OVERVIEW
OVERVIEW
Malignant histiocytosis is a malignant clonal histiocytic proliferative disease characterized by high fever, anemia, enlarged liver, spleen, and lymph nodes, pancytopenia, hemorrhage, jaundice, and progressive failure; it progresses rapidly; treatment is based on chemotherapy; and it has a very poor prognosis (due to the development of the pathology, malignant histiocytosis is no longer present in the new 2016 edition of the WHO classification and is classified as, depending on the results of the tests, pathologies, etc., as other different diseases).
Whether medical insurance
Yes
Department
Hematology, Medical Oncology
Clinical symptoms
Fever, pale skin and mucous membranes, bleeding, fatigue, hepatomegaly, splenomegaly, enlarged lymph nodes, etc.
Hazards
There may be manifestations of multi-system damage, which is life-threatening.
Examination
Physical examination, blood routine, bone marrow smear, cellular immunochemical staining, histopathological examination, chromosome examination, etc.
Diagnosis
High fever, hepatomegaly, splenomegaly, enlarged lymph nodes, combined with bone marrow smear and other tests to make the diagnosis.
Treatment principle
There is no effective treatment, but chemotherapy may have some short-term effect.
Curable
Treatment may provide short-term relief of symptoms.
Dietary advice
Balanced diet and supplementation.
Questions you may be concerned about
What is malignant histiocytosis
Malignant histiocytosis is a systemic malignant disease with abnormal proliferation of histiocytes in the monocyte-macrophage system.
The clinical manifestations of malignant histiocytosis are fever, emaciation, complete blood count reduction, and enlargement of liver, spleen, and lymph nodes. It is mostly seen in young people, with rapid onset and poor prognosis.
The cell morphology of malignant histiocytosis has the following characteristics: cell cytoplasm is basophilic, with vacuoles, nuclei are large and irregular, medium to high density, occasionally binucleated, with thicker nuclear membranes, and the nuclei of the cells show mitotic phenomenon, which is often multipolar.
Due to the progress of medical technology, the efficacy of treatment for malignant histiocytosis has been significantly improved. After systematic treatment, most patients are able to alleviate clinical symptoms to a certain extent. Therefore, even if you suffer from malignant histiocytosis, do not panic, cooperate with the doctor, and actively carry out systematic treatment, which can improve the survival rate.
Causes
Epidemiology
The most common age is 20 to 40 years old, and men are slightly more common than women.
Etiology
The cause of the disease is unknown and may be related to immunity or viral infection.
Symptoms and Diagnosis
Typical symptoms
Clinical manifestations are diverse and non-specific. Generally, it can be categorized into two types: acute type and chronic type. The acute type is common, with a rapid onset, aggressive condition and a short course. The main symptom is fever, which is often the first manifestation, mostly persistent high fever, or irregular, often accompanied by excessive sweating, fatigue, progressive anemia and systemic failure. There are also cases of recurrent upper respiratory tract infections or hepatomegaly, splenomegaly, enlarged lymph nodes, which are often progressive and obvious. Superficial lymph node enlargement is more common in the late stage, with the neck and axilla being the most common, while intra-abdominal enlarged lymph nodes may form lumps. Bleeding from the skin and/or mucous membranes is common. Jaundice is seen in a few patients. Special signs or symptoms may develop and become predominant due to a particularly prominent lesion in one area. When the lungs are infiltrated, cough, hemoptysis, chest pain, etc., or even respiratory failure; the digestive system, including the esophagus, stomach, small intestine, large intestine, and pancreas, can be involved, and abdominal pain, diarrhea, constipation, nausea, vomiting, and tarry stools may occur; the heart may be involved with palpitations and other discomforts; the kidneys are involved with proteinuria, hematuria, etc.; the skeleton is involved with bone pain; and polypoid plasmacythemia is common, with the presence of Multiple plasmacytomas are also common, presenting with effusions in the plasma cavities of the chest, abdomen, and pericardium. In addition, skin nodules or lumps, breast nodules, and neurological symptoms such as limb paralysis, pain, and seizures may also be present. Chronic malignant histiocytosis is less common and has a slow onset; the main symptoms may be anemia, splenomegaly, or intermittent fever, with milder symptoms and slow progression until more than 1 year, or in the long term, even more than 10 years, before the acute, typical clinical manifestations described above begin to appear.
Diagnostic basis
1. Clinical manifestations are characterized by acute onset, prolonged fever, accompanied by progressive hepatomegaly, splenomegaly, enlarged lymph nodes, progressive hepatic failure, progressive pancytopenia, progressive systemic failure, often accompanied by jaundice, hemorrhage, skin lesions and plasma membrane cavity effusion. 2. Bone marrow smear and biopsy can reveal a certain number of abnormal cells such as huge and strange malignant cells, monocyte-like cells, lymphoid cells, immunoblast-like cells, and phagocytes. 3. The bone marrow smear and biopsy may reveal a certain number of large and strange malignant cells, mononuclear cells, lymphoid cells, immunoblast-like cells and phagocytes. Multiple examinations may be necessary because the bone marrow is infrequently involved. Concentrated smear of peripheral blood has a higher probability of detecting abnormal cells and can be used as a complementary test to the bone marrow smear.3. Immunochemical staining of cells is diffusely moderately to strongly positive for acid phosphatase and non-specific esterase in malignant histiocytosis cells. Non-specific esterase staining with AS-D naphthol acetate as a substrate is positive in monocytes, which can be inhibited by sodium fluoride, whereas cells in this disease are not inhibited by sodium fluoride and remain positive. Malignant tissue cells are positive for lysozyme in the cytoplasm. Neutrophil alkaline phosphatase positivity and points are significantly lower than normal.4. Chromosome examinationChromosomal karyotype changes, polyploidy is more significant, there is a high proportion of subdiploid and hyperdiploid. The number of subdiploid chromosomes is 45, and the constant characteristic change is the loss of one chromosome in group D. There may be a 1p11 translocation, and a 17p13 abnormality contributes to the determination of clonal anomaly. Group C chromosomes and marker chromosomes may also be present.
Treatment
Therapeutic approach
There is no effective treatment, but chemotherapy may have some short-term effect.
Radiotherapy
Refer to the chemotherapy regimen for intermediate and high malignant lymphoma, which may have some short-term effect but the maintenance time is short. Successful cases of allogeneic hematopoietic stem cell transplantation have been reported.
Prognosis
The disease is aggressive, with a mortality rate of 100% in untreated patients. About 50% of patients treated with chemotherapy may have a short remission, but most die within a few months.
Nursing care
Daily care
1. Keep the environment quiet and comfortable, reduce the adverse stimulation and psychological pressure on the patients. 2. If the patients have bleeding tendency, they should choose soft, non-irritating clothes and bedding, avoid limb collision or trauma, and avoid high water temperature or scrubbing the skin forcefully in bathing, and avoid scratching the skin. 3. The patients with high fever can be given physical cooling, such as cold compresses, etc. The patients with bleeding tendency should not be given alcohol or alcohol. Those with bleeding tendency should not use alcohol or warm water to swab the bath. 4. timely psychological guidance, try to make the patient maintain a good state of mind, avoid emotional fluctuations and mental stimulation. 5. terminal patients should be given hospice care to alleviate their suffering.
Dietary management
Balanced diet, supplemental nutrition, prepare food according to patient’s preference. Those with bleeding tendency should avoid rough, hard and stimulating food.