Chronic myelomonocytic leukemia
Introduction
Introduction to chronic myelomonocytic leukemia Chronic myelomonocyticleukaemia (CMML) is a chronic myeloid leukemia. The incidence rate is about 1 to 2/100000/year, most of them occur after the age of 60, and the ratio of male to female is about 1.5 to 3:1. Mainly manifested as two groups of symptoms, that is, symptoms associated with bone marrow pathological hematopoiesis and symptoms associated with bone marrow proliferation. The patient's cytopenia due to abnormal myelodysplasia may be manifested as fatigue, palpitations, paleness, hypothermia, infection or bleeding. The characteristics of bone marrow proliferative are as follows: abnormal mononuclear cell proliferation, and this cell has infiltration characteristics, such as extramedullary infiltration of skin, glands, gums, bones, lymph nodes, hepatosplenomegaly, and even spleen. Some authors reported that of the 24 patients, 50% had liver enlargement, 45.8% had splenomegaly, and 20.8% had lymph node involvement. basic knowledge The proportion of illness: 0.002% Susceptible people: after 60 years old Mode of infection: non-infectious Complications: bronchitis multiple lung infections
Cause
Chronic myelomonocytic leukemia
MML originates from pluripotent hematopoietic stem cells, which can cause erythroid hyperplasia, abnormal platelet count and quantity, and abnormal lymphocyte function. Different from adult type, its abnormal proliferation mainly occurs in the granule single system, and in vitro stem cell culture mainly forms CFU-GM. Chromosomal examinations are mostly normal.
Prevention
Chronic myelomonocytic leukemia prevention
1. Avoid contact with harmful factors Pregnant women and children should avoid exposure to harmful chemicals, ionizing radiation and other factors causing leukemia. When contacting poisons or radioactive materials, various protective measures should be strengthened; avoid environmental pollution, especially indoor environmental pollution; Rational use of drugs, caution with cytotoxic drugs.
2. Vigorously carry out prevention and treatment of various infectious diseases, especially viral infectious diseases. Do a good job of vaccination.
Complication
Chronic myelomonocytic leukemia complications Complications bronchitis multiple lung infections
Bronchitis, pulmonary infection, liver, spleen, lymphadenopathy, eczema-like rash, suppurative rash, bleeding due to thrombocytopenia.
Symptom
Symptoms of chronic granulocyte monocytic leukemia Common symptoms Brushing bleeding palpitations with fatigue, pale complexion, low fever, spleen, mucous membranes, bleeding spots, skin mucosal bleeding, pale lymph node enlargement
Mainly manifested as two groups of symptoms, that is, symptoms associated with bone marrow pathological hematopoiesis and symptoms associated with bone marrow proliferation.
The patient's cytopenia due to abnormal myelodysplasia may be manifested as fatigue, palpitations, paleness, hypothermia, infection or bleeding. The characteristics of bone marrow proliferative are as follows: abnormal mononuclear cell proliferation, and this cell has infiltration characteristics, such as extramedullary infiltration of skin, glands, gums, bones, lymph nodes, hepatosplenomegaly, and even spleen. Some authors reported that of the 24 patients, 50% had liver enlargement, 45.8% had splenomegaly, and 20.8% had lymph node involvement.
According to the characteristics of CMML, CMML can be divided into chronic phase, accelerated phase and acute phase. Chronic patients are relatively stable, asymptomatic or only low fever, fatigue, etc., rare hepatosplenomegaly. After entering the accelerated period, the disease progresses rapidly, especially in the case of a marked increase in monocytes. The condition is extremely serious, and most of them die from bleeding, infection or organ failure before acute changes. There is also a lack of acceleration period and the transition from chronic to acute.
Examine
Chronic myelomonocytic leukemia
1, blood routine examination has diversity. Often manifested as anemia and increased white blood cells, mainly granulocytes and monocytes increased, the absolute number of peripheral blood mononuclear cells > 10 × 109 / L, can appear naive mononuclear cells. Granulocytes often show mature granulocytes, with or without granulocyte dysplasia, showing immature granulocytes. Some patients may also have neutropenia. Most patients have reduced platelets, but some patients have normal or elevated platelet counts.
2, bone marrow puncture examination, the degree of hyperplasia is quite different, most of them are obviously active or extremely active, but also have low proliferation. Granulocytes and monocytes increase. The primary granulocytes are >5%, and the protoplasts are >5%, but the sum of the two is <20%. The erythroid and megakaryocytic proliferation is reduced. The three lines have different degrees of pathological hematopoiesis, and the typical manifestations of the erythroid are giant mega-like changes, nuclear fragmentation, petal-like nucleus, multinucleated red blood cells, and mature red blood cells basophilic spots. Granulocyte-like hematopoiesis is characterized by reduced particles, too little nuclear lobes (P?H malformation), vacuoles in the cytoplasm, etc., excessive granules as a pathological manifestation, poor specificity. The typical pathological hematopoiesis of megakaryocytes is small megakaryocytes, lymphoid megakaryocytes, single-nuclear megakaryocytes, and multi-nuclear megakaryocytes.
Diagnosis
Diagnosis and diagnosis of chronic myelomonocytic leukemia
Differential diagnosis
1. Myelodysplastic Syndrome (MDS) CMML has been classified as one of the MDS categories. The clinical manifestations and laboratory results are similar, but the peripheral blood mononuclear cells of CMML increase by >1×109/L.
2, chronic myeloid leukemia (CML) CML is also one of the myeloproliferative diseases. With the characteristics of myeloproliferative diseases, the degree of myeloproliferation is mostly active to extremely active, mainly granules, and other lines are inhibited. It has a specific Ph chromosome positive.
3, atypical chronic myeloid leukemia (aCML) The so-called atypical CML refers to Ph (-), and there is no Bcr? abl translocation of CML. Peripheral blood leukocytes were normal or decreased, and immature granulocytes increased (>0.15) in the blood, but no basophilic granules increased. The bone marrow showed active or extremely active hyperplasia, with multi-lineage hematopoiesis, mainly granulocyte hyperplasia, granule: red ratio often <10:1, eosinophils were normal or increased, but basophilic granules did not increase. This type is known to be poorly potent with tyrosine kinase inhibitors (STI571, glivec, Gleevec).
4, juvenile granulocyte monocytic leukemia (JMML) This type is a rare infant and young child, more common in infants under 2 years of age, combined with MDS / MPD dual characteristics of mixed myeloid cell tumors. Children often have skin manifestations (such as eczema and xanthomatosis). JMML is characterized by significant myeloid cell pathological hematopoiesis, mononucleosis, and elevated anti-alkaline hemoglobin (HbF). Children with Ph(-), no bcr?abl rearrangement, intramedullary naive and abnormal mononuclear cells, and erythroid precursor cell proliferation, erythroid cell abnormalities in HbF elevation (0.15 ~ 0.50), and red blood cells I antigen expression is reduced.
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