mast cell leukemia
Introduction
Introduction to mast cell leukemia Mast cell leukemia (MCL), also known as tissue basophilic leukemia, is a clonal disease caused by malignant proliferation of mast cells. MCL can be transformed from mast cell disease (type II or III) or it can be MCL from the beginning. Because mast cell leukemia is often a progressive manifestation of mast cell enlargement, there are generally two major types of specific manifestations: 1. Infiltration by mast cells: such as liver, spleen, lymph nodes, or bone damage, bone tenderness or dissolution Bone damage; skin infiltration caused itching, redness, pigmented urticaria, ranging in size, brownish brown rash or papules, sometimes nodules, skin scratch test positive. 2, mast cells in the cytoplasm of the mast cells have heterogeneous particles: containing heparin, hyaluronic acid, and can produce histamine, a variety of glycosaminoglycans (glycosaminoglycans), kinins, prostaglandins and other release of histamine can cause more stubborn Gastroduodenal ulcer, abdominal pain, hematemesis, melena, and headache; edema of the face and limbs. basic knowledge The proportion of illness: 0.005% Susceptible people: no special people Mode of infection: non-infectious Complications: digestive ulcers shock
Cause
The cause of mast cell leukemia
Causes:
The cause is unknown, and the reported incidence may be related to the C-KIT gene mutation.
Pathogenesis
MCL also has the pathological and clinical features of AML. Only the cells infiltrating the organs are mainly mast cells. Lymph nodes, hepatomegaly, splenomegaly, and sometimes osteolytic lesions may occur. Infiltration of the skin is a major feature of MCL. The skin lesion is pigmented urticaria, which is brownish rash or papule, which can form nodules and is positive for skin scratch test.
The cytoplasm of mast cells contains granules that secrete various active substances, which secrete histamine, form hyperhistamine and the corresponding clinical manifestations are a special set of MCL, such as skin flushing, itching, bronchospasm, and even Hypotension, syncope and shock can also cause refractory stomach, duodenal ulcer, with blood, perforation and other serious complications. If excessive heparin is released, it can cause various bleeding tendency.
Prevention
Mast cell 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.
Complication
Mast cell leukemia complications Complications, digestive ulcer, shock
The main complications are bronchospasm, hypotension shock, digestive ulcer, hemorrhage, and perforation, which are caused by the release of histamine from mast cells.
Symptom
Mast cell leukemia symptoms Common symptoms Abdominal pain Lymph node enlargement Black stool spotted dyspnea Difficult bleeding tendency
Because mast cell leukemia is often a progressive manifestation of mast cell enlargement, there are generally two broad categories of specific manifestations.
1, caused by infiltration of mast cells: such as liver, spleen, swollen lymph nodes, or cause bone damage, bone tenderness or osteolytic damage; skin infiltration caused by itching, redness, pigmented urticaria, ranging in size, Brownish brown rash or papules, sometimes nodules, positive skin scratch test.
2, mast cells have cytoplasmic granules containing heparin, hyaluronic acid, and can produce histamine, a variety of glycosaminoglycans (glycosaminoglycans), kinins, prostaglandins, etc.: release of histamine can cause more stubborn Gastric duodenal ulcer, abdominal pain, hematemesis, melena, and headache; facial, limb edema, massive histamine release can cause sudden flushing of the skin, bronchospasm, palpitations, urticaria, even shock, heparin release More can cause bleeding tendency, in addition, there may be fatigue, fever, weight loss, anorexia.
Examine
Examination of mast cell leukemia
1, blood: often manifested as mild to moderate anemia, platelets can be reduced or normal, white blood cell count increased as high (10 ~ 150) × 109 / L, the number of mast cells increased significantly.
2, bone marrow: hyperplasia is active or extremely active, there are more tissue basophils, basophils can also increase, other cells are suppressed, megakaryocytes and thrombocytopenia, mast cells and basophilic The morphology of granulocytes is different. The former is larger and irregular, with a diameter of 15-20 m. It has more cytoplasm. It can have pseudopods or vacuoles. The particles are coarse and dense, and the size is relatively uniform. It is dark purple and can cover the nucleus, but it is generally nuclear. Still visible, the nucleus is round or oval, centered or deviated, with more nuclear chromatin and no nucleoli. Under electron microscopy, the particles of mast cells have an amorphous structure or a typical paper-like feature.
3. Cytochemistry: Sudan black B (SB) and alcian blue staining were positive, chloroacetate and acid phosphatase were positive, peroxidase (POX) and -naphthol esterase were negative.
4. Immunophenotype: The myeloid marker CD33 may be positive, indicating that it is derived from the myeloid line, while CD4 and CD2 indicate that it is related to the T cell line.
X-ray examination showed osteosclerosis or destruction; B-ultrasound, hepatosplenomegaly, lymphadenopathy; according to the condition, clinical manifestations, symptoms, signs, choose to do ECG, CT, gastrointestinal endoscopy.
Diagnosis
Diagnosis and identification of mast cell leukemia
Diagnostic criteria
The domestic diagnostic criteria are as follows:
First, the clinical manifestations of leukemia or mast cell hyperplasia: 1, lymph nodes, liver or splenomegaly; 2, mast cells release histamine and other substances caused by local and systemic changes, including skin flushing, pigmented urticaria , itchy skin, episodes of bronchospasm, difficulty breathing, palpitations, hypotension, syncope, shock and other symptoms.
Second, there are mast cells (tissue basophils) in the peripheral blood.
Third, the mast cells in the bone marrow are obviously proliferating, occupying more than 30% of the nuclear cells.
Fourth, elevated urine histamine.
5. When the bone marrow is pumped or has skin and organ infiltration, a biopsy is needed to confirm the diagnosis.
Differential diagnosis
I. Mast cell disease: Mast cell disease also has a series of clinical manifestations caused by hyperhomocytemia, and the identification of mast cell leukemia is mainly based on the number of mast cells in the bone marrow, >30% is diagnosed as mast cell leukemia, in addition, anemia The presence of thrombocytopenia and the appearance of naive mast cells also support the diagnosis of leukemia.
Second, basophilic leukemia: It and mast cell leukemia can also have a series of clinical manifestations of hyperhomocytemia.
