OVERVIEW
Overview of Mast Cell Leukemia
Mast cell leukemia is a clonal disease caused by malignant proliferation of mast cells. It is extremely rare and only reported in isolated cases, with fever, chills, weight loss, maculopapular rash, malaise, abdominal pain, black stools, and bone pain as the main clinical manifestations.
Whether medical insurance
yes
Department
Hematology
Alias
Basophilic histiocytic leukemia
Clinical Symptoms
Fever, weight loss, abdominal pain, black stools, dyspnea, hepatomegaly, splenomegaly, enlarged lymph nodes, maculopapular rash, and malaise.
Harms
It can affect the respiratory system and digestive system, causing complications such as bronchospasm, gastrointestinal ulcers, hemorrhage, perforation, etc. In severe cases, it can lead to shock and death.
Complications
Bronchospasm, hypotensive shock, peptic ulcer, bleeding, perforation, etc.
Examination
Blood routine, blood smear, bone marrow smear, electrocardiogram, blood routine, urine routine and other tests.
Diagnosis
Diagnosis can be made on the basis of clinical manifestations, combined with laboratory tests and imaging tests.
Treatment principle
Hormone therapy, combined chemotherapy, immune cell therapy and molecular targeted therapy can be used.
Curability
The disease develops rapidly, and the prognosis is less favorable.
Dietary advice
Strengthen nutrition and supplement foods rich in protein and vitamins.
Causes
Etiology
The etiology is nominal and may be related to mutations in the C-KIT gene.
Symptoms and Diagnosis
Typical Symptoms
As mast cell leukemia is often a progressive manifestation of mast cell hyperplasia, there are generally two major types of specific manifestations.1. It is caused by infiltration of mast cells into multiple organ systems, such as enlargement of the liver, spleen, lymph nodes, or bone damage, bone tenderness, or osteolytic lesions; the skin infiltration causes itching, reddening, and the emergence of pigmented urticaria, which varies in scope and size, and appears as a brown pigmented macule or papule, or sometimes as nodules, and the skin is scratchy and positive. 2. Positive skin scratch test.2. Mast cell cytoplasm has heterochromatic particles containing heparin, hyaluronic acid, and can produce histamine, various glycosaminoglycans, kinin, prostaglandins, etc. The release of histamine can cause more persistent urticaria. The release of histamine can cause more stubborn gastroduodenal ulcer, abdominal pain, vomiting blood, black stools, and headache; facial and limb edema, a large amount of histamine release can cause sudden skin flushing, bronchospasm, palpitation, urticaria, and even shock, and excessive release of heparin can cause hemorrhagic tendency.
Diagnostic basis
Hepatomegaly, splenomegaly, enlarged lymph nodes, or cause bone damage, bone pressure pain or osteolytic damage.2. Skin infiltration causes itching, redness, pigmented urticaria, ranging in size, brown pigmented macules or papules, sometimes nodular, and a positive skin scratch test.3. The patient may suddenly develop flushing of the skin, bronchial spasms, palpitations, urticaria, or even shock.4. Mast cells in the bone marrow exceeding 20% in bone marrow or more than 10% in peripheral blood.
Treatment
Treatment guidelines
Hormonal therapy, combined chemotherapy, immune cell therapy and molecular targeted therapy can be used.
Drug therapy
1. Glucocorticoids, sodium cromoglycate, leukotriene blockers, etc. can be used to treat acute and chronic inflammation. 2. immune cell therapy, e.g. interferon-alpha. 3. molecular targeted therapy, imatinib combined with hormone therapy is available.
Radiotherapy
Chemotherapy should be chosen for patients with progressive mast cell leukemia.
Prognosis
The disease develops rapidly and has a poor prognosis.
Nursing care
Daily care
1. Maintain the appropriate greenhouse degree and ventilate frequently.2. Keep the patient’s body clean to prevent the spread of bacteria in the body, and do a good job in oral care, perineal and anal care to prevent all kinds of infections.3. Family members should be good at understanding and supporting the patient, and learn to listen to avoid the patient’s bad emotions.4. Keep the bed sheets flat, bedding and clothing light and soft, and avoid the collision of the limbs or traumas.5. The patient should use a soft-bristled toothbrush to brush the teeth. Avoid picking teeth with toothpicks. 6. Avoid physical stimuli: e.g. temperature change, physical exertion, friction and pressure on the skin injury site, excessive sunlight exposure, etc.
Dietary management
High protein, high vitamin, easy to digest food should be given, try to avoid fried, spiny or bone-containing food, nuts with shells and hard fruits.