network
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